Advertisement

POEMS Syndrome and Other Atypical Plasma Cell Disorders

  • Angela Dispenzieri
Part of the Contemporary Hematology book series (CH)

Atypical PCD Associated with Peripheral Neuropathy as Dominant Phenotype

Other important peripheral neuropathies (PNs)-associated plasma cell disorders (PCDs) include those induced by chemotherapy and those related to Waldenström macroglobulinemia, multiple myeloma, and light-chain amyloid, but these are beyond the scope of this chapter. Disorders like cryoglob-ulinemia, scleromyxedema can also be associated with PN, but those entities will be reviewed in later sections since PN is not their dominant phenotype. In this section, the focus will be on POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome and MGUS (mono-clonal gammopathy of undetermined significance)-associated PN.

POEMS Syndrome

The complexity of the interaction with plasma cell dyscrasia and PN became increasingly evident in 1956 with Crow's description of two patients with osteosclerotic plasmacytomas with neuropathy, and other “striking features,” which included clubbing, skin...

Keywords

Vascular Endothelial Growth Factor Multiple Myeloma Vascular Endothelial Growth Factor Level Monoclonal Gammopathy Chronic Inflammatory Demyelinating Polyneuropathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

Financial support: AD was supported in part by grants CA125614, CA062242, CA107476, CA15083, and CA11345 from the National Cancer Institute. Financial disclosures: Clinical trial support from Celgene, Cytogen, Neurochem.

References

  1. 1.
    Crow R. Peripheral neuritis in myelomatosis. Brit Med J 1956;2:802–4.PubMedGoogle Scholar
  2. 2.
    Driedger H, Pruzanski W. Plasma cell neoplasia with osteosclerotic lesions. A study of five cases and a review of the literature. Arch Intern Med 1979;139(8):892–6.PubMedGoogle Scholar
  3. 3.
    Iwashita H, Ohnishi A, Asada M, Kanazawa Y, Kuroiwa Y. Polyneuropathy, skin hyperpigmentation, edema, and hypertrichosis in localized osteosclerotic myeloma. Neurology 1977;27(7):675–81.PubMedGoogle Scholar
  4. 4.
    Mangalik A, Veliath AJ. Osteosclerotic myeloma and peripheral neuropathy. A case report. Cancer 1971;28(4):1040–5.PubMedGoogle Scholar
  5. 5.
    Evison G, Evans KT. Sclerotic bone deposits in multiple myeloma [letter]. Br J Radiol 1983;56(662):145.Google Scholar
  6. 6.
    Reitan JB, Pape E, Fossa SD, Julsrud OJ, Slettnes ON, Solheim OP. Osteosclerotic myeloma with polyneuropathy. Acta Medica Scandinavica 1980;208(1–2):137–44.PubMedGoogle Scholar
  7. 7.
    Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine 1980;59(4):311–22.PubMedGoogle Scholar
  8. 8.
    Kelly JJ, Jr., Kyle RA, Miles JM, Dyck PJ. Osteosclerotic myeloma and peripheral neuropathy. Neurology 1983;33(2):202–10.PubMedGoogle Scholar
  9. 9.
    Takatsuki K, Sanada I. Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol 1983;13(3):543–55.PubMedGoogle Scholar
  10. 10.
    Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984;34(6):712–20.PubMedGoogle Scholar
  11. 11.
    Soubrier MJ, Dubost JJ, Sauvezie BJ. POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome. Am J Med 1994;97(6):543–53.PubMedGoogle Scholar
  12. 12.
    Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003;101(7):2496–506.PubMedGoogle Scholar
  13. 13.
    Soubrier M, Dubost JJ, Serre AF, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum 1997;40(4):786–7.PubMedGoogle Scholar
  14. 14.
    Gherardi RK, Belec L, Soubrier M, et al. Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. Blood 1996;87(4):1458–65.PubMedGoogle Scholar
  15. 15.
    Hitoshi S, Suzuki K, Sakuta M. Elevated serum interleukin-6 in POEMS syndrome reflects the activity of the disease. Intern Med 1994;33(10):583–7.PubMedGoogle Scholar
  16. 16.
    Rose C, Zandecki M, Copin MC, et al. POEMS syndrome: report on six patients with unusual clinical signs, elevated levels of cytokines, macrophage involvement and chromosomal aberrations of bone marrow plasma cells. Leukemia 1997;11(8):1318–23.PubMedGoogle Scholar
  17. 17.
    Orefice G, Morra VB, De Michele G, et al. POEMS syndrome: clinical, pathological and immunological study of a case. Neurol Res 1994;16(6):477–80.PubMedGoogle Scholar
  18. 18.
    Nakazawa K, Itoh N, Shigematsu H, Koh CS. An autopsy case of Crow-Fukase (POEMS) syndrome with a high level of IL-6 in the ascites. Special reference to glomerular lesions. Acta Pathologica Japonica 1992;42(9):651–6.PubMedGoogle Scholar
  19. 19.
    Emile C, Danon F, Fermand JP, Clauvel JP. Castleman disease in POEMS syndrome with elevated interleukin-6 [letter; comment]. Cancer 1993;71(3):874.PubMedGoogle Scholar
  20. 20.
    Saida K, Ohta M, Kawakami H, Saida T. Cytokines and myelin antibodies in Crow-Fukase syndrome. Muscle Nerve 1996;19(12):1620–2.PubMedGoogle Scholar
  21. 21.
    Feinberg L, Temple D, de Marchena E, Patarca R, Mitrani A. Soluble immune mediators in POEMS syndrome with pulmonary hypertension: case report and review of the literature. Crit Rev Oncog 1999;10(4):293–302.PubMedGoogle Scholar
  22. 22.
    Hashiguchi T, Arimura K, Matsumuro K, et al. Highly concentrated vascular endothelial growth factor in platelets in Crow-Fukase syndrome. Muscle Nerve 2000;23(7):1051–6.PubMedGoogle Scholar
  23. 23.
    Belec L, Mohamed AS, Authier FJ, et al. Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman's disease. Blood 1999;93(11):3643–53.PubMedGoogle Scholar
  24. 24.
    Belec L, Authier FJ, Mohamed AS, Soubrier M, Gherardi RK. Antibodies to human herpesvirus 8 in POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome with multicentric Castleman's disease. Clin Infect Dis 1999;28(3):678–9.PubMedGoogle Scholar
  25. 25.
    Bosch EP, Smith BE. Peripheral neuropathies associated with monoclonal proteins. [Review] [63 refs]. Med Clin North Am 1993;77(1):125–39.PubMedGoogle Scholar
  26. 26.
    Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome [letter]. Lancet 1996;347(9002):702.PubMedGoogle Scholar
  27. 27.
    Gherardi RK, Belec L, Fromont G, et al. Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome. Blood 1994;83(9):2587–93.PubMedGoogle Scholar
  28. 28.
    Shikama N, Isono A, Otsuka Y, Terano T, Hirai A. A case of POEMS syndrome with high concentrations of interleukin-6 in pericardial fluid. J Intern Med 2001;250(2):170–3.PubMedGoogle Scholar
  29. 29.
    Gherardi RK, Chouaib S, Malapert D, Belec L, Intrator L, Degos JD. Early weight loss and high serum tumor necrosis factor-alpha levels in polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome. Ann Neurol 1994;35(4):501–5.PubMedGoogle Scholar
  30. 30.
    Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998;21(11):1390–7.PubMedGoogle Scholar
  31. 31.
    Scarlato M, Previtali SC, Carpo M, et al. Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 2005;128(Pt 8):1911–20.PubMedGoogle Scholar
  32. 32.
    Tokashiki T, Hashiguchi T, Arimura K, Eiraku N, Maruyama I, Osame M. Predictive value of serial platelet count and VEGF determination for the management of DIC in the Crow-Fukase (POEMS) syndrome. Intern Med 2003;42(12):1240–3.PubMedGoogle Scholar
  33. 33.
    Bryce AH, Ketterling RP, Gertz MA, et al Cytogenetic analysis using multiple myeloma targets in POEMS syndrome. In: Proceedings of American Society of Oncology Meeting ; 2007; Chicago, IL.Google Scholar
  34. 34.
    Soubrier M, Labauge P, Jouanel P, Viallard JL, Piette JC, Sauvezie B. Restricted use of Vlambda genes in POEMS syndrome. Haematologica 2004;89(4):ECR02.Google Scholar
  35. 35.
    Sung JY, Kuwabara S, Ogawara K, Kanai K, Hattori T. Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve 2002;26(2):189–93.PubMedGoogle Scholar
  36. 36.
    Suarez GA, Dispenzieri A, Gertz MA, Kyle RA. The electrophysiologic findings of the peripheral neuropathy associated with POEMS. Clin Neurophysiol 2002;113(Suppl 1):S9.Google Scholar
  37. 37.
    Vital C, Vital A, Ferrer X, et al. Crow-Fukase (POEMS) syndrome: a study of peripheral nerve biopsy in five new cases. J Peripher Nerv Syst 2003;8(3):136–44.PubMedGoogle Scholar
  38. 38.
    Crisci C, Barbieri F, Parente D, Pappone N, Caruso G. POEMS syndrome: follow-up study of a case. Clin Neurol Neurosurg 1992;94(1):65–8.PubMedGoogle Scholar
  39. 39.
    Bergouignan FX, Massonnat R, Vital C,. Uncompacted lamellae in three patients with POEMS syndrome. Eur Neurol 1987;27(3):173–81.PubMedGoogle Scholar
  40. 40.
    Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine (Baltimore) 1980;59(4):311–22.Google Scholar
  41. 41.
    Lesprit P, Authier FJ, Gherardi R, et al. Acute arterial obliteration: a new feature of the POEMS syndrome? Medicine 1996;75(4):226–32.PubMedGoogle Scholar
  42. 42.
    Zenone T, Bastion Y, Salles G, et al. POEMS syndrome, arterial thrombosis and thrombocythaemia. J Intern Med 1996;240(2):107–9.PubMedGoogle Scholar
  43. 43.
    Soubrier M, Guillon R, Dubost JJ, et al. Arterial obliteration in POEMS syndrome: possible role of vascular endothelial growth factor. J Rheumatol 1998;25(4):813–5.PubMedGoogle Scholar
  44. 44.
    Bova G Pasqui AL, Saletti M, Bruni F, Auteri A. POEMS syndrome with vascular lesions: a role for interleukin-1beta and interleukin-6 increase—a case report. Angiology 1998;49(11):937–40.PubMedGoogle Scholar
  45. 45.
    Kang K, Chu K, Kim DE, Jeong SW, Lee JW, Roh JK. POEMS syndrome associated with ischemic stroke. Arch Neurol 2003;60(5):745–9.PubMedGoogle Scholar
  46. 46.
    Koga H, Tokunaga Y, Hisamoto T, et al. Ratio of serum vascular endothelial growth factor to platelet count correlates with disease activity in a patient with POEMS syndrome. Eur J Intern Med 2002;13(1):70–4.PubMedGoogle Scholar
  47. 47.
    Ghandi GY, Basu R, Dispenzieri A, Basu A, Montori V, Brennan MD. Endocrinopathy in POEMS syndrome: the Mayo Clinic Experience. Mayo Clin Proc;(In press).Google Scholar
  48. 48.
    Sanada S, Ookawara S, Karube H, et al. Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation. Am J Kidney Dis 2006;47(4):672–9.PubMedGoogle Scholar
  49. 49.
    Navis GJ, Dullaart RP, Vellenga E, Elema JD, de Jong PE. Renal disease in POEMS syndrome: report on a case and review of the literature. [Review] [25 refs]. Nephrol Dial Transplant 1994;9(10):1477–81.PubMedGoogle Scholar
  50. 50.
    Viard JP, Lesavre P, Boitard C, et al. POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions. Am J Med 1988;84(3 Pt 1):524–8.PubMedGoogle Scholar
  51. 51.
    Sano M, Terasaki T, Koyama A, Narita M, Tojo S. Glomerular lesions associated with the Crow-Fukase syndrome. Virchows Arch A Pathol Anat Histopathol 1986;409(1):3–9.PubMedGoogle Scholar
  52. 52.
    Takazoe K, Shimada T, Kawamura T, et al. Possible mechanism of progressive renal failure in Crow-Fukase syndrome [letter]. Clin Nephrol 1997;47(1):66–7.PubMedGoogle Scholar
  53. 53.
    Mizuiri S, Mitsuo K, Sakai K,. Renal involvement in POEMS syndrome. Nephron 1991;59(1):153–6.PubMedGoogle Scholar
  54. 54.
    Stewart PM, McIntyre MA, Edwards CR. The endocrinopathy of POEMS syndrome. Scott Med J 1989;34(5):520–2.PubMedGoogle Scholar
  55. 55.
    Nakamoto Y, Imai H, Yasuda T, Wakui H, Miura AB. A spectrum of clinicopatho-logical features of nephropathy associated with POEMS syndrome. Nephrol Dial Transplant 1999;14(10):2370–8.PubMedGoogle Scholar
  56. 56.
    Fukatsu A, Ito Y, Yuzawa Y, et al. A case of POEMS syndrome showing elevated serum interleukin 6 and abnormal expression of interleukin 6 in the kidney. Nephron 1992;62(1):47–51.PubMedGoogle Scholar
  57. 57.
    Mufti GJ, Hamblin TJ, Gordon J. Melphalan-induced pulmonary fibrosis in osteo-sclerotic myeloma [letter]. Acta Haematol 1983;69(2):140–1.PubMedGoogle Scholar
  58. 58.
    Iwasaki H, Ogawa K, Yoshida H, et al. Crow-Fukase syndrome associated with pulmonary hypertension. Intern Med 1993;32(7):556–60.PubMedGoogle Scholar
  59. 59.
    Lesprit P, Godeau B, Authier FJ, et al. Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 1998;157(3 Pt 1):907–11.PubMedGoogle Scholar
  60. 60.
    Dispenzieri A, Moreno-Aspitia A, Suarez GA,. Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature. Blood 2004;104(10):3400–7.PubMedGoogle Scholar
  61. 61.
    Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A. Pulmonary manifestations are common and associated with shortened survival in POEMS syndrome: a retrospective review of 141 patients. Submitted.Google Scholar
  62. 62.
    Lewerenz J, Gocht A, Hoeger PH, et al. Multiple vascular abnormalities and a paradoxical combination of vitamin B(12) deficiency and thrombocytosis in a case with POEMS syndrome. J Neurol 2003;250(12):1488–91.PubMedGoogle Scholar
  63. 63.
    Yishay O, Eran E. POEMS syndrome: failure of newly suggested diagnostic criteria to anticipate the development of the syndrome. Am J Hematol 2005;79(4):316–8.PubMedGoogle Scholar
  64. 64.
    Mineta M, Hatori M, Sano H, et al. Recurrent Crow-Fukase syndrome associated with increased serum levels of vascular endothelial growth factor: a case report and review of the literature. Tohoku J Exp Med 2006;210(3):269–77.PubMedGoogle Scholar
  65. 65.
    Morley JB, Schwieger AC. The relation between chronic polyneuropathy and osteosclerotic myeloma. J Neurol Neurosurg Psychiatry 1967;30(5):432–42.PubMedGoogle Scholar
  66. 66.
    Davis L, Drachman D. Myeloma neuropathy. Arch Neurol 1972;27:507–11.PubMedGoogle Scholar
  67. 67.
    Ku A, Lachmann E, Tunkel R, Nagler W. Severe polyneuropathy: initial manifestation of Castleman's disease associated with POEMS syndrome. Arch Phys Med Rehabil 1995;76(7):692–4.PubMedGoogle Scholar
  68. 68.
    Judge MR, McGibbon DH, Thompson RP. Angioendotheliomatosis associated with Castleman's lymphoma and POEMS syndrome. Clin Exp Dermatol 1993;18(4):360–2.PubMedGoogle Scholar
  69. 69.
    Kuwabara S, Hattori T, Shimoe Y, Kamitsukasa I. Long term melphalan-pred-nisolone chemotherapy for POEMS syndrome. J Neurol Neurosurg Psychiatry 1997;63(3):385–7.PubMedGoogle Scholar
  70. 70.
    Arima F, Dohmen K, Yamano Y, et al. [Five cases of Crow-Fukase syndrome]. [Japanese]. Fukuoka Igaku Zasshi Fukuoka Acta Medica 1992;83(2):112–20.Google Scholar
  71. 71.
    Wong VA, Wade NK. POEMS syndrome: an unusual cause of bilateral optic disk swelling. Am J Ophthalmol 1998;126(3):452–4.PubMedGoogle Scholar
  72. 72.
    Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA. Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation. Bone Marrow Transplant 2001;28(3):305–9.PubMedGoogle Scholar
  73. 73.
    Rovira M, Carreras E, Blade J, et al. Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation. Br J Haematol 2001;115(2):373–5.PubMedGoogle Scholar
  74. 74.
    Jaccard A, Royer B, Bordessoule D, Brouet JC, Fermand JP. High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome. Blood 2002;99(8):3057–9.PubMedGoogle Scholar
  75. 75.
    Peggs KS, Paneesha S, Kottaridis PD, et al. Peripheral blood stem cell transplantation for POEMS syndrome. Bone Marrow Transplant 2002;30(6):401–4.PubMedGoogle Scholar
  76. 76.
    Soubrier M, Ruivard M, Dubost JJ, Sauvezie B, Philippe P. Successful use of autol-ogous bone marrow transplantation in treating a patient with POEMS syndrome. Bone Marrow Transplant 2002;30(1):61–2.PubMedGoogle Scholar
  77. 77.
    Wiesmann A, Weissert R, Kanz L, Einsele H. Long-term follow-up on a patient with incomplete POEMS syndrome undergoing high-dose therapy and autologous blood stem cell transplantation. Blood 2002;100(7):2679–80.PubMedGoogle Scholar
  78. 78.
    Dispenzieri A, Kyle RA, Lacy MQ, et al. Superior survival in primary systemic amyloidosis patients undergoing peripheral blood stem cell transplantation: a case-control study. Blood 2004;103(10):3960–3.PubMedGoogle Scholar
  79. 79.
    Takai K, Niikuni K, Kurasaki T. [Successful treatment of POEMS syndrome with high-dose chemotherapy and autologous peripheral blood stem cell transplantation]. Rinsho Ketsueki 2004;45(10):1111–4.PubMedGoogle Scholar
  80. 80.
    Ganti AK, Pipinos I, Culcea E, Armitage JO, Tarantolo S. Successful hematopoie tic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. Am J Hematol 2005;79(3):206–10.PubMedGoogle Scholar
  81. 81.
    Kastritis E, Terpos E, Anagnostopoulos A, Xilouri I, Dimopoulos MA. Angiogenetic factors and biochemical markers of bone metabolism in POEMS syndrome treated with high-dose therapy and autologous stem cell support. Clin Lymphoma Myeloma 2006;7(1):73–6.PubMedGoogle Scholar
  82. 82.
    Kuwabara S, Misawa S, Kanai K, et al. Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology 2006;66(1):105–7.PubMedGoogle Scholar
  83. 83.
    Kojima H, Katsuoka Y, Katsura Y, et al. Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34 + purged stem cell rescue. Int J Hematol 2006;84(2):182–5.PubMedGoogle Scholar
  84. 84.
    Imai N, Kitamura E, Tachibana T, et al. Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy. Intern Med 2007;46(3):135–8.PubMedGoogle Scholar
  85. 85.
    Barrier JH, Le Noan H, Mussini JM, Brisseau JM. Stabilisation of a severe case of P.O.E.M.S. syndrome after tamoxifen administration [letter]. J Neurol Neurosurg Psychiatry 1989;52(2):286.PubMedGoogle Scholar
  86. 86.
    Matsui H, Udaka F, Kubori T, Oda M, Nishinaka K, Kameyama M. POEMS syndrome demonstrating VEGF decrease by ticlopidine. Intern Med 2004;43(11):1082–3.PubMedGoogle Scholar
  87. 87.
    Saida K, Kawakami H, Ohta M, Iwamura K. Coagulation and vascular abnormalities in Crow-Fukase syndrome. Muscle Nerve 1997;20(4):486–92.PubMedGoogle Scholar
  88. 88.
    Coto V, Auletta M, Oliviero U, et al. POEMS syndrome: an Italian case with diagnostic and therapeutic implications. Annali Italiani di Medicina Interna 1991;6(4):416–9.PubMedGoogle Scholar
  89. 89.
    Authier FJ, Belec L, Levy Y, et al. All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflamma-tory cytokines. Arthritis Rheum 1996;39(8):1423–6.PubMedGoogle Scholar
  90. 90.
    Sternberg AJ, Davies P, Macmillan C, Abdul-Cader A, Swart S. Strontium-89: a novel treatment for a case of osteosclerotic myeloma associated with life-threatening neuropathy. Br J Haematol 2002;118(3):821–4.PubMedGoogle Scholar
  91. 91.
    Kim SY, Lee SA, Ryoo HM, Lee KH, Hyun MS, Bae SH. Thalidomide for POEMS syndrome. Ann Hematol 2006;85(8):545–6.PubMedGoogle Scholar
  92. 92.
    Sinisalo M, Hietaharju A, Sauranen J, Wirta O. Thalidomide in POEMS syndrome: case report. Am J Hematol 2004;76(1):66–8.PubMedGoogle Scholar
  93. 93.
    Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood 2005;106(3):1135.PubMedGoogle Scholar
  94. 94.
    Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome. Blood 2006;107(12):4972–3; author reply 3–4.PubMedGoogle Scholar
  95. 95.
    Dispenzieri A, Klein CJ, Mauermann ML. Letter to the editor: lenalidomide therapy in a patient with POEMS syndrome. Blood In press.Google Scholar
  96. 96.
    Philips ED, el-Mahdi AM, Humphrey RL, Furlong MB, Jr. The effect of the radiation treatment on the polyneuropathy of multiple myeloma. J Can Assoc Radiol 1972;23(2):103–6.PubMedGoogle Scholar
  97. 97.
    Broussolle E, Vighetto A, Bancel B, Confavreux C, Pialat J, Aimard G. P. O.E.M.S. syndrome with complete recovery after treatment of a solitary plasmo-cytoma. Clin Neurol Neurosurg 1991;93(2):165–70.PubMedGoogle Scholar
  98. 98.
    Cabezas-Agricola JM, Lado-Abeal JJ, Otero-Anton E, Sanchez-Leira J, Cabezas-Cerrato J. Hypoparathyroidism in POEMS syndrome [letter]. Lancet 1996;347(9002):701–2.PubMedGoogle Scholar
  99. 99.
    Nakano A, Mitsui T, Endo I, Takeda Y, Ozaki S, Matsumoto T. Solitary plas-macytoma with VEGF overproduction: report of a patient with polyneuropathy. Neurology 2001;56(6):818–9.PubMedGoogle Scholar
  100. 100.
    Anonymous. Combination chemotherapy versus melphalan plus prednisone as treatment for multiple myeloma: an overview of 6,633 patients from 27 randomized trials. Myeloma Trialists' Collaborative Group. J Clin Oncol 1998;16(12):3832–42.Google Scholar
  101. 101.
    Satoh K, Miura I, Chubachi A, Utsumi S, Imai H, Miura AB. Development of secondary leukemia associated with (1;7)(q10;p10) in a patient with Crow-Fukase syndrome. Intern Med 1996;35(8):660–2.PubMedGoogle Scholar
  102. 102.
    Gertz MA, Lacy MQ, Dispenzieri A, et al. Stem cell transplantation for the management of primary systemic amyloidosis. Am J Med 2002;113(7):549–55.PubMedGoogle Scholar
  103. 103.
    Silberstein LE, Duggan D, Berkman EM. Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. J Clin Apher 1985;2(3):253–7.PubMedGoogle Scholar
  104. 104.
    Atsumi T, Kato K, Kurosawa S, Abe M, Fujisaku A. A case of Crow-Fukase syndrome with elevated soluble interleukin-6 receptor in cerebrospinal fluid. Response to double-filtration plasmapheresis and corticosteroids. Acta Haematol 1995;94(2):90–4.PubMedGoogle Scholar
  105. 105.
    Chang YJ, Huang CC, Chu CC. Intravenous immunoglobulin therapy in POEMS syndrome: a case report. Chung Hua i Hsueh Tsa Chih Chinese Medical Journal 1996;58(5):366–9.Google Scholar
  106. 106.
    Enevoldson TP, Harding AE. Improvement in the POEMS syndrome after administration of tamoxifen [letter]. J Neurol Neurosurg Psychiatry 1992;55(1):71–2.PubMedGoogle Scholar
  107. 107.
    Benito-Leon J, Lopez-Rios F, Rodriguez-Martin FJ, Madero S, Ruiz J. Rapidly deteriorating polyneuropathy associated with osteosclerotic myeloma responsive to intravenous immunoglobulin and radiotherapy. J Neurol Sci 1998 ; 158 (1) : 113–7.PubMedGoogle Scholar
  108. 108.
    Henze T, Krieger G. Combined high-dose 7S-IgG and dexamethasone is effective in severe polyneuropathy of the POEMS syndrome [letter] [see comments]. J Neurol 1995;242(7):482–3.PubMedGoogle Scholar
  109. 109.
    Rotta FT, Bradley WG. Marked improvement of severe polyneuropathy associated with multifocal osteosclerotic myeloma following surgery, radiation, and chemotherapy. Muscle Nerve 1997;20(8):1035–7.PubMedGoogle Scholar
  110. 110.
    Huang CC, Chu CC. Poor response to intravenous immunoglobulin therapy in patients with Castleman's disease and the POEMS syndrome [letter; comment]. J Neurol 1996;243(10):726–7.PubMedGoogle Scholar
  111. 111.
    Singhal S, Mehta J, Desikan R, et al. Antitumor activity of thalidomide in refractory multiple myeloma. N Engl J Med 1999;341(21):1565–71PubMedGoogle Scholar
  112. 112.
    Kyle RA, Therneau TM, Rajkumar SV, et al. A long-term study of prognosis in monoclonal gammopathy of undetermined significance. N Engl J Med 2002;346(8):564–9.PubMedGoogle Scholar
  113. 113.
    Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol 2003;121(5):749–57.Google Scholar
  114. 114.
    Kahn SN, Riches PG, Kohn J. Paraproteinaemia in neurological disease: incidence, associations, and classification of monoclonal immunoglobulins. J Clin Pathol 1980;33(7):617–21.PubMedGoogle Scholar
  115. 115.
    Kelly JJ, Jr., Kyle RA, O'Brien PC, Dyck PJ. Prevalence of monoclonal protein in peripheral neuropathy. Neurology 1981;31(11):1480–3.PubMedGoogle Scholar
  116. 116.
    Johansen P, Leegaard OF. Peripheral neuropathy and paraproteinemia: an immu-nohistochemical and serologic study. Clin Neuropathol 1985;4(3):99–104.PubMedGoogle Scholar
  117. 117.
    Isobe T, Osserman EF. Pathologic conditions associated with plasma cell dyscra-sias: a study of 806 cases. Ann N Y Acad Sci 1971;190:507–18.PubMedGoogle Scholar
  118. 118.
    Nobile-Orazio E, Barbieri S, Baldini L, et al. Peripheral neuropathy in monoclonal gammopathy of undetermined significance: prevalence and immunopathogenetic studies. Acta Neurol Scand 1992;85(6):383–90.PubMedGoogle Scholar
  119. 119.
    Vrethem M, Cruz M, Wen-Xin H, Malm C, Holmgren H, Ernerudh J. Clinical, neurophysiological and immunological evidence of polyneuropathy in patients with monoclonal gammopathies. J Neurol Sci 1993;114(2):193–9.PubMedGoogle Scholar
  120. 120.
    Baldini L, Nobile-Orazio E, Guffanti A, et al. Peripheral neuropathy in IgM monoclonal gammopathy and Waldenstrom's macroglobulinemia: a frequent complication in elderly males with low MAG-reactive serum monoclonal component. Am J Hematol 1994;45(1):25–31.PubMedGoogle Scholar
  121. 121.
    Latov N, Sherman WH, Nemni R, et al. Plasma-cell dyscrasia and peripheral neuropathy with a monoclonal antibody to peripheral-nerve myelin. N Engl J Med 1980;303(11):618–21.PubMedGoogle Scholar
  122. 122.
    Dellagi K, Dupouey P, Brouet JC, et al. Waldenstrom's macroglobulinemia and peripheral neuropathy: a clinical and immunologic study of 25 patients. Blood 1983;62(2):280–5.PubMedGoogle Scholar
  123. 123.
    Nemni R, Galassi G, Latov N, Sherman WH, Olarte MR, Hays AP. Polyneuropathy in nonmalignant IgM plasma cell dyscrasia: a morphological study. Ann Neurol 1983 ; 14 (1) : 43–54.PubMedGoogle Scholar
  124. 124.
    Latov N, Hays AP, Sherman WH. Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol 1988;3(4):301–32.PubMedGoogle Scholar
  125. 125.
    Quarles RH, Weiss MD. Autoantibodies associated with peripheral neuropathy. Muscle Nerve 1999;22(7):800–22.PubMedGoogle Scholar
  126. 126.
    Nobile-Orazio E, Francomano E, Daverio R, et al. Anti-myelin-associated glyco-protein IgM antibody titers in neuropathy associated with macroglobulinemia. Ann Neurol 1989;26(4):543–50.PubMedGoogle Scholar
  127. 127.
    Meucci N, Baldini L, Cappellari A, et al. Anti-myelin-associated glycoprotein antibodies predict the development of neuropathy in asymptomatic patients with IgM monoclonal gammopathy. Ann Neurol 1999;46(1):119–22.PubMedGoogle Scholar
  128. 128.
    Dispenzieri A, Kyle RA. Neurological aspects of multiple myeloma and related disorders. Best Pract Res Clin Haematol 2005;18(4):673–88.PubMedGoogle Scholar
  129. 129.
    Suarez GA, Kelly JJ, Jr. Polyneuropathy associated with monoclonal gammopa-thy of undetermined significance: further evidence that IgM-MGUS neuropathies are different than IgG-MGUS. Neurology 1993;43(7):1304–8.PubMedGoogle Scholar
  130. 130.
    Chassande B, Leger JM, Younes-Chennoufi AB, et al. Peripheral neuropathy associated with IgM monoclonal gammopathy: correlations between M-protein antibody activity and clinical/electrophysiological features in 40 cases. Muscle Nerve 1998;21(1):55–62.PubMedGoogle Scholar
  131. 131.
    Melmed C, Frail D, Duncan I, et al. Peripheral neuropathy with IgM kappa monoclonal immunoglobulin directed against myelin-associated glycoprotein. Neurology 1983;33(11):1397–405.PubMedGoogle Scholar
  132. 132.
    Kelly JJ, Adelman LS, Berkman E, Bhan I. Polyneuropathies associated with IgM monoclonal gammopathies. Arch Neurol 1988;45(12):1355–9.PubMedGoogle Scholar
  133. 133.
    Kelly JJ, Jr. The electrodiagnostic findings in polyneuropathies associated with IgM monoclonal gammopathies. Muscle Nerve 1990;13(12):1113–7.PubMedGoogle Scholar
  134. 134.
    Ilyas AA, Cook SD, Dalakas MC, Mithen FA. Anti-MAG IgM paraproteins from some patients with polyneuropathy associated with IgM paraproteinemia also react with sulfatide. J Neuroimmunol 1992;37(1–2):85–92.PubMedGoogle Scholar
  135. 135.
    Simmons Z, Albers JW, Bromberg MB, Feldman EL. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain 1995;118(Pt 2):359–68.PubMedGoogle Scholar
  136. 136.
    Notermans NC, Franssen H, Eurelings M, Van der Graaf Y, Wokke JH. Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopa-thy. Muscle Nerve 2000;23(1):73–9.PubMedGoogle Scholar
  137. 137.
    Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneu-ropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 1997;48(2):321–8.PubMedGoogle Scholar
  138. 138.
    Nobile-Orazio E, Meucci N, Baldini L, Di Troia A, Scarlato G. Long-term prognosis of neuropathy associated with anti-MAG IgM M- proteins and its relationship to immune therapies. Brain 2000;123(Pt 4):710–7.PubMedGoogle Scholar
  139. 139.
    Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N Engl J Med 1991;325(21):1482–6.PubMedGoogle Scholar
  140. 140.
    Oksenhendler E, Chevret S, Leger JM, Louboutin JP, Bussel A, Brouet JC. Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. IgM-associated Polyneuropathy Study Group. J Neurol Neurosurg Psychiatry 1995;59(3):243–7.PubMedGoogle Scholar
  141. 141.
    Mariette X, Chastang C, Clavelou P, Louboutin JP, Leger JM, Brouet JC. A randomised clinical trial comparing interferon-alpha and intravenous immunoglobu-lin in polyneuropathy associated with monoclonal IgM. The IgM-associated Polyneuropathy Study Group. J Neurol Neurosurg Psychiatry 1997;63(1):28–34.PubMedGoogle Scholar
  142. 142.
    Comi G, Roveri L, Swan A,. A randomised controlled trial of intravenous immu-noglobulin in IgM paraprotein associated demyelinating neuropathy. J Neurol 2002;249(10):1370–7.PubMedGoogle Scholar
  143. 143.
    Mariette X, Brouet JC, Chevret S, et al. A randomised double blind trial versus placebo does not confirm the benefit of alpha-interferon in polyneuropathy associated with monoclonal IgM [letter]. J Neurol Neurosurg Psychiatry 2000;69(2):279–80.PubMedGoogle Scholar
  144. 144.
    Dalakas MC, Quarles RH, Farrer RG, et al. A controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy. Ann Neurol 1996;40(5):792–5.PubMedGoogle Scholar
  145. 145.
    Sherman WH, Olarte MR, McKiernan G, Sweeney K, Latov N, Hays AP. Plasma exchange treatment of peripheral neuropathy associated with plasma cell dyscra-sia. J Neurol Neurosurg Psychiatry 1984;47(8):813–9.PubMedGoogle Scholar
  146. 146.
    Yeung KB, Thomas PK, King RH, et al. The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraprotein-aemia. Comparative clinical, immunological and nerve biopsy findings. J Neurol 1991;238(7):383–91.PubMedGoogle Scholar
  147. 147.
    Wilson HC, Lunn MP, Schey S, Hughes RA. Successful treatment of IgM paraproteinaemic neuropathy with fludarabine. J Neurol Neurosurg Psychiatry 1999;66(5):575–80.PubMedGoogle Scholar
  148. 148.
    Levine TD, Pestronk A. IgM antibody-related polyneuropathies: B-cell depletion chemotherapy using rituximab. Neurology 1999;52(8):1701–4.PubMedGoogle Scholar
  149. 149.
    Renaud S, Gregor M, Fuhr P. Rituximab in the treatment of polyneuropathy associated with anti-MAG antibodies. Muscle Nerve 2003; 27(5):611–5.PubMedGoogle Scholar
  150. 150.
    Pestronk A, Florence J, Miller T, Choksi R, Al-Lozi MT, Levine TD. Treatment of IgM antibody associated polyneuropathies using rituximab. J Neurol Neurosurg Psychiatry 2003;74(4):485–9.PubMedGoogle Scholar
  151. 151.
    Wintrobe M, Buell M. Hyperproteinemia associated with multiple myeloma. Bull Johns Hopkins Hosp 1933;52:156–165.Google Scholar
  152. 152.
    Lerner A, Watson C. Studies of cryoglobulins. Unusual purpura associated with the presence of a high concentration of cryoglobulin (cold precipitable serum globulin). Am J Med Sci 1947;214:410.PubMedGoogle Scholar
  153. 153.
    Grey HM, Kohler PF. Cryoimmunoglobulins. Semin Hematol 1973;10(2):87–112.PubMedGoogle Scholar
  154. 154.
    Meltzer M, Franklin EC. Cryoglobulinemia—a study of twenty-nine patients. I. IgG and IgM cryoglobulins and factors affecting cryoprecipitability. Am J Med 1966;40:828–56.PubMedGoogle Scholar
  155. 155.
    Meltzer M, Franklin EC, Elias K, McCluskey RT, Cooper N. Cryoglobulinemia— a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. Am J Med 1966;40(6):837–56.PubMedGoogle Scholar
  156. 156.
    Brouet J, Clauvel J, Danon F, Klein M, Seligmann M. Biological and clinical significance of cryoglobulins. A report of 86 cases. Am J Med 1974;57:775–88.PubMedGoogle Scholar
  157. 157.
    Gorevic PD, Kassab HJ, Levo Y, et al. Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am J Med 1980;69(2):287–308.PubMedGoogle Scholar
  158. 158.
    Dispenzieri A, Gorevic PD. Cryoglobulinemia. Hematol Oncol Clin North Am 1999;13(6):1315–49.PubMedGoogle Scholar
  159. 159.
    Monti G, Galli M, Invernizzi F, et al. Cryoglobulinaemias: a multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease. GISC. Italian Group for the Study of Cryoglobulinaemias. Qjm 1995;88(2):115–26.PubMedGoogle Scholar
  160. 160.
    Pascual M, Perrin L, Giostra E, Schifferli JA. Hepatitis C virus in patients with cryoglobulinemia type II. J Infect Dis 1990;162(2):569–70.PubMedGoogle Scholar
  161. 161.
    Ferri C, Greco F, Longombardo G, et al. Antibodies to hepatitis C virus in patients with mixed cryoglobulinemia. Arthritis Rheum 1991;34(12):1606–10.PubMedGoogle Scholar
  162. 162.
    Goldman M, Renversez JC, Lambert PH. Pathological expression of idiotypic interactions: immune complexes and cryoglobulins. [33 refs]. Springer Semin Immunopathol 1983;6(1):33–49.PubMedGoogle Scholar
  163. 163.
    Wilson MR, Arroyave CM, Miles L, Tan EM. Immune reactants in cryoproteins. Relationship to complement activation. Ann Rheum Dis 1977;36(6):540–8.PubMedGoogle Scholar
  164. 164.
    Agnello V, Chung R, Kaplan L. A role for hepatitis C virus infection in type II cryoglobulinemia. N Engl J Med 1992;327(21):1490–5.PubMedGoogle Scholar
  165. 165.
    Misiani R, Bellavita P, Fenili D, et al. Hepatitis C virus infection in patients with essential mixed cryoglobulinemia. Ann Intern Med 1992;117(7):573–7.PubMedGoogle Scholar
  166. 166.
    Pechere-Bertschi A, Perrin L, de Saussure P, Widmann JJ, Giostra E, Schifferli JA. Hepatitis C: a possible etiology for cryoglobulinaemia type II. Clin Exp Immunol 1992;89(3):419–22.Google Scholar
  167. 167.
    Bichard P, Ounanian A, Girard M, et al. High prevalence of hepatitis C virus RNA in the supernatant and the cryoprecipitate of patients with essential and secondary type II mixed cryoglobulinemia. J Hepatol 1994;21(1):58–63.PubMedGoogle Scholar
  168. 168.
    Cacoub P, Fabiani FL, Musset L, et al. Mixed cryoglobulinemia and hepatitis C virus. Am J Med 1994;96(2):124–32.PubMedGoogle Scholar
  169. 169.
    Munoz-Fernandez S, Barbado FJ, Martin Mola E, et al. Evidence of hepatitis C virus antibodies in the cryoprecipitate of patients with mixed cryoglobulinemia. J Rheumatol 1994;21(2):229–33.Google Scholar
  170. 170.
    Tanaka K, Aiyama T, Imai J, Morishita Y, Fukatsu T, Kakumu S. Serum cry-oglobulin and chronic hepatitis C virus disease among Japanese patients. Am J Gastroenterol 1995;90(10):1847–52.PubMedGoogle Scholar
  171. 171.
    Lunel F, Musset L, Cacoub P, et al. Cryoglobulinemia in chronic liver diseases: role of hepatitis C virus and liver damage. Gastroenterology 1994 ; 106 (5) : 1291–300.PubMedGoogle Scholar
  172. 172.
    Sansonno D, Iacobelli AR, Cornacchiulo V, et al. Immunochemical and biomo-lecular studies of circulating immune complexes isolated from patients with acute and chronic hepatitis C virus infection. Eur J Clin Invest 1996;26(6):465–75.PubMedGoogle Scholar
  173. 173.
    Invernizzi F, Galli M, Serino G, et al. Secondary and essential cryoglobulinemias. Frequency, nosological classification, and long-term follow-up. Acta Haematol 1983;70(2):73–82.PubMedGoogle Scholar
  174. 174.
    Osserman E. Plasma cell myeloma. N Eng J Med 1959;261:952–60.Google Scholar
  175. 175.
    Tarantino A, De Vecchi A, Montagnino G, et al. Renal disease in essential mixed cryoglobulinaemia. Long-term follow-up of 44 patients. Q J Med 1981;50:1.PubMedGoogle Scholar
  176. 176.
    Singer DR, Venning MC, Lockwood CM, Pusey CD. Cryoglobulinaemia: clinical features and response to treatment. Ann Med Interne 1986;137(3):251–3.Google Scholar
  177. 177.
    Monti G, Saccardo F, Pioltelli P, Rinaldi G. The natural history of cryoglob-ulinemia: symptoms at onset and during follow-up. A report by the Italian Group for the Study of Cryoglobulinemias (GISC). Clin Exp Rheumatol 1995;13(13):S129–33.PubMedGoogle Scholar
  178. 178.
    Ferri C, Sebastiani M, Giuggioli D, et al. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients. Semin Arthritis Rheum 2004;33(6):355–74.PubMedGoogle Scholar
  179. 179.
    Trejo O, Ramos-Casals M, Garcia-Carrasco M, et al. Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore) 2001;80(4):252–62.PubMedGoogle Scholar
  180. 180.
    Rieu V, Cohen P, Andre MH, et al. Characteristics and outcome of 49 patients with symptomatic cryoglobulinaemia. Rheumatology (Oxford) 2002;41(3):290–300.PubMedGoogle Scholar
  181. 181.
    Bryce AH, Kyle RA, Dispenzieri A, Gertz MA. Natural history and therapy of 66 patients with mixed cryoglobulinemia. Am J Hematol 2006;81(7):511–8.PubMedGoogle Scholar
  182. 182.
    Mendez P, Saeian K, Reddy KR, et al. Hepatitis C, cryoglobulinemia, and cutaneous vasculitis associated with unusual and serious manifestations. Am J Gastroenterol 2001;96(8):2489–93.PubMedGoogle Scholar
  183. 183.
    Donada C, Crucitti A, Donadon V, et al. Systemic manifestations and liver disease in patients with chronic hepatitis C and type II or III mixed cryoglobulinaemia. J Viral Hepat 1998;5(3):179–85.PubMedGoogle Scholar
  184. 184.
    Montagnino G. Reappraisal of the clinical expression of mixed cryoglobulinemia. Springer Semin Immunopathol 1988;10(1):1–19.PubMedGoogle Scholar
  185. 185.
    Cohen SJ, Pittelkow MR, Su WP. Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients. J Am Acad Dermatol 1991;25(1 Pt 1):21–7.PubMedGoogle Scholar
  186. 186.
    Gemignani F, Brindani F, Alfieri S, et al. Clinical spectrum of cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry 2005;76(10):1410–4.PubMedGoogle Scholar
  187. 187.
    Chad D, Pariser K, Bradley WG, Adelman LS, Pinn VW. The pathogenesis of cryoglobulinemic neuropathy. Neurology 1982;32(7):725–9.PubMedGoogle Scholar
  188. 188.
    Nemni R, Corbo M, Fazio R, Quattrini A, Comi G, Canal N. Cryoglobulinaemic neuropathy. A clinical, morphological and immunocytochemical study of 8 cases. Brain 1988;111(Pt 3):541–52.PubMedGoogle Scholar
  189. 189.
    Cavaletti G, Petruccioli MG, Crespi V, Pioltelli P, Marmiroli P, Tredici G. A clinico-pathological and follow up study of 10 cases of essential type II cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry 1990;53(10):886–9.PubMedGoogle Scholar
  190. 190.
    Khella SL, Frost S, Hermann GA, et al. Hepatitis C infection, cryoglobulinemia, and vasculitic neuropathy. Treatment with interferon alfa: case report and literature review. Neurology 1995;45(3 Pt 1):407–11.PubMedGoogle Scholar
  191. 191.
    Garcia-Bragado F, Fernandez JM, Navarro C, Villar M, Bonaventura I. Peripheral neuropathy in essential mixed cryoglobulinemia. Arch Neurol 1988;45(11):1210–4.Google Scholar
  192. 192.
    Valli G, De Vecchi A, Gaddi L, Nobile-Orazio E, Tarantino A, Barbieri S. Peripheral nervous system involvement in essential cryoglobulinemia and neph-ropathy. Clin Exp Rheumatol 1989;7(5):479–83.PubMedGoogle Scholar
  193. 193.
    Ammendola A, Sampaolo S, Ambrosone L, et al. Peripheral neuropathy in hepatitis-related mixed cryoglobulinemia: electrophysiologic follow-up study. Muscle Nerve 2005;31(3):382–5.PubMedGoogle Scholar
  194. 194.
    Casato M, Saadoun D, Marchetti A, et al. Central nervous system involvement in hepatitis C virus cryoglobulinemia vasculitis: a multicenter case-control study using magnetic resonance imaging and neuropsychological tests. J Rheumatol 2005;32(3):484–8.PubMedGoogle Scholar
  195. 195.
    Ferri C, La Civita L, Longombardo G, Zignego AL, Pasero G. Mixed cryoglob-ulinaemia: a cross-road between autoimmune and lymphoproliferative disorders. Lupus 1998;7(4):275–9.PubMedGoogle Scholar
  196. 196.
    Cordonnier D, Vialtel P, Renversez JC, et al. Renal diseases in 18 patients with mixed type II IgM-IgG cryoglobulinemia: monoclonal lymphoid infiltration (2 cases) and membranoproliferative glomerulonephritis (14 cases). Adv Nephrol Necker Hosp 1983;12:177–204.PubMedGoogle Scholar
  197. 197.
    D' Amico G. Renal involvement in hepatitis C infection: cryoglobulinemic glomerulonephritis. Kidney Int 1998;54(2):650–71.Google Scholar
  198. 198.
    Tarantino A, Campise M, Banfi G, et al. Long-term predictors of survival in essential mixed cryoglobulinemic glomerulonephritis. Kidney Int 1995;47(2):618–23.PubMedGoogle Scholar
  199. 199.
    Gorevic PD, Frangione B. Mixed cryoglobulinemia cross-reactive idiotypes: implications for the relationship of MC to rheumatic and lymphoproliferative diseases. Semin Hematol 1991;28(2):79–94.PubMedGoogle Scholar
  200. 200.
    Levo Y, Gorevic PD, Kassab HJ, Tobias H, Franklin EC. Liver involvement in the syndrome of mixed cryoglobulinemia. Ann Intern Med 1977;87(3):287–92.PubMedGoogle Scholar
  201. 201.
    Monteverde A, Ballare M, Bertoncelli MC, et al. Lymphoproliferation in type II mixed cryoglobulinemia. Clin Exp Rheumatol 1995;13(13):S141–7.PubMedGoogle Scholar
  202. 202.
    Monteverde A, Ballare M, Pileri S. Hepatic lymphoid aggregates in chronic hepatitis C and mixed cryoglobulinemia. Springer Semin Immunopathol 1997;19(1):99–110.PubMedGoogle Scholar
  203. 203.
    Tarantino A, Anelli A, Costantino A, De Vecchi A, Monti G, Massaro L. Serum complement pattern in essential mixed cryoglobulinaemia. Clin Exp Immunol 1978;32(1):77–85.PubMedGoogle Scholar
  204. 204.
    Ferri C, Moriconi L, Gremignai G, et al. Treatment of the renal involvement in mixed cryoglobulinemia with prolonged plasma exchange. Nephron 1986;43(4):246–53.PubMedGoogle Scholar
  205. 205.
    Frankel AH, Singer DR, Winearls CG, Evans DJ, Rees AJ, Pusey CD. Type II essential mixed cryoglobulinaemia: presentation, treatment and outcome in 13 patients. Q J Med 1992;82(298):101–24.PubMedGoogle Scholar
  206. 206.
    Ferri C, Marzo E, Longombardo G, et al. Interferon-alpha in mixed cryoglobuline-mia patients: a randomized, crossover-controlled trial. Blood 1993;81(5):1132–6.PubMedGoogle Scholar
  207. 207.
    Ferri C, Marzo E, Longombardo G, et al. Interferon alfa-2b in mixed cryoglobuli-naemia: a controlled crossover trial. Gut 1993;34(2 Suppl):S144–5.PubMedGoogle Scholar
  208. 208.
    Misiani R, Bellavita P, Fenili D, et al. Interferon alfa-2a therapy in cryoglobulinemia associated with hepatitis C virus. N Engl J Med 1994;330(11):751–6.PubMedGoogle Scholar
  209. 209.
    Dammacco F, Sansonno D, Han JH, et al. Natural interferon-alpha versus its combination with 6-methyl-prednisolone in the therapy of type II mixed cryoglobuline-mia: a long-term, randomized, controlled study. Blood 1994;84(10):3336–43.PubMedGoogle Scholar
  210. 210.
    Lauta VM, De Sangro MA. Long-term results regarding the use of recombinant interferon alpha-2b in the treatment of II type mixed essential cryoglobulinemia. Med Oncol 1995;12(4):223–30.PubMedGoogle Scholar
  211. 211.
    Mazzaro C, Lacchin T, Moretti M, et al. Effects of two different alpha-interferon regimens on clinical and virological findings in mixed cryoglobulinemia. Clin Exp Rheumatol 1995;13(13):S181–5.PubMedGoogle Scholar
  212. 212.
    Ferri C, Pietrogrande M, Cecchetti R, et al. Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia. Am J Med 1989;87(5):519–24.PubMedGoogle Scholar
  213. 213.
    Durand Cacoub P, Lunel-Fabiani F, et al. Ribavirin in hepatitis C related cryoglobulinemia. J Rheumatol 1998;25(6):1115–7.PubMedGoogle Scholar
  214. 214.
    Geltner D, Kohn RW, Gorevic P, Franklin EC. The effect of combination therapy (steroids, immunosuppressives, and plasmapheresis) on 5 mixed cryoglobuline-mia patients with renal, neurologic, and vascular involvement. Arthritis Rheum 1981;24(9):1121–7.PubMedGoogle Scholar
  215. 215.
    Valbonesi M, Montani F, Mosconi L, Florio G, Vecchi C. Plasmapheresis and cytotoxic drugs for mixed cryoglobulinemia. Haematologia 1984;17(3):341–51.PubMedGoogle Scholar
  216. 216.
    Sinico RA, Fornasieri A, Fiorini G, et al. Plasma exchange in the treatment of essential mixed cryoglobulinemia nephropathy. Long-term follow up. Int J Artif Organs 1985;2:15–8.Google Scholar
  217. 217.
    Berkman EM, Orlin JB. Use of plasmapheresis and partial plasma exchange in the management of patients with cryoglobulinemia. Transfusion 1980;20(2):171–8.PubMedGoogle Scholar
  218. 218.
    McLeod BC, Sassetti RJ. Plasmapheresis with return of cryoglobulin-depleted autologous plasma (cryoglobulinpheresis) in cryoglobulinemia. Blood 1980;55(5):866–70.PubMedGoogle Scholar
  219. 219.
    Reik L, Jr., Korn JH. Cryoglobulinemia with encephalopathy: successful treatment by plasma exchange. Ann Neurol 1981;10(5):488–90.PubMedGoogle Scholar
  220. 220.
    Ristow SC, Griner PF, Abraham GN, Shoulson I. Reversal of systemic manifestations of cryoglobulinemia. Ach Intern Med 1976;136:467–70.Google Scholar
  221. 221.
    Volpe R, Bruce-Robertson A, Fletcher A, et al. Essential cryoglobulinemia: review of the literature and report of a case treated with ACTH and cortisone. Am J Med 1956;20:533–53.PubMedGoogle Scholar
  222. 222.
    De Vecchi A, Montagnino G, Pozzi C, Tarantino A, Locatelli F, Ponticelli C. Intravenous methylprednisolone pulse therapy in essential mixed cryoglobuline-mia nephropathy. Clin Nephrol 1983;19(5):221–7.PubMedGoogle Scholar
  223. 223.
    Ponticelli C, Imbasciati E, Tarantino A, Pietrogrande M. Acute anuric glomerulone-phritis in monoclonal cryoglobulinaemia. Br Med J 1977;1(6066):948.Google Scholar
  224. 224.
    Mathison DA, Condemi JJ, Leddy JP, Callerame ML, Panner BJ, Vaughan JH. Purpura, arthralgia, and IgM-IgM cryoglobulinemia with rheumatoid fac tor acrivity. Response to cyclophosphamide and splenectomy. Ann Intern Med 1971;74(3):383–90.PubMedGoogle Scholar
  225. 225.
    Germain MJ, Anderson RW, Keane WF. Renal disease in cryoglobulinemia type II: response to therapy. A case report and review of the literature. Am J Nephrol 1982;2(4):221–6.PubMedGoogle Scholar
  226. 226.
    Quigg RJ, Brathwaite M, Gardner DF, Gretch DR, Ruddy S. Successful cyclo-phosphamide treatment of cryoglobulinemic membranoproliferative glomeru-lonephritis associated with hepatitis C virus infection. Am J Kidney Dis 1995;25(5):798–800.PubMedGoogle Scholar
  227. 227.
    Zaja F, De Vita S, Mazzaro C, et al. Efficacy and safety of rituximab in type II mixed cryoglobulinemia. Blood 2003;101(10):3827–34.PubMedGoogle Scholar
  228. 228.
    Sansonno D, De Re V, Lauletta G, Tucci FA, Boiocchi M, Dammacco F Monoclonal antibody treatment of mixed cryoglobulinemia resistant to interferon alpha with an anti-CD20. Blood 2003;101(10):3818–26.PubMedGoogle Scholar
  229. 229.
    Bryce AH, Dispenzieri A, Kyle RA, et al. Response to rituximab in patients with type II cryoglobulinemia. Clin Lymphoma Myeloma 2006;7(2):140–4.PubMedGoogle Scholar
  230. 230.
    Roccatello D, Baldovino S, Rossi D,. Long-term effects of anti-CD20 monoclonal antibody treatment of cryoglobulinaemic glomerulonephritis. Nephrol Dial Transplant 2004;19(12):3054–61.PubMedGoogle Scholar
  231. 231.
    Basse G, Ribes D, Kamar N, et al. Rituximab therapy for mixed cryoglobulinemia in seven renal transplant patients. Transplant Proc 2006;38(7):2308–10.PubMedGoogle Scholar
  232. 232.
    Lamprecht P, Lerin-Lozano C, Merz H, et al. Rituximab induces remission in refractory HCV associated cryoglobulinaemic vasculitis. Ann Rheum Dis 2003;62(12):1230–3.PubMedGoogle Scholar
  233. 233.
    Ghijsels E, Lerut E, Vanrenterghem Y, Kuypers D. Anti-CD20 monoclonal antibody (rituximab) treatment for hepatitis C-negative therapy-resistant essential mixed cryoglobulinemia with renal and cardiac failure. Am J Kidney Dis 2004;43(5):e34–8.PubMedGoogle Scholar
  234. 234.
    Sampson A, Callen JP. The cutting edge: thalidomide for type 1 cryoglobulinemic vasculopathy. Arch Dermatol 2006;142(8):972–4.PubMedGoogle Scholar
  235. 235.
    Cem Ar M, Soysal T, Hatemi G, Salihoglu A, Yazici H, Ulku B. Successful management of cryoglobulinemia-induced leukocytoclastic vasculitis with tha-lidomide in a patient with multiple myeloma. Ann Hematol 2005;84(9):609–13.Google Scholar
  236. 236.
    Ballare M, Bobbio F, Poggi G,. A pilot study on the effectiveness of cyclosporine in type II mixed cryoglobulinemia. Clin Exp Rheumatol 1995;13(13):S201–3.PubMedGoogle Scholar
  237. 237.
    Chandesris MO, Gayet S, Schleinitz N, Doudier B, Harle JR, Kaplanski G. Infliximab in the treatment of refractory vasculitis secondary to hepatitis C-associated mixed cryoglobulinaemia. Rheumatology (Oxford) 2004;43(4):532–3.Google Scholar
  238. 238.
    Monti G, Saccardo F, Rinaldi G, Petrozzino MR, Gomitoni A, Invernizzi F. Colchicine in the treatment of mixed cryoglobulinemia. Clin Exp Rheumatol 1995;13(13):S197–9.PubMedGoogle Scholar
  239. 239.
    Barton JC, Herrera GA, Galla JH, Bertoli LF, Work J, Koopman WJ. Acute cry-oglobulinemic renal failure after intravenous infusion of gamma globulin. Am J Med 1987;82(3 Spec No):624–9.PubMedGoogle Scholar
  240. 240.
    Boom BW, Brand A, Bavinck JN, Eernisse JG, Daha MR, Vermeer BJ. Severe leukocytoclastic vasculitis of the skin in a patient with essential mixed cryoglob-ulinemia treated with high-dose gamma-globulin intravenously. Arch Dermatol 1988;124(10):1550–3.PubMedGoogle Scholar
  241. 241.
    Enzenauer RJ, Judson PH. Type II mixed cryoglobulinemia treated with fludara-bine. J Rheumatol 1996;23(4):794–5.PubMedGoogle Scholar
  242. 242.
    Zaja F. Fludarabine in the treatment of essential mixed cryoglobulinaemia. Eur J Haematol 1996;57(3):259–60.PubMedGoogle Scholar
  243. 243.
    Odum J, D'Costa D, Freeth M, Taylor D, Smith N, MacWhannell A. Cryoglobulinaemic vasculitis caused by intravenous immunoglobulin treatment. Nephrol Dial Transplant 2001;16(2):403–6.PubMedGoogle Scholar
  244. 244.
    Migliaresi S, Tirri G. Interferon in the treatment of mixed cryoglobulinemia. Clin Exp Rheumatol 1995;13(13):S175–80.PubMedGoogle Scholar
  245. 245.
    Alric L, Plaisier E, Thebault S, et al. Influence of antiviral therapy in hepatitis C virus-associated cryoglobulinemic MPGN. Am J Kidney Dis 2004;43(4):617–23.PubMedGoogle Scholar
  246. 246.
    Zignego AL, Ferri C, Pileri SA, Caini P, Bianchi FB. Extrahepatic manifestations of hepatitis C virus infection: a general overview and guidelines for a clinical approach. Dig Liver Dis 2006.Google Scholar
  247. 247.
    Dispenzieri A. Symptomatic cryoglobulinemia. Curr Treat Options Oncol 1999;1(2):105–18.Google Scholar
  248. 248.
    Bruchfeld A, Lindahl K, Stahle L, Soderberg M, Schvarcz R. Interferon and ribavirin treatment in patients with hepatitis C-associated renal disease and renal insufficiency. Nephrol Dial Transplant 2003;18(8):1573–80.PubMedGoogle Scholar
  249. 249.
    Casato M, Lagana B, Antonelli G, Dianzani F, Bonomo L. Long-term results of therapy with interferon-alpha for type II essential mixed cryoglobulinemia. Blood 1991;78(12):3142–7.PubMedGoogle Scholar
  250. 250.
    Ohta S, Yokoyama H, Wada T, et al. Exacerbation of glomerulonephritis in subjects with chronic hepatitis C virus infection after interferon therapy. Am J Kidney Dis 1999;33(6):1040–8.PubMedGoogle Scholar
  251. 251.
    Beuthien W, Mellinghoff HU, Kempis J. Vasculitic complications of interferon-alpha treatment for chronic hepatitis C virus infection: case report and review of the literature. Clin Rheumatol 2005;24(5):507–15.PubMedGoogle Scholar
  252. 252.
    Cid MC, Hernandez-Rodriguez J, Robert J, et al. Interferon-alpha may exacerbate cryoblobulinemia-related ischemic manifestations: an adverse effect potentially related to its anti-angiogenic activity. Arthritis Rheum 1999;42(5):1051–5.PubMedGoogle Scholar
  253. 253.
    Cresta P, Musset L, Cacoub P, et al. Response to interferon alpha treatment and disappearance of cryoglobulinaemia in patients infected by hepatitis C virus [see comments]. Gut 1999;45(1):122–8.PubMedGoogle Scholar
  254. 254.
    Calleja JL, Albillos A, Moreno-Otero R, et al. Sustained response to interferon-alpha or to interferon-alpha plus ribavirin in hepatitis C virus-associated symptomatic mixed cryoglobulinaemia. Aliment Pharmacol Ther 1999;13(9):1179–86.PubMedGoogle Scholar
  255. 255.
    Zuckerman E, Keren D, Slobodin G, et al. Treatment of refractory, symptomatic, hepatitis C virus related mixed cryoglobulinemia with ribavirin and interferon-alpha. J Rheumatol 2000;27(9):2172–8.PubMedGoogle Scholar
  256. 256.
    Mazzaro C, Zorat F, Comar C, et al. Interferon plus ribavirin in patients with hepatitis C virus positive mixed cryoglobulinemia resistant to interferon. J Rheumatol 2003;30(8):1775–81.PubMedGoogle Scholar
  257. 257.
    Cacoub P, Lidove O, Maisonobe T, et al. Interferon-alpha and ribavirin treatment in patients with hepatitis C virus-related systemic vasculitis. Arthritis Rheum 2002;46(12):3317–26.PubMedGoogle Scholar
  258. 258.
    Donada C, Crucitti A, Donadon V, Chemello L, Alberti A. Interferon and ribavirin combination therapy in patients with chronic hepatitis C and mixed cryoglob-ulinemia. Blood 1998;92(8):2983–4.PubMedGoogle Scholar
  259. 259.
    Bonomo L, Casato M, Afeltra A, Caccavo D. Treatment of idiopathic mixed cryoglobulinemia with alpha interferon. Am J Med 1987;83(4):726–30.PubMedGoogle Scholar
  260. 260.
    Mazzaro C, Zorat F, Caizzi M, et al. Treatment with peg-interferon alfa-2b and ribavirin of hepatitis C virus-associated mixed cryoglobulinemia: a pilot study. J Hepatol 2005;42(5):632–8.PubMedGoogle Scholar
  261. 261.
    Saadoun D, Resche-Rigon M, Thibault V, Piette JC, Cacoub P. Antiviral therapy for hepatitis C virus-associated mixed cryoglobulinemia vasculitis: a long-term follow-up study. Arthritis Rheum 2006;54(11):3696–706.PubMedGoogle Scholar
  262. 262.
    Ghobrial IM, Uslan DZ, Call TG, Witzig TE, Gertz MA. Initial increase in the cryoglobulin level after rituximab therapy for type II cryoglobulinemia secondary to Waldenstr ö m macroglobulinemia does not indicate failure of response. Am J Hematol 2004;77(4):329–30.PubMedGoogle Scholar
  263. 263.
    Frickhofen N, Wiesneth M, Jainta C, et al. Hepatitis C virus infection is a risk factor for liver failure from veno-occlusive disease after bone marrow transplantation. Blood 1994;83(7):1998–2004.PubMedGoogle Scholar
  264. 264.
    Ubara Y, Hara S, Katori H, et al. Splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia. Am J Kidney Dis 2000;35(6):1186–92.PubMedGoogle Scholar
  265. 265.
    Cokonis Georgakis CD, Falasca G, Georgakis A, Heymann WR. Scleromyxedema. Clin Dermatol 2006;24(6):493–7.PubMedGoogle Scholar
  266. 266.
    Kucher C, Xu X, Pasha T, Elenitsas R. Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J Cutan Pathol 2005;32(7):484–90.PubMedGoogle Scholar
  267. 267.
    Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol 1995;33(1):37–43.PubMedGoogle Scholar
  268. 268.
    Berger JR, Dobbs MR, Terhune MH, Maragos WF. The neurologic complications of scleromyxedema. Medicine (Baltimore) 2001;80(5):313–9.Google Scholar
  269. 269.
    Pomann JJ, Rudner EJ. Scleromyxedema revisited. Int J Dermatol 2003;42(1):31–5.PubMedGoogle Scholar
  270. 270.
    Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxe-dematosus, and scleromyxedema. J Am Acad Dermatol 2001 ; 44 (2) : 273–81.PubMedGoogle Scholar
  271. 271.
    Lacy MQ, Hogan WJ, Gertz MA,. Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. Arch Dermatol 2005;141(10):1277–82.PubMedGoogle Scholar
  272. 272.
    Sansbury JC, Cocuroccia B, Jorizzo JL, Gubinelli E, Gisondi P, Girolomoni G. Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. J Am Acad Dermatol 2004;51(1):126–31.PubMedGoogle Scholar
  273. 273.
    Fernandez-Herrera J, Pedraz J. Necrobiotic xanthogranuloma. Semin Cutan Med Surg 2007;26(2):108–13.Google Scholar
  274. 274.
    Finan MC, Winkelmann RK. Necrobiotic xanthogranuloma with paraproteine-mia. A review of 22 cases. Medicine (Baltimore) 1986;65(6):376–88.Google Scholar
  275. 275.
    Bullock JD, Bartley GB, Campbell RJ, Yanes B, Connelly PJ, Funkhouser JW. Necrobiotic xanthogranuloma with paraproteinemia: case report and a pathoge-netic theory. Trans Am Ophthalmol Soc 1986;84:342–54.PubMedGoogle Scholar
  276. 276.
    Matsuura F, Yamashita S, Hirano K, et al. Activation of monocytes in vivo causes intracellular accumulation of lipoprotein-derived lipids and marked hypocholesterolemia—a possible pathogenesis of necrobiotic xanthogranuloma. Atherosclerosis 1999;142(2):355–65.PubMedGoogle Scholar
  277. 277.
    Venencie PY, Le Bras P, T oan ND, Tchernia G, Delfraissy JF. Recombinant inter- feron alfa-2b treatment of necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol 1995;32(4):666–7.PubMedGoogle Scholar
  278. 278.
    Goede JS, Misselwitz B, Taverna C, et al. Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation. Ann Hematol 2007;86(4):303–6.PubMedGoogle Scholar
  279. 279.
    Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore) 2001;80(1):37–44.Google Scholar
  280. 280.
    de Koning HD, Bodar EJ, van der Meer JW, Simon A. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007.Google Scholar
  281. 281.
    de Castro FR, Masouye I, Winkelmann RK, Saurat JH. Urticarial pathology in Schnitzler's (hyper-IgM) syndrome. Dermatology 1996;193(2):94–9.PubMedGoogle Scholar
  282. 282.
    Lipsker D, Spehner D, Drillien R, et al. Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions. Br J Dermatol 2000;142(5):954–9.PubMedGoogle Scholar
  283. 283.
    Schneider SW, Gaubitz M, Luger TA, Bonsmann G. Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol 2007;56(5 Suppl):S120–2.PubMedGoogle Scholar
  284. 284.
    Eiling E, Moller M, Kreiselmaier I, Brasch J, Schwarz T. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol 2007.Google Scholar
  285. 285.
    Martinez-Taboada VM, Fontalba A, Blanco R, Fernandez-Luna JL. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum 2005;52(7):2226–7.Google Scholar
  286. 286.
    Schnitzler L, Schubert B, Boasson M, Gardais J, Tourmen A. Urticaire chronique, lesions osseuses, macroglobulinemie IgM: maladie de Waldenstrom-IIe presentation. Bull Soc Fr Dermatol Syphiligr 1974;81:363.Google Scholar
  287. 287.
    Lipsker D, Imrie K, Simon A, Sullivan KE. Hot and hobbling with hives: Schnitzler syndrome. Clin Immunol 2006;119(2):131–4.PubMedGoogle Scholar
  288. 288.
    Randall RE, Williamson WC, Jr., Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med 1976;60(2):293–9.PubMedGoogle Scholar
  289. 289.
    Gallo G, Picken M, Frangione B, Buxbaum J. Nonamyloidotic monoclonal immu-noglobulin deposits lack amyloid P component. Mod Pathol 1988;1(6):453–6.PubMedGoogle Scholar
  290. 290.
    Kambham N, Markowitz GS, Appel GB, Kleiner MJ, Aucouturier P, D'Agati VD. Heavy chain deposition disease: the disease spectrum. Am J Kidney Dis 1999;33(5):954–62.PubMedGoogle Scholar
  291. 291.
    Vidal R, Goni F, Stevens F, et al. Somatic mutations of the L12a gene in V-kappa(1) light chain deposition disease: potential effects on aberrant protein conformation and deposition. Am J Pathol 1999;155(6):2009–17.PubMedGoogle Scholar
  292. 292.
    Gallo GR, Lazowski P, Kumar A, Vidal R, Baldwin DS, Buxbaum JN. Renal and cardiac manifestations of B-cell dyscrasias with nonamyloidotic monoclonal light chain and light and heavy chain deposition diseases. Adv Nephrol Necker Hosp 1998;28:355–82.PubMedGoogle Scholar
  293. 293.
    Keeling J, Teng J, Herrera GA. AL-amyloidosis and light-chain deposition disease light chains induce divergent phenotypic transformations of human mesang-ial cells. Lab Invest 2004;84(10):1322–38.PubMedGoogle Scholar
  294. 294.
    Pozzi C, Locatelli F. Kidney and liver involvement in monoclonal light chain disorders. Semin Nephrol 2002;22(4):319–30.PubMedGoogle Scholar
  295. 295.
    Bhargava P, Rushin JM, Rusnock EJ, et al. Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol 2007;31(2):267–76.PubMedGoogle Scholar
  296. 296.
    Pozzi C, D'Amico M, Fogazzi GB, et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis 2003;42(6):1154–63.PubMedGoogle Scholar
  297. 297.
    Heilman RL, Velosa JA, Holley KE, Offord KP, Kyle RA. Long-term follow-up and response to chemotherapy in patients with light-chain deposition disease. Am J Kidney Dis 1992;20(1):34–41.PubMedGoogle Scholar
  298. 298.
    Leung N, Lager DJ, Gertz MA, Wilson K, Kanakiriya S, Fervenza FC. Long-term outcome of renal transplantation in light-chain deposition disease. Am J Kidney Dis 2004;43(1):147–53.PubMedGoogle Scholar
  299. 299.
    Royer B, Arnulf B, Martinez F, et al. High dose chemotherapy in light chain or light and heavy chain deposition disease. Kidney Int 2004;65(2):642–8.PubMedGoogle Scholar
  300. 300.
    Weichman K, Dember LM, Prokaeva T, et al. Clinical and molecular characteristics of patients with non-amyloid light chain deposition disorders, and outcome following treatment with high-dose melphalan and autologous stem cell transplantation. Bone Marrow Transplant 2006;38(5):339–43.PubMedGoogle Scholar
  301. 301.
    Ma CX, Lacy MQ, Rompala JF, et al. Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma. Blood 2004;104(1):40–2.PubMedGoogle Scholar
  302. 302.
    Messiaen T, Deret S, Mougenot B, et al. Adult Fanconi syndrome secondary to light chain gammopathy. Clinicopathologic heterogeneity and unusual features in 11 patients. Medicine (Baltimore) 2000;79(3):135–54.Google Scholar
  303. 303.
    Aucouturier P, Bauwens M, Khamlichi AA, et al. Monoclonal Ig L chain and L chain V domain fragment crystallization in myeloma-associated Fanconi's syndrome. J Immunol 1993;150(8 Pt 1):3561–8.PubMedGoogle Scholar
  304. 304.
    Rocca A, Khamlichi AA, Touchard G, et al. Sequences of V kappa L subgroup light chains in Fanconi's syndrome. Light chain V region gene usage restriction and peculiarities in myeloma-associated Fanconi's syndrome. J Immunol 1995;155(6):3245–52.PubMedGoogle Scholar
  305. 305.
    Pronovost PH, Brady HR, Gunning ME, Espinoza O, Rennke HG. Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy. Nephrol Dial Transplant 1996;11(5):837–42.PubMedGoogle Scholar
  306. 306.
    Strom EH, Hurwitz N, Mayr AC, Krause PH, Mihatsch MJ. Immunotactoid-like glomerulopathy with massive fibrillary deposits in liver and bone marrow in monoclonal gammopathy. Am J Nephrol 1996;16(6):523–8.PubMedGoogle Scholar
  307. 307.
    Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D'Agati V D Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int 2003;63(4):1450–61.PubMedGoogle Scholar
  308. 308.
    Takemura T, Yoshioka K, Akano N, et al. Immunotactoid glomerulopathy in a child with Down syndrome. Pediatr Nephrol 1993;7(1):86–8.PubMedGoogle Scholar
  309. 309.
    Wahner-Roedler DL, Kyle RA. Mu-heavy chain disease: presentation as a benign monoclonal gammopathy. Am J Hematol 1992;40(1):56–60.Google Scholar
  310. 310.
    Wahner-Roedler DL, Witzig TE, Loehrer LL, Kyle RA. Gamma-heavy chain disease: review of 23 cases. Medicine (Baltimore) 2003;82(4):236–50.Google Scholar
  311. 311.
    Galanti LM, Doyen C, Vander Maelen C, et al. Biological diagnosis of a gamma-1-heavy chain disease in an asymptomatic patient. Eur J Haematol 1995;54(3):202–4.PubMedGoogle Scholar
  312. 312.
    Seijffers MJ, Levy M, Hermann G. Intractable watery diarrhea, hypokalemia, and malabsorption in a patient with Mediterranean type of abdominal lymphoma. Gastroenterology 1968;55(1):118–24.PubMedGoogle Scholar
  313. 313.
    Rambaud JC, Galian A, Matuchansky C, et al. Natural history of alpha-chain disease and the so-called Mediterranean lymphoma. Recent Results Cancer Res 1978;64:271–6.PubMedGoogle Scholar
  314. 314.
    Alpha-chain disease and related small-intestinal lymphoma: a memorandum. Bull World Health Organ 1976;54(6):615–24.Google Scholar
  315. 315.
    Al-Saleem T, Al-Mondhiry H. Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms. Blood 2005;105(6):2274–80.PubMedGoogle Scholar
  316. 316.
    Suarez F, Lortholary O, Hermine O, Lecuit M. Infection-associated lymphomas derived from marginal zone B cells: a model of antigen-driven lymphoprolifera-tion. Blood 2006;107(8):3034–44.PubMedGoogle Scholar
  317. 317.
    Loughran TP, Jr., Kadin ME, Deeg HJ. T-cell intestinal lymphoma associated with celiac sprue. Ann Intern Med 1986;104(1):44–7.PubMedGoogle Scholar
  318. 318.
    Vaiphei K, Kumari N, Sinha SK, et al. Roles of syndecan-1, bcl6 and p53 in diagnosis and prognostication of immunoproliferative small intestinal disease. World J Gastroenterol 2006;12(22):3602–8.PubMedGoogle Scholar
  319. 319.
    Fischbach W, Tacke W, Greiner A, Konrad H, Muller H. Regression of immu-noproliferative small intestinal disease after eradication of Helicobacter pylori. Lancet 1997;349(9044):31–2.PubMedGoogle Scholar
  320. 320.
    Lecuit M, Abachin E, Martin A, et al. Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med 2004;350(3):239–48.PubMedGoogle Scholar
  321. 321.
    Fine KD, Stone MJ. Alpha-heavy chain disease, Mediterranean lymphoma, and immunoproliferative small intestinal disease: a review of clinicopatho-logical features, pathogenesis, and differential diagnosis. Am J Gastroenterol 1999;94(5):1139–52.PubMedGoogle Scholar
  322. 322.
    Rambaud JC, Halphen M, Galian A, Tsapis A. Immunoproliferative small intestinal disease (IPSID): relationships with alpha-chain disease and “Mediterranean” lymphomas. Springer Semin Immunopathol 1990;12(2–3):239–50.PubMedGoogle Scholar
  323. 323.
    Goossens T, Klein U, Kuppers R. Frequent occurrence of deletions and duplications during somatic hypermutation: implications for oncogene translocations and heavy chain disease. Proc Natl Acad Sci U S A 1998;95(5):2463–8.PubMedGoogle Scholar
  324. 324.
    Galian A, Lecestre MJ, Scotto J, Bognel C, Matuchansky C, Rambaud JC. Pathological study of alpha-chain disease, with special emphasis on evolution. Cancer 1977;39(5):2081–101.PubMedGoogle Scholar
  325. 325.
    Mir-Madjlessi SH, Mir-Ahmadian M. Alpha-chain disease—a report of eleven patients from Iran. J Trop Med Hyg 1979;82(11–12):229–36.Google Scholar
  326. 326.
    Zamir A, Parasher G, Moukarzel AA, Guarini L, Zeien L, Feldman F. Immunoproliferative small intestinal disease in a 16-Year-old boy presenting as severe malabsorption with excellent response to tetracycline treatment. J Clin Gastroenterol 1998;27(1):85–9.PubMedGoogle Scholar
  327. 327.
    Martin IG, Aldoori MI. Immunoproliferative small intestinal disease: Mediterranean lymphoma and alpha heavy chain disease. Br J Surg 1994;81(1):20–4.PubMedGoogle Scholar
  328. 328.
    Salimi M, Spinelli JJ. Chemotherapy of Mediterranean abdominal lymphoma. Retrospective comparison of chemotherapy protocols in Iranian patients. Am J Clin Oncol 1996;19(1):18–22.PubMedGoogle Scholar
  329. 329.
    Ben-Ayed F, Halphen M, Najjar T, et al. Treatment of alpha chain disease. Results of a prospective study in 21 Tunisian patients by the Tunisian-French intestinal Lymphoma Study Group. Cancer 1989;63(7):1251–6.Google Scholar
  330. 330.
    Akbulut H, Soykan I, Yakaryilmaz F, et al. Five-Year results of the treatment of 23 patients with immunoproliferative small intestinal disease: a Turkish experience. Cancer 1997;80(1):8–14.PubMedGoogle Scholar
  331. 331.
    Price SK. Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease. Histopathology 1990;17(1):7–17.PubMedGoogle Scholar
  332. 332.
    Shih LY, Liaw SJ, Hsueh S, Kuo TT. Alpha-chain disease. Report of a case from Taiwan. Cancer 1987;59(3):545–8.PubMedGoogle Scholar
  333. 333.
    Malik IA, Shamsi Z, Shafquat A, et al. Clinicopathological features and management of immunoproliferative small intestinal disease and primary small intestinal lymphoma in Pakistan. Med Pediatr Oncol 1995;25(5):400–6.PubMedGoogle Scholar
  334. 334.
    McLaughlin P, Grillo-Lopez AJ, Link BK, et al. Rituximab chimeric anti-CD20 monoclonal antibody therapy for relapsed indolent lymphoma: half of patients respond to a four-dose treatment program. J Clin Oncol 1998;16(8):2825–33.PubMedGoogle Scholar
  335. 335.
    Franklin EC, Lowenstein J, Bigelow B, Meltzer M. Heavy chain disease—a new disorder of serum γ-globulins: report of the first case. Am J Med 1964;37:332.PubMedGoogle Scholar
  336. 336.
    Kyle RA, Greipp PR, Banks PM. The diverse picture of gamma heavy-chain disease. Report of seven cases and review of literature. Mayo Clin Proc 1981;56(7):439–51.PubMedGoogle Scholar
  337. 337.
    Fermand JP, Brouet JC. Heavy-chain diseases. Hematol Oncol Clin North Am 1999;13(6):1281–94.PubMedGoogle Scholar
  338. 338.
    Lassoued K, Picard C, Danon F, et al. Cutaneous manifestations associated with gamma heavy chain disease. Report of an unusual case and review of literature. J Am Acad Dermatol 1990;23(5 Pt 2):988–91.PubMedGoogle Scholar
  339. 339.
    Zawadzki ZA, Benedek TG, Ein D, Easton JM. Rheumatoid arthritis terminating in heavy-chain disease. Ann Intern Med 1969;70(2):335–47.PubMedGoogle Scholar
  340. 340.
    Jacqueline F, Renversez JC, Groslambert P, Fine JM. Gamma heavy chain disease during rheumatoid polyarthritis. A clinical and immunochemical study [French]. Rev Rhum Mal Osteoartic 1978;45(11):661–5.PubMedGoogle Scholar
  341. 341.
    Gaucher A, Bertrand F, Brouet JC, et al. Gamma heavy chain disease associated with rheumatoid arthritis. Spontaneous disappearance of the pathologic protein [French]. Sem Hop 1977;53(38):2117–20.PubMedGoogle Scholar
  342. 342.
    Westin J, Eyrich R, Falsen E, et al. Gamma heavy chain disease. Reports of three patients. Acta Med Scand 1972;192(4):281–92.PubMedGoogle Scholar
  343. 343.
    Lyons RM, Chaplin H, Tillack TW, Majerus PW. Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone. Blood 1975;46(1):1–9.PubMedGoogle Scholar
  344. 344.
    Wager O, Rasanen JA, Lindeberg L, Makela V. Two cases of IgG heavy-chain disease. Acta Pathol Microbiol Scand 1969;75(2):350–2.PubMedGoogle Scholar
  345. 345.
    Frangione B, Franklin EC. Heavy chain diseases: clinical features and molecular significance of the disordered immunoglobulin structure. Semin Hematol 1973;10(1):53–64.PubMedGoogle Scholar
  346. 346.
    Fermand JP, Brouet JC, Danon F, Seligmann M. Gamma heavy chain “disease”: heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature. Medicine (Baltimore) 1989;68(6):321–35.Google Scholar
  347. 347.
    Wester SM, Banks PM, Li CY. The histopathology of gamma heavy-chain disease. Am J Clin Pathol 1982;78(4):427–36.PubMedGoogle Scholar
  348. 348.
    Forte FA, Prelli F, Yount WJ, et al. Heavy chain disease of the gamma (gamma M) type: report of the first case. Blood 1970;36(2):137–44.PubMedGoogle Scholar
  349. 349.
    Ballard HS, Hamilton LM, Marcus AJ, Illes CH. A new variant of heavy-chain disease (mu-chain disease). N Engl J Med 1970;282(19):1060–2.PubMedGoogle Scholar
  350. 350.
    Wahner-Roedler DL, Kyle RA. Heavy chain diseases. Best Pract Res Clin Haematol 2005;18(4):729–46.Google Scholar
  351. 351.
    Pruzanski W, Hasselback R, Katz A, Parr DM. Multiple myeloma (light chain disease) with rheumatoid-like amyloid arthropathy and mu-heavy chain fragment in the serum. Am J Med 1978;65(2):334–41.PubMedGoogle Scholar
  352. 352.
    Mihaesco E, Barnikol-Watanabe S, Barnikol HU, Mihaesco C, Hilschmann N. The primary structure of the constant part of mu-chain-disease protein BOT. Eur J Biochem 1980;111(1):275–86.PubMedGoogle Scholar
  353. 353.
    Brouet JC, Seligmann M, Danon F, Belpomme D, Fine JM. mu-chain disease. Report of two new cases. Arch Intern Med 1979;139(6):672–4.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Angela Dispenzieri
    • 1
  1. 1.Division of Hematology, Mayo ClinicRochesterUSA

Personalised recommendations