Plasma Cell Dyscrasias: Definition and Diagnostic Evaluation

  • Robert A KyleJr.
Part of the Topics in the Neurosciences book series (TNSC, volume 5)


The plasma cell dyscrasias (table 1–1), which are synonymous with monoclonal gammopathies, are characterized by proliferation of a single clone of plasma cells which produces a homogeneous monoclonal (M) protein. Each monoclonal protein consists of two heavy polypeptide chains of the same class and subclass and two light polypeptide chains of the same type. In contrast, polyclonal immunoglobulins consist of one or more heavy-chain classes and both light-chain types.


Multiple Myeloma Familial Mediterranean Fever Carpal Tunnel Syndrome Spinal Muscular Atrophy Amyloid Fibril 
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  1. 1.
    Aksoy M, Erdem S, Dincol G, Kutlar A, Bakioğlu I, Hcpyükscl T: Clinical observations showing the role of some factors in the etiology of multiple myeloma: A study in 7 patients. Acta Haematol (Basel) 71:116–120, 1984.CrossRefGoogle Scholar
  2. 2.
    Alexanian R, Barlogie B, Fritsche H: Beta2 microglobulin in multiple myeloma. Am J Hematol 20:345–351, 1985.PubMedCrossRefGoogle Scholar
  3. 3.
    Andersson R: Familial amyloidosis with polyneuropathy: A clinical study based on patients living in Northern Sweden. Acta Med Scand (suppl) 590:1–64, 1976.Google Scholar
  4. 4.
    Andrade C: A peculiar form of peripheral neuropathy: Familial atypical generalized amyloidosis with special involvement of the peripheral nerves. Brain 75:408–427, 1952.PubMedCrossRefGoogle Scholar
  5. 5.
    Barlogie B, Smith L, Alexanian R: Effective treatment of advanced multiple myeloma refractory to alkylating agents. N Engl J Med 310:1353–1356, 1984.PubMedCrossRefGoogle Scholar
  6. 6.
    Benson MD, Cohen AS; Generalized amyloid in a family of Swedish origin: A study of 426 family members in seven generations of a new kinship with neuropathy, nephropathy, and central nervous system involvement. Ann Intern Med 86:419–424, 1977.PubMedGoogle Scholar
  7. 7.
    Bloch KJ, Maki DG: Hyperviscosity syndromes associated with immunoglobulin abnormalities. Semin Hematol 10:113–124, 1973.PubMedGoogle Scholar
  8. 8.
    Broder S, Waldmann TA: The suppressor-cell network in cancer. N Engl J Med 299:1281–1284; 1335–1341, 1978.PubMedCrossRefGoogle Scholar
  9. 9.
    Bruckman JE, Bloomer WD: Management of spinal cord compression. Semin Oncol 5:135–140, 1978.PubMedGoogle Scholar
  10. 10.
    Carmagnola AL, Boccadoro M, Massaia M, Pileri A: The idiotypic specificities of lymphocytes in human monoclonal gammopathies: Analysis with the fluorescence activated cell sorter. Clin Exp Immunol 51:173–177, 1983.PubMedGoogle Scholar
  11. 11.
    Cohen AS, Rubinow A, Kayne H, Skinner M: The life span of patients with primary (AL) amyloid: Treated with colchicine (1976–1984) and untreated (before 1976) (abstract). IVth International Symposium on Amyloidosis, New York, College of Physicians and Surgeons, Columbia University, 1984a.Google Scholar
  12. 12.
    Cohen DJ, Sherman WH, Osserman EF, Appel GB: Acute renal failure in patients with multiple myeloma. Am J Med 76: 247–256, 1984b.PubMedCrossRefGoogle Scholar
  13. 13.
    Coppeto, JR, Monteiro MLR, Collias J, Uphoff D, Bear L: Foster-Kennedy syndrome caused by solitary intracranial plasmacytoma. Surg Neurol 19:267–272, 1983.PubMedCrossRefGoogle Scholar
  14. 14.
    Cosgrove GR, Leblanc R, Meagher-Villemure K, Ethier R: Cerebral amyloid angiopathy. Neurology 35:625–631, 1985.PubMedGoogle Scholar
  15. 15.
    Cuzick J: Radiation-induced myelomatosis. N Engl J Med 304:204–210, 1981.PubMedCrossRefGoogle Scholar
  16. 16.
    Deuel TF, Davis P, Avioli LV: Waldenström’s macroglobulinemia. Arch Intern Med 143:986–988, 1983.PubMedCrossRefGoogle Scholar
  17. 17.
    Durie BGM, Salmon SE: A clinical staging system for multiple myeloma: Correlation of measured myeloma cell mass with presenting clinical features, response to treatment, and survival. Cancer 36:842–854, 1975.PubMedCrossRefGoogle Scholar
  18. 18.
    Durie BGM, Salmon SE, Moon TE: Prctreatmcnt tumor mass, cell kinetics, and prognosis in multiple myeloma. Blood 55:364–372, 1980.PubMedGoogle Scholar
  19. 19.
    Durie BGM, Pcrsky B, Soehnlen BJ, Grogan TM, Salmon SE: Amyloid production in human myeloma stem-cell culture, with morphologic evidence of amyloid secretion by associated macrophages. N Engl J Med 307:1689–1692, 1982.PubMedCrossRefGoogle Scholar
  20. 20.
    Fahey JL, McKelvey EM: Quantitative determination of serum immunoglobulins in antibody-agar plates. J Immunol 94:84–90, 1965.PubMedGoogle Scholar
  21. 21.
    Frederiksen T, Gøtzschc H, Harboe N, Kiær W, Mellcmgaard K: Familial primary amyloidosis with severe amyloid heart disease. Am J Med 33:328–348, 1962.PubMedCrossRefGoogle Scholar
  22. 22.
    Gonchoroff NJ, Greipp PR, Kyle RA, Katzmann JA: A monoclonal antibody reactive with 5-bromo-2-dcoxyuridine that does not require DNA dcnaturation. Cytometry 6:506–512, 1985.PubMedCrossRefGoogle Scholar
  23. 23.
    Greipp PR, Kyle RA: Clinical, morphological, and cell kinetic differences among multiple myeloma, monoclonal gammopathy of undetermined significance, and smoldering multiple myeloma. Blood 62:166–171, 1983.PubMedGoogle Scholar
  24. 24.
    Harper L, LeBlanc HJ, McDowell JR: Intracranial extension and spontaneous hemorrhage of a sphenoid plasmacytoma. Neurosurgery 11:797–799, 1982.PubMedCrossRefGoogle Scholar
  25. 25.
    Hill GS, Morel-Maroger L, Méry J-P, Brouet JC, Mignon F: Renal lesions in multiple myeloma: Their relationship to associated protein abnormalities. Am J Kidney Dis 2:423–438, 1983.PubMedGoogle Scholar
  26. 26.
    Hinberg IH, Katz L, Waddell L: Sensitivity of in vitro diagnostic dipstick tests to urinary protein. Clin Biochem 11:62–64, 1978.PubMedCrossRefGoogle Scholar
  27. 27.
    Jordan DR, Drouin J, Berry G, Watson AG: Intracranial plasmacytoma associated with multiple myeloma. Can J Ophthalmol 19:275–278, 1984.PubMedGoogle Scholar
  28. 28.
    Kintzer JS Jr, Rosenow EC III, Kyle RA: Thoracic and pulmonary abnormalities in multiple myeloma: A review of 958 cases. Arch Intern Med 138:727–730, 1978.PubMedCrossRefGoogle Scholar
  29. 29.
    Knowling MA, Harwood AR, Bergsagel DE: Comparison of extramedullary plasmacytomas with solitary and multiple plasma cell tumors of bone. J Clin Oncol 1:255–262, 1983.PubMedGoogle Scholar
  30. 30.
    Kohn J: A cellulose acetate supporting medium for zone electrophoresis. Clin Chim Acta 2:297–303, 1957.PubMedCrossRefGoogle Scholar
  31. 31.
    Kyle RA: Multiple myeloma: Review of 869 cases. Mayo Clin Proc 50:29–40, 1975.PubMedGoogle Scholar
  32. 32.
    Kyle RA: “Benign” monoclonal gammopathy: A misnomer? JAMA 251:1849–1854, 1984a.PubMedCrossRefGoogle Scholar
  33. 33.
    Kyle RA: Second malignancies associated with chemotherapy. In: Toxicity of Chemotherapy, MC Perry, JW Yarbro (eds). New York: Grune & Stratton, p 479–506, 1984b.Google Scholar
  34. 34.
    Kyle RA: Amyloidosis. In: Neoplastic Diseases of the Blood (vol 2), PH Wiernik, GP Canellos, RA Kyle, CA Schiffer (eds). New York: Churchill Livingstone, p 607–651, 1985.Google Scholar
  35. 35.
    Kyle RA: Classification and diagnosis of monoclonal gammopathies. In: Manual of Clinical Laboratory Immunology 3rd, NR Rose, H Friedman, JL Fahey (eds). Washington DC: American Society for Microbiology, p. 152–167, 1986.Google Scholar
  36. 36.
    Kyle RA, Greipp PR: Smoldering multiple myeloma. N Engl J Med 302:1347–1349, 1980.PubMedCrossRefGoogle Scholar
  37. 37.
    Kyle RA, Greipp PR: Amyloidosis (AL): Clinical and laboratory features in 229 cases. Mayo Clin Proc 58:665–683, 1983.PubMedGoogle Scholar
  38. 38.
    Kyle RA, Greipp PR, Garton JP, Gertz MA: Primary systemic amyloidosis: Comparison of melphalan/prednisone versus colchicine. Am J Med 79:708–716, 1985a.PubMedCrossRefGoogle Scholar
  39. 39.
    Kyle RA, Greipp PR, O’Fallon WM: Primary systemic amyloidosis: Multivariate analysis for prognostic factors in 168 cases. Blood 68:220–229, 1986.PubMedGoogle Scholar
  40. 40.
    Kyle RA, Robinson RA, Katzmann JA: The clinical aspects of biclonal gammopathies. Review of 57 cases. Am J Med 71:999–1008, 1981.PubMedCrossRefGoogle Scholar
  41. 41.
    Kyle RA, Schreiman JS, McLeod RA, Beabout JW: Computed tomography in diagnosis and management of multiple myeloma and its variants. Arch Intern Med 145:1451–1452, 1985b.PubMedCrossRefGoogle Scholar
  42. 42.
    Lanham JG, Meltzer ML, De Beer FC, Hughes GRV, Pepys MB: Familial amyloidosis of Ostertag. Q J Med 51:25–32, 1982.PubMedGoogle Scholar
  43. 43.
    Lee BJ, Lake-Lewin D, Myers JE: Intensive treatment of multiple myeloma. In: Controversies in Oncology, PH Wiernik (ed). New York: John Wiley & Sons, p 61–79, 1982.Google Scholar
  44. 44.
    Libbey CA, Rubinow A, Shirahama T, Deal C, Cohen AS: Familial amyloid polyneuropathy: Demonstration of prealbumin in a kinship of German/English ancestry with onset in the seventh decade. Am J Med 76:18–24, 1984.PubMedCrossRefGoogle Scholar
  45. 45.
    Linos A, Kyle RA, O’Fallon WM, Kurland LT: Incidence and secular trend of multiple myeloma in Olmsted County, Minnesota: 1965–77. J Natl Cancer Inst 66:17–20, 1981.PubMedGoogle Scholar
  46. 46.
    Longsworth LG, Shedlovsky T, Maclnnes DA: Electrophoretic patterns of normal and pathological human blood serum and plasma. J Exp Med 70:399–413, 1939.PubMedCrossRefGoogle Scholar
  47. 47.
    Lyon LW, McCormick WF, Schochet SS Jr: Progressive multifocal leukoencephalopathy. Arch Intern Med 128:420–426, 1971.PubMedCrossRefGoogle Scholar
  48. 48.
    Maldonado JE, Kyle RA: Familial myeloma: Report of eight families and a study of serum proteins in their relatives. Am J Med 57:875–884, 1974.PubMedCrossRefGoogle Scholar
  49. 49.
    Mancardi GL, Mandybur TI: Solitary intracranial plasmacytoma. Cancer 51:2226–2233, 1983.PubMedCrossRefGoogle Scholar
  50. 50.
    McAnena OJ, Feely MP, Kealy WF: Spinal cord compression by amyloid tissue. J Neurol Neurosurg Psychiatry 45:1067–1069, 1982.PubMedCrossRefGoogle Scholar
  51. 51.
    Mills KHG, Cawley JC: Abnormal monoclonal antibody-defined helper/suppressor T-cell subpopulations in multiple myeloma: Relationship to treatment and clinical stage. Br J Haematol 53:271–275, 1983.PubMedCrossRefGoogle Scholar
  52. 52.
    Mizutani T, Morimatsu Y, Hayakawa K: Necrotizing leukoencephalopathy and treated multiple myeloma: An autopsy case without intrathecal chemotherapy or irradiation of the brain. Acta Pathol Jpn 34:655–662, 1984.PubMedGoogle Scholar
  53. 53.
    Moreno AJ, Brown JM, Brown TJ, Graham GD, Yedinak MA: Scintigraphic findings in a primary cerebral amyloidoma. Clin Nucl Med 8:528–530, 1983.PubMedCrossRefGoogle Scholar
  54. 54.
    Oken MM, Tsiatis A, Abramson N, Glick J: Comparison of standard (MP) with intensive (VBMCP) therapy for the treatment of multiple myeloma (MM) (abstract). Proc Am Soc Clin Oncol 3:270, 1984.Google Scholar
  55. 55.
    Papadimitriou CS, Schwarze EW: Extramedullary nongastrointestinal plasmacytoma: An immunohistochemical study of 16 cases. Pathol Res Pract 176:306–312, 1983.PubMedGoogle Scholar
  56. 56.
    Perri RT, Hebbel RP, Oken MM: Influence of treatment and response status on infection risk in multiple myeloma. Am J Med 71:935–940, 1981.PubMedCrossRefGoogle Scholar
  57. 57.
    Peters HA, Clatanoff DV: Spinal muscular atrophy secondary to macroglobulinemia: Reversal of symptoms with chlorambucil therapy. Neurology (Minneap) 18:101–108, 1968.Google Scholar
  58. 58.
    Platia EV, Saral R: Deafness and Waldenström’s macroglobulinemia. South Med J 72:1495–1496, 1979.PubMedCrossRefGoogle Scholar
  59. 59.
    Pritchard PB III, Martinez RA, Hungerford GD, Powers JM, Perot PL Jr: Dural plasmacytoma. Neurosurgery 12:576–570, 1983.PubMedCrossRefGoogle Scholar
  60. 60.
    Pruzanski W, Baron M, Shupak R: Neuroarthropathy (Charcot joints) in familial amyloid polyneuropathy. J Rheumatol 8:477–481, 1981.PubMedGoogle Scholar
  61. 61.
    Rukavina JG, Block WD, Curtis AC: Familial primary systemic amyloidosis: An experimental, genetic, and clinical study. J Invest Dermatol 27:111–131, 1956a.PubMedGoogle Scholar
  62. 62.
    Rukavina JG, Block WD, Jackson CE, Falls HF, Carey JH, Curtis AC: Primary systemic amyloidosis: A review and an experimental, genetic, and clinical study of 29 cases with particular emphasis on the familial form. Medicine (Baltimore) 35:239–334, 1956b.Google Scholar
  63. 63.
    Saraiva MJM, Costa PP, Goodman DS: Biochemical marker in familial amyloidotic polyneuropathy, Portuguese type: Family studies on the transthyretin (prealbumin)-meth-ionine-30 variant. J Clin Invest 76:2171–2177, 1985.PubMedCrossRefGoogle Scholar
  64. 64.
    Slager UT, Taylor WF, Opfell RW, Myers A: Leptomeningeal myeloma. Arch Pathol Lab Med 103:680–682, 1979.PubMedGoogle Scholar
  65. 65.
    Stark RJ, Henson RA: Cerebral compression by myeloma. J Neurol Neurosurg Psychiatry 44:833–836, 1981.PubMedCrossRefGoogle Scholar
  66. 66.
    Tiselius A: Electrophoresis of serum globulin. II. Electrophoretic analysis of normal and immune sera. Biochem J 31:1464–1477, 1937.PubMedGoogle Scholar
  67. 67.
    Tiselius A, Kabat EA: An electrophoretic study of immune sera and purified antibody preparations. J Exp Med 69:119–131, 1939.PubMedCrossRefGoogle Scholar
  68. 68.
    Torrey JJ, Katakkar SB: Treatable meningeal involvement in Waldenström’s macroglobulinemia. Ann Intern Med 101:345–347, 1984.PubMedGoogle Scholar
  69. 69.
    Truong LD, Kim H-S, Estrada R: Meningeal myeloma. Am J Clin Pathol 78:532–535, 1982.PubMedGoogle Scholar
  70. 70.
    Van Allen MW, Frohlich JA, Davis JR: Inherited predisposition to generalized amyloidosis: Clinical and pathological study of a family with neuropathy, nephropathy, and peptic ulcer. Neurology (Minneap) 19:10–25, 1969.Google Scholar
  71. 71.
    Whicher JT, Hawkins L, Higginson J: Clinical applications of immunofixation: A more sensitive technique for the detection of Bence Jones protein. J Clin Pathol 33:779–780, 1980.PubMedCrossRefGoogle Scholar
  72. 72.
    Wiltshaw E: The natural history of extramedullar plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine (Baltimore) 55:217–238, 1976.CrossRefGoogle Scholar

Copyright information

© Martinus Nijhoff Publishing, Boston 1987

Authors and Affiliations

  • Robert A KyleJr.
    • 1
  1. 1.Division of Hematology and Internal MedicineMayo Medical School, Mayo Clinic and Mayo FoundationsRochesterUSA

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