Skip to main content
SpringerLink
Log in
SpringerLink
Log in

Pathology of Histiocytic Disorders and Neoplasms and Related Disorders

  • Chapter
  • First Online:
Histiocytic Disorders

Abstract

The pathology of histiocytic disorders and neoplasms of the macrophage-dendritic cell lineages is heterogeneous and may have overlapping features, all the while made more difficult by their rarity. The morphologic features together with immunophenotype and pattern of involvement should be taken together with the clinical and radiographic findings to make a unifying diagnosis. The “molecular microscope” of these lesions is further unraveling their biologic potential, which in some cases has helped to solidify their standing as inflammatory myeloid neoplasms. The histopathologic clues for the diagnosis of Langerhans cell histiocytosis (LCH), Erdheim-Chester Disease (ECD), the “juvenile xanthogranuloma” (JXG) family of lesions, Rosai-Dorfman disease (RDD), malignant histiocytoses including histiocytic sarcoma and Langerhans cell sarcoma, and other less common histiocytic-related disorders, including hemophagocytic lymphohistiocytosis (HLH), are reviewed in this chapter.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  1. Histiocytosis syndromes in children. Writing group of the Histiocyte Society. Lancet. 1987;1(8526):208–9.

    Google Scholar 

  2. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al. Contemporary classification of histiocytic disorders. The WHO Committee on histiocytic/reticulum cell proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol. 1997;29:157.

    Article  CAS  PubMed  Google Scholar 

  3. Berres ML, Lim KP, Peters T, Price J, Takizawa H, Salmon H, et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med. 2014;211(4):669–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Diamond EL, Durham BH, Haroche J, Yao Z, Ma J, Parikh SA, et al. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov. 2016;6(2):154–65.

    Article  CAS  PubMed  Google Scholar 

  5. Haroche J, Cohen-Aubart F, Charlotte F, Maksud P, Grenier PA, Cluzel P, et al. The histiocytosis Erdheim-Chester disease is an inflammatory myeloid neoplasm. Expert Rev Clin Immunol. 2015;11(9):1033–42.

    Article  CAS  PubMed  Google Scholar 

  6. Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms. Blood. 2016;127:2375.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Berres ML, Allen CE, Merad M. Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Adv Immunol. 2013;120:127–61.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Emile JF, Wechsler J, Brousse N, Boulland ML, Cologon R, Fraitag S, et al. Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples. Am J Surg Pathol. 1995;19(6):636–41.

    Article  CAS  PubMed  Google Scholar 

  10. Chikwava K, Jaffe R. Langerin (CD207) staining in normal pediatric tissues, reactive lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol. 2004;7(6):607–14.

    Article  CAS  PubMed  Google Scholar 

  11. Sahm F, Capper D, Preusser M, Meyer J, Stenzinger A, Lasitschka F, et al. BRAFV600E mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis. Blood. 2012;120(12):e28–34.

    Article  CAS  PubMed  Google Scholar 

  12. Mehes G, Irsai G, Bedekovics J, Beke L, Fazakas F, Rozsa T, et al. Activating BRAF V600E mutation in aggressive pediatric langerhans cell histiocytosis: demonstration by allele-specific PCR/direct sequencing and immunohistochemistry. Am J Surg Pathol. 2014;38:1644.

    Article  PubMed  Google Scholar 

  13. College of American Pathologists. Anatomic pathology checklist predictive markers 2015 [cited 2016 June 1, 2016]. Available from: http://www.cap.org/ShowProperty?nodePath=/UCMCon/Contribution%20Folders/WebContent/pdf/accreditation-checklists-full-listing.pdf.

  14. US Food and Drug Administration (FDA). Overview of IVD regulation silver spring, MD. 2015 [updated 3/19/15; cited 2016 June 30]. Available from: http://www.fda.gov/MedicalDevices/DeviceRegulationandGuidance/IVDRegulatoryAssistance/ucm123682.htm.

  15. Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood. 2015;126(1):26–35.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Heritier S, Emile JF, Barkaoui MA, Thomas C, Fraitag S, Boudjemaa S, et al. BRAF mutation correlates with high-risk langerhans cell histiocytosis and increased resistance to first-line therapy. J Clin Oncol. 2016;34:3023.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Ellison G, Donald E, McWalter G, Knight L, Fletcher L, Sherwood J, et al. A comparison of ARMS and DNA sequencing for mutation analysis in clinical biopsy samples. J Exp Clin Cancer Res. 2010;29:132.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  18. Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116(11):1919–23.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Brown NA, Furtado LV, Betz BL, Kiel MJ, Weigelin HC, Lim MS, et al. High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Blood. 2014;124(10):1655–8.

    Article  CAS  PubMed  Google Scholar 

  20. Nelson DS, Quispel W, Badalian-Very G, van Halteren AG, van den Bos C, Bovee JV, et al. Somatic activating ARAF mutations in Langerhans cell histiocytosis. Blood. 2014;123(20):3152–5.

    Article  CAS  PubMed  Google Scholar 

  21. Nelson DS, van Halteren A, Quispel WT, van den Bos C, Bovée JVMG, Patel B, et al. MAP2K1 and MAP3K1 mutations in langerhans cell histiocytosis. Genes Chromosomes Cancer. 2015;54(6):361–8.

    Article  CAS  PubMed  Google Scholar 

  22. Chakraborty R, Hampton OA, Shen X, Simko SJ, Shih A, Abhyankar H, et al. Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. Blood. 2014;124(19):3007–15.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Prince HM. Identifying mutant pathways in the histiocytoses. Blood. 2014;124(19):2901–3.

    Article  CAS  PubMed  Google Scholar 

  24. Rollins BJ. Genomic alterations in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015;29(5):839–51.

    Article  PubMed  Google Scholar 

  25. Picarsic J, Jaffe R. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015;29(5):799–823.

    Article  PubMed  Google Scholar 

  26. Haupt R, Minkov M, Astigarraga I, Schafer E, Nanduri V, Jubran R, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–84.

    Article  PubMed  Google Scholar 

  27. Roncaroli F, Consales A, Galassi E, Bernardi B, Valeri B. Occipital aneurysmal bone cyst secondary to eosinophilic granuloma. Pediatr Neurosurg. 2001;35(2):103–6.

    Article  CAS  PubMed  Google Scholar 

  28. Brown RSD, Edwards J, Bartlett JW, Jones C, Dogan A. Routine acid decalcification of bone marrow samples can preserve DNA for FISH and CGH studies in metastatic prostate cancer. J Histochem Cytochem. 2002;50(1):113–5.

    Article  CAS  PubMed  Google Scholar 

  29. Brown NA, Betz BL, Weigelin HC, Elenitoba-Johnson KS, Lim MS, Bailey NG. Evaluation of allele-specific PCR and immunohistochemistry for the detection of BRAF V600E mutations in hairy cell leukemia. Am J Clin Pathol. 2015;143(1):89–99.

    Article  PubMed  Google Scholar 

  30. Andrulis M, Penzel R, Weichert W, von Deimling A, Capper D. Application of a BRAF V600E mutation-specific antibody for the diagnosis of hairy cell leukemia. Am J Surg Pathol. 2012;36(12):1796–800.

    Article  PubMed  Google Scholar 

  31. Diamond EL, Dagna L, Hyman DM, Cavalli G, Janku F, Estrada-Veras J, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood. 2014;124(4):483–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Simko SJ, Garmezy B, Abhyankar H, Lupo PJ, Chakraborty R, Lim KPH, et al. Differentiating skin-limited and multisystem Langerhans cell histiocytosis. J Pediatr. 2014;165(5):990–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Jaffe R. Is there a role for histopathology in predicting the clinical outcome in congenital and infant Langerhans cell disease? Pediatr Blood Cancer. 2009;53(6):924–5.

    Article  PubMed  Google Scholar 

  34. Minkov M, Prosch H, Steiner M, Grois N, Potschger U, Kaatsch P, et al. Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer. 2005;45(6):802–7.

    Article  CAS  PubMed  Google Scholar 

  35. Online Mendelian Inheritance in Man O. OMENN SYNDROME Johns Hopkins University, Baltimore, MD.02/11/2014 [updated 02/11/2014]. Available from: http://omim.org/.

  36. Avila EM, Uzel G, Hsu A, Milner JD, Turner ML, Pittaluga S, et al. Highly variable clinical phenotypes of hypomorphic RAG1 mutations. Pediatrics. 2010;126(5):e1248–52.

    Article  PubMed  Google Scholar 

  37. Schuetz C, Huck K, Gudowius S, Megahed M, Feyen O, Hubner B, et al. An immunodeficiency disease with RAG mutations and granulomas. N Engl J Med. 2008;358(19):2030–8.

    Article  CAS  PubMed  Google Scholar 

  38. Wouters CH, Maes A, Foley KP, Bertin J, Rose CD. Blau syndrome, the prototypic auto-inflammatory granulomatous disease. Pediatr Rheumatol Online J. 2014;12:33.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Buchbinder D, Baker R, Lee YN, Ravell J, Zhang Y, McElwee J, et al. Identification of patients with RAG mutations previously diagnosed with common variable immunodeficiency disorders. J Clin Immunol. 2015;35(2):119–24.

    Article  CAS  PubMed  Google Scholar 

  40. Schmitz L, Favara BE. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 1998;12(2):221–46.

    Article  CAS  PubMed  Google Scholar 

  41. Christie LJ, Evans AT, Bray SE, Smith ME, Kernohan NM, Levison DA, et al. Lesions resembling Langerhans cell histiocytosis in association with other lymphoproliferative disorders: a reactive or neoplastic phenomenon? Hum Pathol. 2006;37(1):32–9.

    Article  CAS  PubMed  Google Scholar 

  42. Picarsic J, Egeler RM, Chikwava K, Patterson K, Jaffe R. Histologic patterns of thymic involvement in Langerhans cell proliferations: a clinicopathologic study and review of the literature. Pediatr Dev Pathol. 2015;18(2):127–38.

    Article  PubMed  Google Scholar 

  43. Ronceray L, Potschger U, Janka G, Gadner H, Minkov M. Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr. 2012;161(1):129–33 e1-3.

    Article  PubMed  Google Scholar 

  44. Kim HK, Park CJ, Jang S, Cho YU, Park SH, Koh KN, et al. Bone marrow involvement of Langerhans cell histiocytosis: immunohistochemical evaluation of bone marrow for CD1a, Langerin, and S100 expression. Histopathology. 2014;65(6):742–8.

    Article  PubMed  Google Scholar 

  45. Galluzzo ML, Braier J, Rosenzweig SD, Garcia de Davila MT, Rosso D. Bone marrow findings at diagnosis in patients with multisystem langerhans cell histiocytosis. Pediatr Dev Pathol. 2010;13(2):101–6.

    Article  PubMed  PubMed Central  Google Scholar 

  46. Minkov M, Potschger U, Grois N, Gadner H, Dworzak MN. Bone marrow assessment in Langerhans cell histiocytosis. Pediatr Blood Cancer. 2007;49(5):694–8.

    Article  PubMed  Google Scholar 

  47. Favara BE, Jaffe R, Egeler RM. Macrophage activation and hemophagocytic syndrome in langerhans cell histiocytosis: report of 30 cases. Pediatr Dev Pathol. 2002;5(2):130–40.

    Article  CAS  PubMed  Google Scholar 

  48. Chantranuwat C. Systemic form of juvenile xanthogranuloma: report of a case with liver and bone marrow involvement. Pediatr Dev Pathol. 2004;7(6):646–8.

    Article  PubMed  Google Scholar 

  49. Kesserwan C, Boue DR, Kahwash SB. Isolated juvenile xanthogranuloma in the bone marrow: report of a case and review of the literature. Pediatr Dev Pathol. 2007;10(2):161–4.

    Article  PubMed  Google Scholar 

  50. Takahashi K, Isobe T, Ohtsuki Y, Sonobe H, Takeda I, Akagi T. Immunohistochemical localization and distribution of S-100 proteins in the human lymphoreticular system. Am J Pathol. 1984;116(3):497–503.

    CAS  PubMed  PubMed Central  Google Scholar 

  51. Hadzic N, Pritchard J, Webb D, Portmann B, Heaton ND, Rela M, et al. Recurrence of Langerhans cell histiocytosis in the graft after pediatric liver transplantation. Transplantation. 2000;70(5):815–9.

    Article  CAS  PubMed  Google Scholar 

  52. Christiansen EC, Ellwein M, Neglia JP. Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis. Pediatr Blood Cancer. 2012;58(6):998–9.

    Article  PubMed  Google Scholar 

  53. Baqir M, Vassallo R, Maldonado F, Yi ES, Ryu JH. Utility of bronchoscopy in pulmonary Langerhans cell histiocytosis. J Bronchology Interv Pulmonol. 2013;20(4):309–12.

    Article  PubMed  Google Scholar 

  54. Roden AC, Yi ES. Pulmonary Langerhans cell histiocytosis: an update from the pathologists' perspective. Arch Pathol Lab Med. 2016;140(3):230–40.

    Article  CAS  PubMed  Google Scholar 

  55. Arnaud L, Pierre I, Beigelman-Aubry C, Capron F, Brun AL, Rigolet A, et al. Pulmonary involvement in Erdheim-Chester disease: a single-center study of 34 patients and a review of the literature. Arthritis Rheum. 2010;62(11):3504–12.

    Article  PubMed  Google Scholar 

  56. Kepes J. Histiocytosis X. In: BG VPJ, editor. Handbook of neurology, vol. 38. New York: Elsevier Publishing Company; 1979. p. 93–117.

    Google Scholar 

  57. Norman R. The neuronal storage diseases. In: Greenfield J, editor. Neuropathology. London: Arnold Publication; 1958. p. 398–9.

    Google Scholar 

  58. Grois N, Prayer D, Prosch H, Lassmann H. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain. 2005;128(Pt 4):829–38.

    Article  PubMed  Google Scholar 

  59. Grois N, Fahrner B, Arceci RJ, Henter JI, McClain K, Lassmann H, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 2010;156(6):873–81, 81 e1.

    Article  PubMed  Google Scholar 

  60. Imashuku S, Arceci RJ. Strategies for the prevention of central nervous system complications in patients with Langerhans cell histiocytosis: the problem of neurodegenerative syndrome. Hematol Oncol Clin North Am. 2015;29(5):875–93.

    Article  PubMed  Google Scholar 

  61. Grois N, Barkovich AJ, Rosenau W, Ablin AR. Central nervous system disease associated with Langerhans' cell histiocytosis. Am J Pediatr Hematol Oncol. 1993;15(2):245–54.

    Article  CAS  PubMed  Google Scholar 

  62. Picarsic J. Abstracts from the 32nd Annual Meeting of the Histiocyte Society Dublin, Ireland October 17–19, 2016. Pediatr Blood Cancer. 2016;63(S2):S10–65.

    Google Scholar 

  63. Picarsic J, editor. LCH Symposium: Langerhans cell histiocytosis in the central nervous system: Challenges in pathologic diagnosis. Histiocyte Society 31st Annual Meeting 2015; Athens, Greece.

    Google Scholar 

  64. Zinn D. Abstracts from the 32nd Annual Meeting of the Histiocyte Society Dublin, Ireland October 17–19, 2016. Pediatr Blood Cancer. 2016;63(S2):S10–65.

    Google Scholar 

  65. Grois NG, Favara BE, Mostbeck GH, Prayer D. Central nervous system disease in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 1998;12(2):287–305.

    Article  CAS  PubMed  Google Scholar 

  66. Paulus W, Kirchner T, Michaela M, Kuhl J, Warmuth-Metz M, Sorensen N, et al. Histiocytic tumor of Meckel's cave. An intracranial equivalent of juvenile xanthogranuloma of the skin. Am J Surg Pathol. 1992;16(1):76–83.

    Article  CAS  PubMed  Google Scholar 

  67. Jaffe R. Langerhans cell histiocytosis and Langerhans cell sarcoma. In: Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber DA, editors. Hematopathology. St. Louis: Saunders; 2011.

    Google Scholar 

  68. Singhi AD, Montgomery EA. Gastrointestinal tract Langerhans cell histiocytosis: a clinicopathologic study of 12 patients. Am J Surg Pathol. 2011;35(2):305–10.

    Article  PubMed  Google Scholar 

  69. Favara BE, Jaffe R. The histopathology of Langerhans cell histiocytosis. Br J Cancer Suppl. 1994;23:S17–23.

    CAS  PubMed  PubMed Central  Google Scholar 

  70. Moschovi M, Adamaki M, Vlahopoulos S, Rodriguez-Galindo C. Synchronous and metachronous thyroid cancer in relation to Langerhans cell histiocytosis; involvement of V600E BRAF-mutation? Pediatr Blood Cancer. 2015;62(1):173–4.

    Article  CAS  PubMed  Google Scholar 

  71. Johnson WT, Patel P, Hernandez A, Grandinetti LM, Huen AC, Marks S, et al. Langerhans cell histiocytosis and Erdheim-Chester disease, both with cutaneous presentations, and papillary thyroid carcinoma all harboring the BRAF mutation. J Cutan Pathol. 2015;43:270.

    Article  PubMed  Google Scholar 

  72. Courtillot C, Laugier Robiolle S, Cohen Aubart F, Leban M, Renard-Penna R, Drier A, et al. Endocrine manifestations in a monocentric cohort of 64 patients with Erdheim-Chester disease. J Clin Endocrinol Metab. 2016;101(1):305–13.

    Article  CAS  PubMed  Google Scholar 

  73. Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, et al. Erdheim-Chester disease. Curr Rheumatol Rep. 2014;16(4):412.

    Article  PubMed  CAS  Google Scholar 

  74. Jaffe R. Disseminated Juvenille Xanthogranuloma. In: Swerdlow S, Campo E, Lee Harris N, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissue (IARC WHO classification of Tumours). Lyon: International Agency for Research on Cancer (IARC); 2008.

    Google Scholar 

  75. Haroche J, Abla O. Uncommon histiocytic disorders: Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease. Hematology Am Soc Hematol Educ Program. 2015;2015:571–8.

    PubMed  Google Scholar 

  76. Hervier B, Haroche J, Arnaud L, Charlotte F, Donadieu J, Neel A, et al. Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation: a multicenter study of 23 cases. Blood. 2014;124:1119.

    Google Scholar 

  77. Emile JF, Diamond EL, Helias-Rodzewicz Z, Cohen-Aubart F, Charlotte F, Hyman DM, et al. Recurrent RAS and PIK3CA mutations in Erdheim-Chester disease. Blood. 2014;124(19):3016–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  78. Badalian-Very G. A common progenitor cell in LCH and ECD. Blood. 2014. 2014-08-14 00:00:00;124:991–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  79. O'Malley DP, Duong A, Barry TS, Chen S, Hibbard MK, Ferry JA, et al. Co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: possible relationship of two histiocytic disorders in rare cases. Mod Pathol. 2010;23(12):1616–23.

    Article  PubMed  Google Scholar 

  80. Abla O, Halliday W, Laughlin S, Weitzman S. Central nervous system juvenile xanthogranuloma after Langerhans cell histiocytosis. Pediatr Blood Cancer. 2013;60(2):342–3.

    Article  PubMed  Google Scholar 

  81. Bains A, Parham DM. Langerhans cell histiocytosis preceding the development of juvenile xanthogranuloma: a case and review of recent developments. Pediatr Dev Pathol. 2011;14(6):480–4.

    Article  PubMed  Google Scholar 

  82. Zaba LC, Fuentes-Duculan J, Steinman RM, Krueger JG, Lowes MA. Normal human dermis contains distinct populations of CD11c+BDCA-1+ dendritic cells and CD163+FXIIIA+ macrophages. J Clin Invest. 2007;117(9):2517–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Chasset F, Barete S, Charlotte F, Cohen-Aubart F, Arnaud L, Le Pelletier F, et al. Cutaneous manifestations of Erdheim-Chester disease (ECD): clinical, pathological, and molecular features in a monocentric series of 40 patients. J Am Acad Dermatol. 2016;74(3):513–20.

    Article  PubMed  Google Scholar 

  84. Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, et al. Erdheim-Chester disease. Rheum Dis Clin N Am. 2013;39(2):299–311.

    Article  Google Scholar 

  85. Drier A, Haroche J, Savatovsky J, Godeneche G, Dormont D, Chiras J, et al. Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 2010;255(2):586–94.

    Article  PubMed  Google Scholar 

  86. Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, et al. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease report of 6 new cases and a literature review. Medicine (Baltimore). 2004;83(6):371.

    Article  Google Scholar 

  87. Hyman DM, Diamond EL, Vibat CR, Hassaine L, Poole JC, Patel M, et al. Prospective blinded study of BRAFV600E mutation detection in cell-free DNA of patients with systemic histiocytic disorders. Cancer Discov. 2015;5(1):64–71.

    Article  CAS  PubMed  Google Scholar 

  88. Burgdorf WH, Zelger B. The non-Langerhans' cell histiocytoses in childhood. Cutis. 1996;58(3):201–7.

    CAS  PubMed  Google Scholar 

  89. Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses. Pediatr Blood Cancer. 2005;45(3):256–64.

    Article  PubMed  Google Scholar 

  90. Kraus MD, Haley JC, Ruiz R, Essary L, Moran CA, Fletcher CD. "juvenile" xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis. Am J Dermatopathol. 2001;23(2):104–11.

    Article  CAS  PubMed  Google Scholar 

  91. Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27(5):579–93.

    Article  PubMed  Google Scholar 

  92. Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am J Surg Pathol. 2005;29(1):21–8.

    Article  PubMed  Google Scholar 

  93. Sandell RF, Carter JM, Folpe AL. Solitary (juvenile) xanthogranuloma: a comprehensive immunohistochemical study emphasizing recently developed markers of histiocytic lineage. Hum Pathol. 2015;46(9):1390–7.

    Article  PubMed  Google Scholar 

  94. Haynes E, Schmitt L, Guo H, Jaffe R, Picarsic J. Abstracts from the 2017 Spring Society for Pediatric Pathology Meeting, San Antonio, TX, March 3-5, 2017. S100 Immunohistochemistry in 65 localized juvenile xanthogranuloma family leasions: Understanding staining patterns with clinicopathologic correlation. Pediatric and Developmental Pathology: S43.

    Google Scholar 

  95. Helwig EHV. Juvenile xanthogranuloma (nevoxanthoendothelioma) proceedings of the American Association of Pathologists and Bacteriologists. Am J Pathol. 1954;30(3):625–6.

    Google Scholar 

  96. Cline MJ. Histiocytes and histiocytosis. Blood. 1994;84(9):2840–53.

    CAS  PubMed  Google Scholar 

  97. Marrogi AJ, Dehner LP, Coffin CM, Wick MR. Benign cutaneous histiocytic tumors in childhood and adolescence, excluding Langerhans' cell proliferations. A clinicopathologic and immunohistochemical analysis. Am J Dermatopathol. 1992;14(1):8–18.

    Article  CAS  PubMed  Google Scholar 

  98. Zelger BW, Sidoroff A, Orchard G, Cerio R. Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol. 1996;18(5):490–504.

    Article  CAS  PubMed  Google Scholar 

  99. Jaffe R. Histiocytic proliferations in childhood. In: Proytcheva M, editor. Diagnostic pediatric hematopathology. Illinois/Cambridge: Feinberg School of Medicine, Northwestern University/Cambridge University Press; 2011. p. 504–39.

    Chapter  Google Scholar 

  100. Liu DT, Choi PC, Chan AY. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry. Am J Surg Pathol. 2005;29(8):1117. author reply -8

    PubMed  Google Scholar 

  101. Chang MW, Frieden IJ, Good W. The risk intraocular juvenile xanthogranuloma: survey of current practices and assessment of risk. J Am Acad Dermatol. 1996;34(3):445–9.

    Article  CAS  PubMed  Google Scholar 

  102. Shah AS, Wilson DP. Primary hypertriglyceridemia in children and adolescents. J Clin Lipidol. 2015;9(5 Suppl):S20–8.

    Article  PubMed  Google Scholar 

  103. Barroca H, Farinha NJ, Lobo A, Monteiro J, Lopes JM. Deep-seated congenital juvenile xanthogranuloma: report of a case with emphasis on cytologic features. Acta Cytol. 2007;51(3):473–6.

    Article  PubMed  Google Scholar 

  104. Globerman H, Burstein S, Girardina PJ, Winchester P, Frankel S. A xanthogranulomatous histiocytosis in a child presenting with short stature. Am J Pediatr Hematol Oncol. 1991;13(1):42–6.

    Article  CAS  PubMed  Google Scholar 

  105. Zhi Y, Duan Y, Zhang H, Yin X, Qu T, Guan G, et al. Solitary juvenile xanthogranuloma with tibial involvement: a case report. Int J Clin Exp Pathol. 2015;8(1):164–70.

    PubMed  PubMed Central  Google Scholar 

  106. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP. Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. J Pediatr. 1996;129(2):227–37.

    Article  CAS  PubMed  Google Scholar 

  107. Bostrom J, Janssen G, Messing-Junger M, Felsberg JU, Neuen-Jacob E, Engelbrecht V, et al. Multiple intracranial juvenile xanthogranulomas. Case report. J Neurosurg. 2000;93(2):335–41.

    Article  CAS  PubMed  Google Scholar 

  108. Orsey A, Paessler M, Lange BJ, Nichols KE. Central nervous system juvenile xanthogranuloma with malignant transformation. Pediatr Blood Cancer. 2008;50(4):927–30.

    Article  PubMed  Google Scholar 

  109. Kitchen ND, Davies MS, Taylor W. Juvenile xanthogranuloma of nerve root origin. Br J Neurosurg. 1995;9(2):233–7.

    Article  CAS  PubMed  Google Scholar 

  110. Ernemann U, Skalej M, Hermisson M, Platten M, Jaffe R, Voigt K. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis. Neuroradiology. 2002;44(9):759–63.

    Article  CAS  PubMed  Google Scholar 

  111. Hu WK, Gilliam AC, Wiersma SR, Dahms BB. Fatal congenital systemic juvenile xanthogranuloma with liver failure. Pediatr Dev Pathol. 2004;7(1):71–6.

    Article  PubMed  Google Scholar 

  112. Fan R, Sun J. Neonatal systemic juvenile xanthogranuloma with an ominous presentation and successful treatment. Clin Med Insights Oncol. 2011;5:157–61.

    Article  PubMed  PubMed Central  Google Scholar 

  113. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70.

    CAS  PubMed  Google Scholar 

  114. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer. 1972;30(5):1174–88.

    Article  CAS  PubMed  Google Scholar 

  115. Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bulletin de la Societe de pathologie exotique et de ses filiales. 1965;58(6):1169–75.

    CAS  PubMed  Google Scholar 

  116. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. Arch Otolaryngol. (Chicago, Ill : 1960). 1978;104(12):687–93.

    Article  CAS  PubMed  Google Scholar 

  117. Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol. 1979;87(3):354–67.

    Article  CAS  PubMed  Google Scholar 

  118. Foucar E, Rosai J, Dorfman RF, Brynes RK. The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology. 1982;32(4):365–72.

    Article  CAS  PubMed  Google Scholar 

  119. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19–73.

    CAS  PubMed  Google Scholar 

  120. Online Mendelian Inheritance in Man O. histiocytosis-lymphadenopathy plus syndrome Johns Hopkins University, Baltimore, MD.07/18/2013. Available from: World Wide Web URL: http://omim.org/.

  121. Maric I, Pittaluga S, Dale JK, Niemela JE, Delsol G, Diment J, et al. Histologic features of sinus histiocytosis with massive lymphadenopathy in patients with autoimmune lymphoproliferative syndrome. Am J Surg Pathol. 2005;29(7):903–11.

    Article  PubMed  Google Scholar 

  122. Maia DM, Dorfman RF. Focal changes of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with nodular lymphocyte predominant Hodgkin's disease. Hum Pathol. 1995;26(12):1378–82.

    Article  CAS  PubMed  Google Scholar 

  123. Delacretaz F, Meuge-Moraw C, Anwar D, Borisch B, Chave JP. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient. Virchows Arch A Pathol Anat Histopathol. 1991;419(3):251–4.

    Article  CAS  PubMed  Google Scholar 

  124. Lu D, Estalilla OC, Manning JT Jr, Medeiros LJ. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature. Mod Pathol. 2000;13(4):414–9.

    Article  CAS  PubMed  Google Scholar 

  125. OMIM OMIiM. Autoimmune lymphoproliferative syndrome Johns Hopkins University, Baltimore, MD.03/01/2016 [cited 2016]. Available from: World Wide Web URL: http://omim.org/.

  126. Menon MP, Evbuomwan MO, Rosai J, Jaffe ES, Pittaluga S. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease? Histopathology. 2014;64(3):455–9.

    Article  PubMed  Google Scholar 

  127. Liu L, Perry AM, Cao W, Smith LM, Hsi ED, Liu X, et al. Relationship between Rosai-Dorfman disease and IgG4-related disease: study of 32 cases. Am J Clin Pathol. 2013;140(3):395–402.

    Article  PubMed  Google Scholar 

  128. Colmenero I, Molho-Pessach V, Torrelo A, Zlotogorski A, Requena L. Emperipolesis: an additional common histopathologic finding in H syndrome and Rosai-Dorfman disease. Am J Dermatopathol. 2012;34(3):315–20.

    Article  PubMed  Google Scholar 

  129. Morgan NV, Morris MR, Cangul H, Gleeson D, Straatman-Iwanowska A, Davies N, et al. Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease. PLoS Genet. 2010;6(2):e1000833.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  130. Koduru PR, Susin M, Kolitz JE, Soni M, Teichberg S, Siques MJ, et al. Morphological, ultrastructural, and genetic characterization of an unusual T-cell lymphoma in a patient with sinus histiocytosis with massive lymphadenopathy. Am J Hematol. 1995;48(3):192–200.

    Article  CAS  PubMed  Google Scholar 

  131. Foucar E, Rosai J, Dorfman RF, Eyman JM. Immunologic abnormalities and their significance in sinus histiocytosis with massive lymphadenopathy. Am J Clin Pathol. 1984;82(5):515–25.

    Article  CAS  PubMed  Google Scholar 

  132. Rangwala AF, Zinterhofer LJ, Nyi KM, Ferreira PP. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma. An unreported association. Cancer. 1990;65(4):999–1002.

    Article  CAS  PubMed  Google Scholar 

  133. Krzemieniecki K, Pawlicki M, Marganska K, Parczewska J. The Rosai-Dorfman syndrome in a 17-year-old woman with transformation into high-grade lymphoma. A rare disease presentation. Ann Oncol. 1996;7(9):977.

    Article  CAS  PubMed  Google Scholar 

  134. Lossos IS, Okon E, Bogomolski-Yahalom V, Ron N, Polliack A. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a patient with isolated renotesticular involvement after cure of non-Hodgkin's lymphoma. Ann Hematol. 1997;74(1):41–4.

    Article  CAS  PubMed  Google Scholar 

  135. Moore JC, Zhao X, Nelson EL. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Rep. 2008;2:70.

    Article  PubMed  PubMed Central  Google Scholar 

  136. Shoda H, Oka T, Inoue M, Kusaka S, Tsuneyoshi H, Miyazaki J, et al. Sinus histiocytosis with massive lymphadenopathy associated with malignant lymphoma. Intern Med. 2004;43(8):741–5.

    Article  PubMed  Google Scholar 

  137. Garel L, Lucaya J, Piqueras J. Clinical quiz. Large-cell anaplastic lymphoma coexisting with Rosai-Dorfman disease. Pediatr Radiol. 2004;34(6):509–10.

    Article  PubMed  Google Scholar 

  138. Wu W, Cao L, Li Y, Yu X, Huang H, He J. Primary splenic diffuse large B-cell lymphoma in a patient with thymus Rosai-Dorfman disease. Am J Med Sci. 2012;344(2):155–9.

    Article  PubMed  Google Scholar 

  139. Llamas-Velasco M, Cannata J, Dominguez I, Garcia-Noblejas A, Aragues M, Fraga J, et al. Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. J Cutan Pathol. 2012;39(12):1125–30.

    Article  PubMed  Google Scholar 

  140. Di Tommaso L, Rahal D, Bossi P, Roncalli M. Hepatic rosai-dorfman disease with coincidental lymphoma: report of a case. Int J Surg Pathol. 2010;18(6):540–3.

    Article  PubMed  Google Scholar 

  141. Falk S, Stutte HJ, Frizzera G. Hodgkin's disease and sinus histiocytosis with massive lymphadenopathy-like changes. Histopathology. 1991;19(3):221–4.

    Article  CAS  PubMed  Google Scholar 

  142. Brown RA, Kwong BY, McCalmont TH, Ragsdale B, Ma L, Cheung C, et al. ETV3-NCOA2 in indeterminate cell histiocytosis: clonal translocation supports sui generis. Blood. 2015;126(20):2344–5.

    Article  PubMed  Google Scholar 

  143. Chan JK, Lamant L, Algar E, Delsol G, Tsang WY, Lee KC, et al. ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy. Blood. 2008;112(7):2965–8.

    Article  CAS  PubMed  Google Scholar 

  144. Wood GS, Hu CH, Beckstead JH, Turner RR, Winkelmann RK. The indeterminate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis. J Dermatol Surg Oncol. 1985;11(11):1111–9.

    Article  CAS  PubMed  Google Scholar 

  145. Rezk SA, Spagnolo DV, Brynes RK, Weiss LM. Indeterminate cell tumor: a rare dendritic neoplasm. Am J Surg Pathol. 2008;32(12):1868–76.

    Article  PubMed  Google Scholar 

  146. Roh J, Kim SW, Park CS. Indeterminate dendritic cell tumor: a case report of a rare Langerhans cell lineage disease. J Pathol Transl Med. 2016;50(1):78–81.

    Article  PubMed  Google Scholar 

  147. Jaffe R, Favara B. Non-Langerhans' dendritic cell histiocytosis (abstract). Pediatr Dev Pathol. 2002;5:99.

    Google Scholar 

  148. Castro P, Rebocho AP, Soares RJ, Magalhaes J, Roque L, Trovisco V, et al. PAX8-PPARgamma rearrangement is frequently detected in the follicular variant of papillary thyroid carcinoma. J Clin Endocrinol Metab. 2006;91(1):213–20.

    Article  CAS  PubMed  Google Scholar 

  149. Go H, Jeon YK, Huh J, Choi SJ, Choi YD, Cha HJ, et al. Frequent detection of BRAF(V600E) mutations in histiocytic and dendritic cell neoplasms. Histopathology. 2014;65(2):261–72.

    Article  PubMed  Google Scholar 

  150. Liu Q, Tomaszewicz K, Hutchinson L, Hornick JL, Woda B, Yu H. Somatic mutations in histiocytic sarcoma identified by next generation sequencing. Virchows Arch. 2016;469:233.

    Article  CAS  PubMed  Google Scholar 

  151. Heath JL, Burgett SE, Gaca AM, Jaffe R, Wechsler DS. Successful treatment of pediatric histiocytic sarcoma using abbreviated high-risk leukemia chemotherapy. Pediatr Blood Cancer. 2014;61(10):1874–6.

    Article  PubMed  Google Scholar 

  152. Ikdahl T, Josefsen D, Jakobsen E, Delabie J, Fossa SD. Concurrent mediastinal germ-cell tumour and haematological malignancy: case report and short review of literature. Acta Oncol. 2008;47(3):466–9.

    Article  PubMed  Google Scholar 

  153. Takahashi E, Nakamura S. Histiocytic sarcoma : an updated literature review based on the 2008 WHO classification. J Clin Exp Hematop. 2013;53(1):1–8.

    Article  PubMed  Google Scholar 

  154. Durham BH, Diamond EL, Abdel-Wahab O. Histiocytic neoplasms in the era of personalized genomic medicine. Curr Opin Hematol. 2016;23(4):416–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  155. Kumar R, Khan SP, Joshi DD, Shaw GR, Ketterling RP, Feldman AL. Pediatric histiocytic sarcoma clonally related to precursor B-cell acute lymphoblastic leukemia with homozygous deletion of CDKN2A encoding p16(INK4A). Pediatr Blood Cancer. 2010;56:307.

    Article  Google Scholar 

  156. Feldman AL. Clonal relationships between malignant lymphomas and histiocytic/dendritic cell tumors. Surg Pathol Clin. 2013;6(4):619–29.

    Article  PubMed  Google Scholar 

  157. Castro EC, Blazquez C, Boyd J, Correa H, de Chadarevian JP, Felgar RE, et al. Clinicopathologic features of histiocytic lesions following ALL, with a review of the literature. Pediatr Dev Pathol. 2010;13(3):225–37.

    Article  PubMed  Google Scholar 

  158. Shao H, Xi L, Raffeld M, Feldman AL, Ketterling RP, Knudson R, et al. Clonally related histiocytic/dendritic cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: a study of seven cases. Mod Pathol. 2011;24(11):1421–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  159. Ansari J, Naqash AR, Munker R, El-Osta H, Master S, Cotelingam JD, et al. Histiocytic sarcoma as a secondary malignancy: pathobiology, diagnosis, and treatment. Eur J Haematol. 2016;97(1):9–16.

    Article  PubMed  Google Scholar 

  160. Brown AF, Fan H, Floyd JR, Henry JM, Higgins RA. Primary central nervous system histiocytic sarcoma arising after precursor B-cell acute lymphoblastic leukemia. J Neuropathol Exp Neurol. 2015;74(12):1120–6.

    CAS  PubMed  Google Scholar 

  161. Fernandez-Pol S, Bangs CD, Cherry A, Arber DA, Gratzinger D. Two cases of histiocytic sarcoma with BCL2 translocations and occult or subsequent follicular lymphoma. Hum Pathol. 2016;55:39–43.

    Article  CAS  PubMed  Google Scholar 

  162. Hayase E, Kurosawa M, Yonezumi M, Suzuki S, Suzuki H. Aggressive sporadic histiocytic sarcoma with immunoglobulin heavy chain gene rearrangement and t(14;18). Int J Hematol. 2010;92(4):659–63.

    Article  CAS  PubMed  Google Scholar 

  163. Howard JE, Dwivedi RC, Masterson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev. 2015;41(4):320–31.

    Article  CAS  PubMed  Google Scholar 

  164. Zwerdling T, Won E, Shane L, Javahara R, Jaffe R. Langerhans cell sarcoma: case report and review of world literature. J Pediatr Hematol Oncol. 2014;36(6):419–25.

    Article  PubMed  Google Scholar 

  165. Mourah S, Lorillon G, Meignin V, Vercellino L, de Margerie-Mellon C, Pages C, et al. Dramatic transient improvement of metastatic BRAF(V600E)-mutated Langerhans cell sarcoma under treatment with dabrafenib. Blood. 2015;126(24):2649–52.

    Article  CAS  PubMed  Google Scholar 

  166. Chen W, Jaffe R, Zhang L, Hill C, Block AM, Sait S, et al. Langerhans cell sarcoma arising from chronic lymphocytic lymphoma/small lymphocytic leukemia: lineage analysis and BRAF V600E mutation study. N Am J Med Sci. 2013;5(6):386–91.

    Article  PubMed  PubMed Central  Google Scholar 

  167. Deng A, Lee W, Pfau R, Harrington A, DiGiovani J, Prickett KA, et al. Primary cutaneous Langerhans cell sarcoma without Birbeck granules: indeterminate cell sarcoma? J Cutan Pathol. 2008;35(9):849–54.

    Article  PubMed  Google Scholar 

  168. Dalia S, Shao H, Sagatys E, Cualing H, Sokol L. Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment. Cancer Control J Moffitt Cancer Center. 2014;21(4):290–300.

    Article  Google Scholar 

  169. O'Malley DP, Agrawal R, Grimm KE, Hummel J, Glazyrin A, Dim DC, et al. Evidence of BRAF V600E in indeterminate cell tumor and interdigitating dendritic cell sarcoma. Ann Diagn Pathol. 2015;19(3):113–6.

    Article  PubMed  Google Scholar 

  170. Lupato V, Romeo S, Franchi A, Mantovani M, Dei Tos AP, Tirelli G, et al. Head and neck extranodal interdigitating dendritic cell sarcoma: case report and review of the literature. Head Neck Pathol. 2016;10(2):145–51.

    Article  PubMed  Google Scholar 

  171. Khashab T, Sehgal L, Medeiros LJ, Samaniego F. Spontaneous regression of interdigitating dendritic sarcoma in a patient with concurrent small lymphocytic lymphoma. BMJ Case Rep. 2015;2015. doi: 10.1136/bcr-2014-209014

  172. Cossu A, Deiana A, Lissia A, Dedola MF, Cocco L, Palmieri G, et al. Synchronous interdigitating dendritic cell sarcoma and B-cell small lymphocytic lymphoma in a lymph node. Arch Pathol Lab Med. 2006;130(4):544–7.

    PubMed  Google Scholar 

  173. Stowman AM, Mills SE, Wick MR. Spindle cell melanoma and interdigitating dendritic cell sarcoma: do they represent the same process? Am J Surg Pathol. 2016;40:1270.

    Article  PubMed  Google Scholar 

  174. Di Liso E, Pennelli N, Lodovichetti G, Ghiotto C, Dei Tos AP, Conte P, et al. Braf mutation in interdigitating dendritic cell sarcoma: a case report and review of the literature. Cancer Biol Ther. 2015;16(8):1128–35.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  175. Facchetti F, Lorenzi L. Follicular dendritic cells and related sarcoma. Semin Diagn Pathol. 2016;33:262.

    Article  PubMed  Google Scholar 

  176. Andriko JW, Kaldjian EP, Tsokos M, Abbondanzo SL, Jaffe ES. Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells. Am J Surg Pathol. 1998;22(9):1048–58.

    Article  CAS  PubMed  Google Scholar 

  177. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.

    Article  PubMed  Google Scholar 

  178. Ost A, Nilsson-Ardnor S, Henter JI. Autopsy findings in 27 children with haemophagocytic lymphohistiocytosis. Histopathology. 1998;32(4):310–6.

    Article  CAS  PubMed  Google Scholar 

  179. Bernstrand C, Cederlund K, Sandstedt B, Ahstrom L, Lundell M, Dahlquist G, et al. Pulmonary abnormalities at long-term follow-up of patients with Langerhans cell histiocytosis. Med Pediatr Oncol. 2001;36(4):459–68.

    Article  CAS  PubMed  Google Scholar 

  180. Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL study Group of the Histiocyte Society. Semin Oncol. 1991;18(1):29–33.

    CAS  PubMed  Google Scholar 

  181. Chen JH, Fleming MD, Pinkus GS, Pinkus JL, Nichols KE, Mo JQ, et al. Pathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol. 2010;34(6):852–67.

    Article  PubMed  Google Scholar 

  182. Macheta M, Will AM, Houghton JB, Wynn RF. Prominent dyserythropoiesis in four cases of haemophagocytic lymphohistiocytosis. J Clin Pathol. 2001;54(12):961–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, University of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA

    Jennifer Picarsic MD

  2. Department of Pathology, Children’s Hospital of Pittsburgh of UPMC, One Children’s Hospital Drive, 4401 Penn Avenue, Main Hospital, B260, Pittsburgh, PA, 15224, USA

    Jennifer Picarsic MD

  3. Department of Pathology, University of Pittsburgh School of Medicine, Magee Women’s Hospital of UPMC, Pittsburgh, PA, USA

    Ronald Jaffe MB, BCh

Authors
  1. Jennifer Picarsic MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ronald Jaffe MB, BCh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jennifer Picarsic MD .

Editor information

Editors and Affiliations

  1. University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada

    Oussama Abla

  2. Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany

    Gritta Janka

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Picarsic, J., Jaffe, R. (2018). Pathology of Histiocytic Disorders and Neoplasms and Related Disorders. In: Abla, O., Janka, G. (eds) Histiocytic Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-59632-7_1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-59632-7_1

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59631-0

  • Online ISBN: 978-3-319-59632-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation