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Immunohistology of Leukemia Cutis and Histiocytic Tumors

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Applied Immunohistochemistry in the Evaluation of Skin Neoplasms

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Abstract

The diseases studied in this chapter constitute a heterogeneous group of lesions that range from reactive conditions to highly aggressive neoplasms. These entities have been grouped into two main sections. The first one deals with myeloproliferative neoplasms and includes mastocytosis as well, since even in cases of mastocytosis with clinical symptoms seemingly limited to the skin a potential systemic involvement should be investigated. Also in the first section, leukemia cutis and myeloid sarcoma have been put together due to the considerable overlap of these two entities. The second group includes conditions caused by proliferation of dendritic cells such as Langerhans cell histiocytosis, Langerhans cell sarcoma, and indeterminate dendritic cell tumor. Then, the chapter deals with macrophage-related diseases, including juvenile xanthogranuloma and related diseases, hemophagocytic lymphohistiocytosis, multicentric reticulohistiocytosis, reticulohistiocytoma, Rosai-Dorfman disease, cutaneous Kikuchi-Fujimoto disease, and cutaneous intralymphatic histiocytosis. Multicentric reticulohistiocytosis and solitary reticulohistiocytoma, also known as solitary epithelioid histiocytoma, have been considered separately since despite their sharing many microscopical and immunohistochemical features they are distinct entities with striking clinical differences. Multicentric reticulohistiocytosis can involve extracutaneous locations and is frequently associated with a rheumatological disease, whereas solitary reticulohistiocytoma seems to be tumoral in nature. Finally, the malignant histiocytic and dendritic cell sarcomas include histiocytic sarcoma, follicular dendritic cell sarcoma, and interdigitating dendritic cell sarcoma.

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References

  1. Cho-Vega JH, Medeiros LJ, Prieto VG, Vega F. Leukemia cutis. Am J Clin Pathol. 2008;129(1):130–42.

    Article  PubMed  Google Scholar 

  2. Pileri SA, Orazi A, Falini B. Myeloid sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon, France: IARC Press; 2008. p. 140–1.

    Google Scholar 

  3. Hsi AC, Robirds DH, Luo J, Kreisel FH, Frater JL, Nguyen TT. T-cell prolymphocytic leukemia frequently shows cutaneous involvement and is associated with gains of MYC, loss of ATM, and TCL1A rearrangement. Am J Surg Pathol. 2014;38(11):1468–83.

    Article  PubMed  Google Scholar 

  4. Tokura Y, Sawada Y, Shimauchi T. Skin manifestations of adult T-cell leukemia/lymphoma: clinical, cytological and immunological features. J Dermatol. 2014;41(1):19–25.

    Article  CAS  PubMed  Google Scholar 

  5. Robak E, Robak T. Skin lesions in chronic lymphocytic leukemia. Leuk Lymphoma. 2007;48(5):855–65.

    Article  PubMed  Google Scholar 

  6. Cerroni L, Zenahlik P, Höfler G, Kaddu S, Smolle J, Kerl H. Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia: a clinicopathologic and prognostic study of 42 patients. Am J Surg Pathol. 1996;20(8):1000–10.

    Article  CAS  PubMed  Google Scholar 

  7. Tapia G, Mate JL, Fuente MJ, Navarro JT, Fernández-Figueras MT, Juncà J, et al. Cutaneous presentation of chronic lymphocytic leukemia as unique extramedullar involvement in a patient with normal peripheral blood lymphocyte count (monoclonal B-cell lymphocytosis). J Cutan Pathol. 2013;40(8):740–4.

    Article  PubMed  Google Scholar 

  8. Bénet C, Gomez A, Aguilar C, Delattre C, Vergier B, Beylot-Barry M, et al. Histologic and immunohistologic characterization of skin localization of myeloid disorders: a study of 173 cases. Am J Clin Pathol. 2011;135(2):278–90.

    Article  PubMed  Google Scholar 

  9. Hurley MY, Ghahramani GK, Frisch S, Armbrecht ES, Lind AC, Nguyen TT, et al. Cutaneous myeloid sarcoma: natural history and biology of an uncommon manifestation of acute myeloid leukemia. Acta Derm Venereol. 2013;93(3):319–24.

    Article  PubMed  Google Scholar 

  10. Amador-Ortiz C, Hurley MY, Ghahramani GK, Frisch S, Klco JM, Lind AC, et al. Use of classic and novel immunohistochemical markers in the diagnosis of cutaneous myeloid sarcoma. J Cutan Pathol. 2011;38(12):945–53.

    Article  PubMed  Google Scholar 

  11. Mathew RA, Bennett JM, Liu JJ, Komrokji RS, Lancet JE, Naghashpour M, et al. Cutaneous manifestations in CMML: indication of disease acceleration or transformation to AML and review of the literature. Leuk Res. 2012;36(1):72–80.

    Article  PubMed  Google Scholar 

  12. Patel LM, Maghari A, Schwartz RA, Kapila R, Morgan AJ, Lambert WC. Myeloid leukemia cutis in the setting of myelodysplastic syndrome: a crucial dermatological diagnosis. Int J Dermatol. 2012;51(4):383–8.

    Article  PubMed  Google Scholar 

  13. Klco JM, Welch JS, Nguyen TT, Hurley MY, Kreisel FH, Hassan A, et al. State of the art in myeloid sarcoma. Int J Lab Hematol. 2011;33(6):555–65.

    Article  CAS  PubMed  Google Scholar 

  14. Aboutalebi A, Korman JB, Sohani AR, Hasserjian RP, Louissaint Jr A, Le L, et al. Aleukemic cutaneous myeloid sarcoma. J Cutan Pathol. 2013;40(12):996–1005.

    Article  PubMed  Google Scholar 

  15. Angulo J, Haro R, González-Guerra E, Fariña MC, Martín L, Requena L. Leukemia cutis presenting as localized cutaneous hyperpigmentation. J Cutan Pathol. 2008;35(7):662–5.

    Article  PubMed  Google Scholar 

  16. Freiman A, Muhn CY, Trudel M, Billick RC. Leukemia cutis presenting with fingertip hypertrophy. J Cutan Med Surg. 2003;7(1):57–60.

    Article  PubMed  Google Scholar 

  17. Hattori T, Amano H, Nagai Y, Ishikawa O. Leukemia cutis in a patient with acute monocytic leukemia presenting as unique facial erythema. J Dermatol. 2008;35(10):671–4.

    Article  PubMed  Google Scholar 

  18. Cañueto J, Meseguer-Yebra C, Román-Curto C, Santos-Briz A, Fernández-López E, Fraile C, et al. Leukemic vasculitis: a rare pattern of leukemia cutis. J Cutan Pathol. 2011;38(4):360–4.

    PubMed  Google Scholar 

  19. Martínez-Escanamé M, Zuriel D, Tee SI, Fried I, Massone C, Cerroni L. Cutaneous infiltrates of acute myelogenous leukemia simulating inflammatory dermatoses. Am J Dermatopathol. 2013;35(4):419–24.

    Article  PubMed  Google Scholar 

  20. Baksh FK, Nathan D, Richardson W, Kestenbaum T, Woodroof J. Leukemia cutis with prominent giant cell reaction. Am J Dermatopathol. 1998;20(1):48–52.

    Article  CAS  PubMed  Google Scholar 

  21. Chavan RN, Cappel MA, Ketterling RP, Wada DA, Rochet NM, Knudson R, et al. Histiocytoid Sweet syndrome may indicate leukemia cutis: a novel application of fluorescence in situ hybridization. J Am Acad Dermatol. 2014;70(6):1021–7.

    Article  PubMed  Google Scholar 

  22. Cibull TL, Thomas AB, O'Malley DP, Billings SD. Myeloid leukemia cutis: a histologic and immunohistochemical review. J Cutan Pathol. 2008;35(2):180–5.

    PubMed  Google Scholar 

  23. Cronin DM, George TI, Sundram UN. An updated approach to the diagnosis of myeloid leukemia cutis. Am J Clin Pathol. 2009;132(1):101–10.

    Article  PubMed  Google Scholar 

  24. Sangle NA, Schmidt RL, Patel JL, Medeiros LJ, Agarwal AM, Perkins SL. Optimized immunohistochemical panel to differentiate myeloid sarcoma from blastic plasmacytoid dendritic cell neoplasm. Mod Pathol. 2014;27(8):1137–43.

    Article  CAS  PubMed  Google Scholar 

  25. Cronin DM, George TI, Reichard KK, Sundram UN. Immunophenotypic analysis of myeloperoxidase-negative leukemia cutis and blastic plasmacytoid dendritic cell neoplasm. Am J Clin Pathol. 2012;137(3):367–76.

    Article  PubMed  Google Scholar 

  26. Murphy MJ. Molecular testing of leukemia cutis. J Cutan Pathol. 2013;40(2):286–8.

    Article  PubMed  Google Scholar 

  27. Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21(2):340–50.

    Article  CAS  PubMed  Google Scholar 

  28. Falini B, Lenze D, Hasserjian R, Coupland S, Jaehne D, Soupir C, et al. Cytoplasmic mutated nucleophosmin (NPM) defines the molecular status of a significant fraction of myeloid sarcomas. Leukemia. 2007;21(7):1566–70.

    Article  CAS  PubMed  Google Scholar 

  29. Sen F, Zhang XX, Prieto VG, Shea CR, Qumsiyeh MB. Increased incidence of trisomy 8 in acute myeloid leukemia with skin infiltration (leukemia cutis). Diagn Mol Pathol. 2000;9(4):190–4.

    Article  CAS  PubMed  Google Scholar 

  30. Agis H, Weltermann A, Fonatsch C, Haas O, Mitterbauer G, Müllauer L, et al. A comparative study on demographic, hematological, and cytogenetic findings and prognosis in acute myeloid leukemia with and without leukemia cutis. Ann Hematol. 2002;81(2):90–5.

    Article  CAS  PubMed  Google Scholar 

  31. Requena L, Kutzner H, Palmedo G, Pascual M, Fernández-Herrera J, Fraga J, et al. Histiocytoid Sweet syndrome: a dermal infiltration of immature neutrophilic granulocytes. Arch Dermatol. 2005;141(7):834–42.

    Article  PubMed  Google Scholar 

  32. Facchetti F, Jones DM, Petrella T. Blastic plasmacytoid dendritic cells neoplasm. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon, France: IARC Press; 2008. p. 146–7.

    Google Scholar 

  33. Brody JP, Allen S, Schulman P, Sun T, Chan WC, Friedman HD, et al. Acute agranular CD4-positive natural killer cell leukemia. Comprehensive clinicopathologic studies including virologic and in vitro culture with inducing agents. Cancer. 1995;75(10):2474–83.

    Article  CAS  PubMed  Google Scholar 

  34. DiGiuseppe JA, Louie DC, Williams JE, Miller DT, Griffin CA, Mann RB, et al. Blastic natural killer cell leukemia/lymphoma: a clinicopathologic study. Am J Surg Pathol. 1997;21(10):1223–30.

    Article  CAS  PubMed  Google Scholar 

  35. Petrella T, Comeau MR, Maynadié M, Couillault G, De Muret A, Maliszewski CR, et al. “Agranular CD4+ CD56+ hematodermic neoplasm” (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol. 2002;26(7):852–62.

    Article  PubMed  Google Scholar 

  36. Julia F, Dalle S, Duru G, Balme B, Vergier B, Ortonne N, et al. Blastic plasmacytoid dendritic cell neoplasms: clinico-immunohistochemical correlations in a series of 91 patients. Am J Surg Pathol. 2014;38(5):673–80.

    Article  PubMed  Google Scholar 

  37. Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, et al. Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review. Am J Clin Pathol. 2005;123(5):662–75.

    Article  PubMed  Google Scholar 

  38. Khoury JD, Medeiros LJ, Manning JT, Sulak LE, Bueso-Ramos C, Jones D. CD56(+) TdT(+) blastic natural killer cell tumor of the skin: a primitive systemic malignancy related to myelomonocytic leukemia. Cancer. 2002;94(9):2401–8.

    Article  CAS  PubMed  Google Scholar 

  39. Herling M, Jones D. CD4+/CD56+ hematodermic tumor: the features of an evolving entity and its relationship to dendritic cells. Am J Clin Pathol. 2007;127(5):687–700.

    Article  PubMed  Google Scholar 

  40. Herling M, Teitell MA, Shen RR, Medeiros LJ, Jones D. TCL1 expression in plasmacytoid dendritic cells (DC2s) and the related CD4+ CD56+ blastic tumors of skin. Blood. 2003;101(12):5007–9.

    Article  CAS  PubMed  Google Scholar 

  41. Petrella T, Meijer CJ, Dalac S, Willemze R, Maynadié M, Machet L, et al. TCL1 and CLA expression in agranular CD4/CD56 hematodermic neoplasms (blastic NK-cell lymphomas) and leukemia cutis. Am J Clin Pathol. 2004;122(2):307–13.

    Article  CAS  PubMed  Google Scholar 

  42. Pilichowska ME, Fleming MD, Pinkus JL, Pinkus GS. CD4+/CD56+ hematodermic neoplasm (“blastic natural killer cell lymphoma”): neoplastic cells express the immature dendritic cell marker BDCA-2 and produce interferon. Am J Clin Pathol. 2007;128(3):445–53.

    Article  CAS  PubMed  Google Scholar 

  43. Montes-Moreno S, Ramos-Medina R, Martínez-López A, Barrionuevo Cornejo C, Parra Cubillos A, Quintana-Truyenque S, et al. SPIB, a novel immunohistochemical marker for human blastic plasmacytoid dendritic cell neoplasms: characterization of its expression in major hematolymphoid neoplasms. Blood. 2013;121(4):643–7.

    Article  CAS  PubMed  Google Scholar 

  44. Marafioti T, Paterson JC, Ballabio E, Reichard KK, Tedoldi S, Hollowood K, et al. Novel markers of normal and neoplastic human plasmacytoid dendritic cells. Blood. 2008;111(7):3778–92.

    Article  CAS  PubMed  Google Scholar 

  45. Leroux D, Mugneret F, Callanan M, Radford-Weiss I, Dastugue N, Feuillard J, et al. CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique. Blood. 2002;99(11):4154–9.

    Article  CAS  PubMed  Google Scholar 

  46. Lucioni M, Novara F, Fiandrino G, Riboni R, Fanoni D, Arra M, et al. Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood. 2011;118(17):4591–4.

    Article  CAS  PubMed  Google Scholar 

  47. Sapienza MR, Fuligni F, Agostinelli C, Tripodo C, Righi S, Laginestra MA, et al. Molecular profiling of blastic plasmacytoid dendritic cell neoplasm reveals a unique pattern and suggests selective sensitivity to NF-kB pathway inhibition. Leukemia. 2014;28(8):1606–16.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Alayed K, Patel KP, Konoplev S, Singh RR, Routbort MJ, Reddy N, et al. TET2 mutations, myelodysplastic features, and a distinct immunoprofile characterize blastic plasmacytoid dendritic cell neoplasm in the bone marrow. Am J Hematol. 2013;88(12):1055–61.

    Article  CAS  PubMed  Google Scholar 

  49. Menezes J, Acquadro F, Wiseman M, Gómez-López G, Salgado RN, Talavera-Casañas JG, et al. Exome sequencing reveals novel and recurrent mutations with clinical impact in blastic plasmacytoid dendritic cell neoplasm. Leukemia. 2014;28(4):823–9.

    Article  CAS  PubMed  Google Scholar 

  50. Assaf C, Gellrich S, Whittaker S, Robson A, Cerroni L, Massone C, et al. CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer. J Clin Pathol. 2007;60(9):981–9.

    Article  PubMed  Google Scholar 

  51. Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JK, et al. Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology. 2002;41(1):1–29.

    Article  CAS  PubMed  Google Scholar 

  52. Vermi W, Facchetti F, Rosati S, Vergoni F, Rossi E, Festa S, et al. Nodal and extranodal tumor-forming accumulation of plasmacytoid monocytes/interferon-producing cells associated with myeloid disorders. Am J Surg Pathol. 2004;28(5):585–95.

    Article  PubMed  Google Scholar 

  53. Borowitz MJ, Chan JKC. T lymphoblastic leukemia/lymphoma. In: Swerdlow SHCE, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 176–8.

    Google Scholar 

  54. Borowitz MJ, Croker BP, Metzgar RS. Lymphoblastic lymphoma with the phenotype of common acute lymphoblastic leukemia. Am J Clin Pathol. 1983;79(3):387–91.

    Article  CAS  PubMed  Google Scholar 

  55. Nabhan C, Tolentino A, Meyer A, Tallman MS. Cutaneous involvement with B-lineage acute lymphoblastic leukemia. Leuk Lymphoma. 2012;53(5):987–9.

    Article  PubMed  Google Scholar 

  56. Boccara O, Laloum-Grynberg E, Jeudy G, Aubriot-Lorton MH, Vabres P, de Prost Y, et al. Cutaneous B-cell lymphoblastic lymphoma in children: a rare diagnosis. J Am Acad Dermatol. 2012;66(1):51–7.

    Article  PubMed  Google Scholar 

  57. Lin P, Jones D, Dorfman DM, Medeiros LJ. Precursor B-cell lymphoblastic lymphoma: a predominantly extranodal tumor with low propensity for leukemic involvement. Am J Surg Pathol. 2000;24:1480–90.

    Article  CAS  PubMed  Google Scholar 

  58. Gallagher G, Chhanabhai M, Song KW, Barnett MJ. Unusual presentation of precursor T-cell lymphoblastic lymphoma: involvement limited to breasts and skin. Leuk Lymphoma. 2007;48:428–30.

    Article  CAS  PubMed  Google Scholar 

  59. Shafer D, Wu H, Al-Saleem T, Reddy K, Borghaei H, Lessin S, et al. Cutaneous precursor B-cell lymphoblastic lymphoma in 2 adult patients: clinicopathologic and molecular cytogenetic studies with a review of the literature. Arch Dermatol. 2008;144(9):1155–62.

    Article  PubMed  Google Scholar 

  60. Lee WJ, Moon HR, Won CH, Chang SE, Choi JH, Moon KC, et al. Precursor B- or T-lymphoblastic lymphoma presenting with cutaneous involvement: a series of 13 cases including 7 cases of cutaneous T-lymphoblastic lymphoma. J Am Acad Dermatol. 2014;70(2):318–25.

    Article  PubMed  Google Scholar 

  61. Suzumiya J, Ohshima K, Kikuchi M, Takeshita M, Akamatsu M, Tashiro K. Terminal deoxynucleotidyl transferase staining of malignant lymphomas in paraffin sections: a useful method for the diagnosis of lymphoblastic lymphoma. J Pathol. 1997;182:86–91.

    Article  CAS  PubMed  Google Scholar 

  62. Savage NM, Johnson RC, Natkunam Y. The spectrum of lymphoblastic, nodal and extranodal T-cell lymphomas: characteristic features and diagnostic dilemmas. Hum Pathol. 2013;44(4):451–71.

    Article  PubMed  Google Scholar 

  63. Chimenti S, Fink-Puches R, Peris K, Pescarmona E, Pütz B, Kerl H, et al. Cutaneous involvement in lymphoblastic lymphoma. J Cutan Pathol. 1999;26(8):379–85.

    Article  CAS  PubMed  Google Scholar 

  64. Ishibashi M, Yamamoto K, Kudo S, Chen KR. Mantle cell lymphoma with skin invasion characterized by the common variant in the subcutis and blastoid transformation in the overlying dermis. Am J Dermatopathol. 2010;32(2):180–2.

    Article  PubMed  Google Scholar 

  65. Cao Q, Li Y, Lin H, Ke Z, Liu Y, Ye Z. Mantle cell lymphoma of blastoid variant with skin lesion and rapid progression: a case report and literature review. Am J Dermatopathol. 2013;35(8):851–5.

    Article  PubMed  Google Scholar 

  66. Motegi S, Okada E, Nagai Y, Tamura A, Ishikawa O. Skin manifestation of mantle cell lymphoma. Eur J Dermatol. 2006;16(4):435–8.

    PubMed  Google Scholar 

  67. Shi Y, Wang E. Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic review. Arch Pathol Lab Med. 2014;138(4):564–9.

    Article  PubMed  Google Scholar 

  68. Machado I, Traves V, Cruz J, Llombart B, Navarro S, Llombart-Bosch A. Superficial small round-cell tumors with special reference to the Ewing’s sarcoma family of tumors and the spectrum of differential diagnosis. Semin Diagn Pathol. 2013;30(1):85–94.

    Article  PubMed  Google Scholar 

  69. Kolhe R, Reid MD, Lee JR, Cohen C, Ramalingam P. Immunohistochemical expression of PAX5 and TdT by Merkel cell carcinoma and pulmonary small cell carcinoma: a potential diagnostic pitfall but useful discriminatory marker. Int J Clin Exp Pathol. 2013;6(2):142–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  70. Buresh CJ, Oliai BR, Miller RT. Reactivity with TdT in Merkel cell carcinoma: a potential diagnostic pitfall. Am J Clin Pathol. 2008;129(6):894–8.

    Article  PubMed  Google Scholar 

  71. Horny HP, Metcalfe DD, Bennett JM, Bain BJ. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon France: IARC Press; 2008. p. 54–63.

    Google Scholar 

  72. Berezowska S, Flaig MJ, Ruëff F, Walz C, Haferlach T, Krokowski M, et al. Adult-onset mastocytosis in the skin is highly suggestive of systemic mastocytosis. Mod Pathol. 2014;27(1):19–29.

    Article  CAS  PubMed  Google Scholar 

  73. Valent P, Escribano L, Broesby-Olsen S, Hartmann K, Grattan C, Brockow K, et al. Proposed diagnostic algorithm for patients with suspected mastocytosis: a proposal of the European Competence Network on Mastocytosis. Allergy. 2014;69(10):1267–74.

    Article  CAS  PubMed  Google Scholar 

  74. Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest. 2007;37(6):435–53.

    Article  CAS  PubMed  Google Scholar 

  75. Kiszewski AE, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol. 2004;18(3):285–90.

    Article  CAS  PubMed  Google Scholar 

  76. Wolff K, Komar M, Petzelbauer P. Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res. 2001;25(7):519–28.

    Article  CAS  PubMed  Google Scholar 

  77. Chiu A, Orazi A. Mastocytosis and related disorders. Semin Diagn Pathol. 2012;29(1):19–30.

    Article  PubMed  Google Scholar 

  78. Hollmann TJ, Brenn T, Hornick JL. CD25 expression on cutaneous mast cells from adult patients presenting with urticaria pigmentosa is predictive of systemic mastocytosis. Am J Surg Pathol. 2008;32(1):139–45.

    Article  PubMed  Google Scholar 

  79. Morgado JM, Sánchez-Muñoz L, Teodósio CG, Jara-Acevedo M, Alvarez-Twose I, Matito A, et al. Immunophenotyping in systemic mastocytosis diagnosis: “CD25 positive” alone is more informative than the “CD25 and/or CD2” WHO criterion. Mod Pathol. 2012;25(4):516–21.

    Article  CAS  PubMed  Google Scholar 

  80. Valent P, Cerny-Reiterer S, Herrmann H, Mirkina I, George TI, Sotlar K, et al. Phenotypic heterogeneity, novel diagnostic markers, and target expression profiles in normal and neoplastic human mast cells. Best Pract Res Clin Haematol. 2010;23(3):369–78.

    Article  CAS  PubMed  Google Scholar 

  81. Morgado JM, Perbellini O, Johnson RC, Teodósio C, Matito A, Álvarez-Twose I, et al. CD30 expression by bone marrow mast cells from different diagnostic variants of systemic mastocytosis. Histopathology. 2013;63(6):780–7.

    Article  PubMed  Google Scholar 

  82. Sotlar K, Cerny-Reiterer S, Petat-Dutter K, Hessel H, Berezowska S, Müllauer L, et al. Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis. Mod Pathol. 2011;24(4):585–95.

    Article  CAS  PubMed  Google Scholar 

  83. Baek JO, Kang HK, Na SY, Lee JR, Roh JY, Lee JH, et al. N822K c-kit mutation in CD30-positive cutaneous pleomorphic mastocytosis after germ cell tumour of the ovary. Br J Dermatol. 2012;166(6):1370–3.

    Article  CAS  PubMed  Google Scholar 

  84. Yanagihori H, Oyama N, Nakamura K, Kaneko F. c-kit Mutations in patients with childhood-onset mastocytosis and genotype-phenotype correlation. J Mol Diagn. 2005;7(2):252–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  85. Bodemer C, Hermine O, Palmérini F, Yang Y, Grandpeix-Guyodo C, Leventhal PS, et al. Pediatric mastocytosis is a clonal disease associated with D816V and other activating c-KIT mutations. J Invest Dermatol. 2010;130(3):804–15.

    Article  CAS  PubMed  Google Scholar 

  86. Ma D, Stence AA, Bossler AB, Hackman JR, Bellizzi AM. Identification of KIT activating mutations in paediatric solitary mastocytoma. Histopathology. 2014;64(2):218–25.

    Article  PubMed  Google Scholar 

  87. Badalian-Very G, Vergilio JA, Fleming M, Rollins BJ. Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 2013;8:1–20.

    Article  CAS  PubMed  Google Scholar 

  88. Jaffe R, Weis LM, Facchetti F. Tumours derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2008. p. 358–630.

    Google Scholar 

  89. 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(3):157–66.

    Article  CAS  PubMed  Google Scholar 

  90. Valentín-Nogueras SM, Seijo-Montes R, Montalván-Miró E, Sánchez JL. Langerhans cell sarcoma: a case report. J Cutan Pathol. 2013;40(7):670–5.

    Article  PubMed  Google Scholar 

  91. Valladeau J, Duvert-Frances V, Pin JJ, Dezutter-Dambuyant C, Vincent C, Massacrier C, et al. The monoclonal antibody DCGM4 recognizes Langerin, a protein specific of Langerhans cells, and is rapidly internalized from the cell surface. Eur J Immunol. 1999;29(9):2695–704.

    Article  CAS  PubMed  Google Scholar 

  92. 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 

  93. Lau SK, Chu PG, Weiss LM. Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. Am J Surg Pathol. 2008;32(4):615–9.

    Article  PubMed  Google Scholar 

  94. Roden AC, Hu X, Kip S, Parrilla Castellar ER, Rumilla KM, Vrana JA, et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and mmunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol. 2014;38(4):548–51.

    Article  PubMed  Google Scholar 

  95. Méhes G, Irsai G, Bedekovics J, Beke L, Fazakas F, Rózsa 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(12):1644–8.

    Article  PubMed  Google Scholar 

  96. Sagransky MJ, Deng AC, Magro CM. Primary cutaneous langerhans cell sarcoma: a report of four cases and review of the literature. Am J Dermatopathol. 2013;35(2):196–204.

    Article  PubMed  Google Scholar 

  97. Badalian-Very G, Virgilio J, 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 

  98. 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 

  99. Hicks MJ, Bonilla DL, Dwyer KC, Berres ML, Poulikakos PI, Merad M, 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  PubMed  PubMed Central  CAS  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  101. Kansal R, Quintanilla-Martinez L, Datta V, Lopategui J, Garshfield G, Nathwani BN. Identification of the V600D mutation in Exon 15 of the BRAF oncogene in congenital, benign langerhans cell histiocytosis. Genes Chromosomes Cancer. 2013;52(1):99–106.

    Article  CAS  PubMed  Google Scholar 

  102. Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 2013;121(9):1495–500.

    Article  CAS  PubMed  Google Scholar 

  103. Baiocchi RA. Driving toward targeted therapy for LCH. Blood. 2014;124(10):1546–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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

    Article  PubMed  Google Scholar 

  105. 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 

  106. Weis LM, Chan JKC, Fletcher CDM. Other rare dendritic cell tumors. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2008. p. 365.

    Google Scholar 

  107. Ferran M, Toll A, Gilaberte M, Barranco C, Lloreta J, Pujol RM. Acquired mucosal indeterminate cell histiocytoma. Pediatr Dermatol. 2007;24(3):253–6.

    Article  PubMed  Google Scholar 

  108. Tardío JC, Aguado M, Borbujo J. Self-regressing S100-negative CD1a-positive cutaneous histiocytosis. Am J Dermatopathol. 2013;35(4):57–9.

    Article  Google Scholar 

  109. Calatayud M, Güell JL, Gris O, Puig J, Arrondo E, Huguet P. Ocular involvement in a case of systemic indeterminate cell histiocytosis: a case report. Cornea. 2001;20(7):769–71.

    Article  CAS  PubMed  Google Scholar 

  110. Ratzinger G, Burgdorf WH, Metze D, Zelger BG, Zelger B. Indeterminate cell histiocytosis: fact or fiction? J Cutan Pathol. 2005;32(8):552–60.

    Article  PubMed  Google Scholar 

  111. Ventura F, Pereira T, da Luz DM, Marques H, Pardal F, Brito C. Indeterminate cell histiocytosis in association with acute myeloid leukemia. Dermatol Res Pract. 2010;2010:569345.

    PubMed  PubMed Central  Google Scholar 

  112. Buser L, Bihl M, Rufle A, Mickys U, Tavoriene I, Griskevicius L, et al. Unique composite hematolymphoid tumor consisting of a pro-T lymphoblastic lymphoma and an indeterminate dendritic cell tumor: evidence for divergent common progenitor cell differentiation. Pathobiology. 2014;81(4):199–205.

    Article  PubMed  Google Scholar 

  113. 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 

  114. Zelger BG, Zelger B, Steiner H, Mikuz G. Solitary giant xanthogranuloma and benign cephalic histiocytosis—variants of juvenile xanthogranuloma. Br J Dermatol. 1995;133(4):598–604. 40.

    Article  CAS  PubMed  Google Scholar 

  115. Torrelo A, Juarez A, Hernández A, Colmenero I. Multiple lichenoid juvenile xanthogranuloma. Pediatr Dermatol. 2009;26(2):238.

    Article  PubMed  Google Scholar 

  116. Koren J, Matecek L, Zamecnik M. Mitotically active deep juvenile xanthogranuloma. Ann Diagn Pathol. 2010;14(1):36–40.

    Article  PubMed  Google Scholar 

  117. Newman CC, Raimer SS, Sánchez RL. Nonlipidized juvenile xanthogranuloma: a histologic and immunohistochemical study. Pediatr Dermatol. 1997;14(2):98–102.

    Article  CAS  PubMed  Google Scholar 

  118. Ngendahayo P, de Saint Aubain N. Mitotically active xanthogranuloma: a case report with review of the literature. Am J Dermatopathol. 2012;34(3):e27–30.

    Article  PubMed  Google Scholar 

  119. Fenot M, Stalder JF, Barbarot S. Juvenile xanthogranulomas are highly prevalent but transient in young children with neurofibromatosis type 1. J Am Acad Dermatol. 2014;71(2):389–90.

    Article  PubMed  Google Scholar 

  120. Iyengar V, Golomb CA, Schachner L. Neurilemmomatosis, NF2, and juvenile xanthogranuloma. J Am Acad Dermatol. 1998;39(5 Pt 2):831–4.

    Article  CAS  PubMed  Google Scholar 

  121. Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol. 1995;131(8):904–8.

    Article  CAS  PubMed  Google Scholar 

  122. Shin HT, Harris MB, Orlow SJ. Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas. J Pediatr Hematol Oncol. 2004;26(9):591–5.

    Article  PubMed  Google Scholar 

  123. Tran DT, Wolgamot GM, Olerud J, Hurst S, Argenyi Z. An “eruptive” variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis. J Cutan Pathol. 2008;35 Suppl 1:50–4.

    Article  PubMed  Google Scholar 

  124. Gonzalez Ruíz A, Bernal Ruíz AI, Aragoneses Fraile H, Peral Martinez I, García MM. Progressive nodular histiocytosis accompanied by systemic disorders. Br J Dermatol. 2000;143(3):628–31.

    Article  PubMed  Google Scholar 

  125. Lüftl M, Seybold H, Simon Jr M, Burgdorf W. Progressive nodular histiocytosis—rare variant of cutaneous non-Langerhans cell histiocytosis. J Dtsch Dermatol Ges. 2006;4(3):236–8.

    Article  PubMed  Google Scholar 

  126. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine. 1996;75(3):157–69.

    Article  CAS  PubMed  Google Scholar 

  127. Mazor RD, Manervich-Mazor M, Shoenfeld Y. Erdheim Chester Disease: A comprehensive review of the literature. Orphanet J Rare Dis. 2013;8:137.

    Google Scholar 

  128. 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 

  129. Tidwell WJ, Googe PB. Tissue histiocyte reactivity with CD31 is comparable to CD68 and CD163 in common skin lesions. J Cutan Pathol. 2014;41(6):489–93.

    Article  PubMed  Google Scholar 

  130. Pouryazdanparast P, Yu L, Cutlan JE, Olsen SH, Fullen DR, Ma L. Diagnostic value of CD163 in cutaneous spindle cell lesions. J Cutan Pathol. 2009;36(8):859–64.

    Article  PubMed  Google Scholar 

  131. Tomaszewski MM, Lupton GP. Unusual expression of S-100 protein in histiocytic neoplasms. J Cutan Pathol. 1998;25(3):129–35.

    Article  CAS  PubMed  Google Scholar 

  132. Amouri M, Gouiaa N, Chaaben H, Masmoudi A, Zahaf A, Boudawara T, et al. Disseminated juvenile xanthogranuloma expressing protein S100. Ann Dermatol Venereol. 2012;139(2):128–31.

    Article  CAS  PubMed  Google Scholar 

  133. Yamamoto Y, Kadota M, Nishimura Y. A case of S-100-positive juvenile xanthogranuloma: a longitudinal observation. Pediatr Dermatol. 2009;26(4):475–6.

    Article  PubMed  Google Scholar 

  134. Perez-Becker R, Szczepanowski M, Leuschner I, Janka G, Gokel M, Imschweiler T, et al. An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T-cell acute lymphoblastic leukemia. Pediatr Blood Cancer. 2011;56(5):859–62.

    Article  PubMed  Google Scholar 

  135. Emile JF, Diamond EL, Hélias-Rodzewicz Z, Charlotte F, Hyman DM, Kim E, et al. Recurent RAS and PIK3CA mutations in Erdheim-Chester disease. Blood. 2014;124(19):3016–09.

    Google Scholar 

  136. Haroche J, Charlotte F, Arnaud L, von Deimling A, Hélias-Rodzewicz Z, et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. Blood. 2012;120(13):2700–3.

    Article  CAS  PubMed  Google Scholar 

  137. Bubolz AM, Weissinger SE, Stenzinger A, Arndt A, Steinestel K, Brüderlein S, et al. Potential clinical implications of BRAF mutations in histiocytic proliferations. Oncotarget. 2014;5(12):4060–70.

    Article  PubMed  PubMed Central  Google Scholar 

  138. Janku F, Vibat CR, Kosco K, Holley VR, Cabrilo G, Meric-Bernstam F, et al. BRAF V600E mutations in urine and plasma cell-free DNA from patients with Erdheim-Chester disease. Oncotarget. 2014;5(11):3607–10.

    Article  PubMed  PubMed Central  Google Scholar 

  139. Cavalli G, Biavasco R, Borgiani B, Dagna L. Oncogene-induced senescence as a new mechanism of disease: the paradigm of Erdheim-Chester disease. Front Immunol. 2014;5:281. doi:10.3389/fimmu.2014.00281.eCollection2014.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  140. Aldabagh B, Ly MN, Hessel AB, Usmani AS. Molluscum contagiosum involving an epidermoid cyst with xanthogranuloma-like reaction in an HIV-infected patient. J Cutan Pathol. 2010;37(2):282–6.

    Article  PubMed  Google Scholar 

  141. Nakamura Y, Nakamura A, Muto M. Solitary spindle cell xanthogranuloma mimicking a spitz nevus. Am J Dermatopathol. 2013;35(8):865–7.

    Article  PubMed  Google Scholar 

  142. Berti S, Coronella G, Galeone M, Balestri R, Patrizi A, Neri I. Giant congenital juvenile xanthogranuloma. Arch Dis Child. 2013;98(4):317.

    Article  PubMed  Google Scholar 

  143. Cohen PR, Prieto VG. Radiation port xanthogranuloma: solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient-report and review of cutaneous neoplasms developing at the site of radiotherapy. J Cutan Pathol. 2010;37(8):891–4.

    Article  PubMed  Google Scholar 

  144. Shimada R, Fujimura T, Kambayashi Y, Ohtani T, Nasu M, Numata Y, et al. CD163+ adult xanthogranuloma arising from Merkel cell carcinoma treated with local radiotherapy. Acta Derm Venereol. 2012;92(6):631–2.

    Article  PubMed  Google Scholar 

  145. Janka GE, Lehmberg K. Hemophagocytic syndromes—an update. Blood Rev. 2014;28(4):135–42.

    Article  PubMed  Google Scholar 

  146. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383(9927):1503.

    Article  PubMed  Google Scholar 

  147. Li J, Wang Q, Zheng W, Ma J, Zhang W, Wang W, et al. Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. Medicine. 2014;93(2):100–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  148. Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology Am Soc Hematol Educ Program. 2013;2013:605–11.

    PubMed  Google Scholar 

  149. Zhang K, Jordan MB, Marsh RA, Johnson JA, Kissell D, Meller J, et al. Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult onset familial HLH. Blood. 2011;118(22):5794–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  150. Jessen B, Bode SF, Ammann S, Chakravorty S, Davies G, Diestelhorst J, et al. The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2. Blood. 2013;121(15):2943–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  151. Lin CI, Yu HH, Lee JH, Wang LC, Lin YT, Yang YH, et al. Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. Clin Rheumatol. 2012;31(8):1223–30.

    Article  PubMed  Google Scholar 

  152. Risma K, Jordan MB. Hemophagocytic lymphohistiocytosis: updates and evolving concepts. Curr Opin Pediatr. 2012;24(1):9–15.

    Article  CAS  PubMed  Google Scholar 

  153. Brisse E, Wouters CH, Matthys P. Hemophagocytic lymphohistiocytosis (HLH): a heterogeneous spectrum of cytokine-driven immune disorders. Cytokine Growth Factor Rev. 2014. doi:10.1016/j.cytogfr.2014.10.001.

    PubMed  Google Scholar 

  154. Kerl K, Wolf IH, Cerroni L, Wolf P, French LE, Kerl H. Hemophagocytosis in cutaneous autoimmune disease. Am J Dermatopathol. 2015;37(7):539–43.

    Google Scholar 

  155. Schaer DJ, Schleiffenbaum B, Kurrer M, Imhof A, Bächli E, Fehr J, et al. Soluble hemoglobinhaptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome. Eur J Haematol. 2005;74:6–10.

    Article  CAS  PubMed  Google Scholar 

  156. Wada T, Sakakibara Y, Nishimura R, Toma T, Ueno Y, Horita S, et al. Down-regulation of CD5 expression on activated CD8+ T cells in familial hemophagocytic lymphohistiocytosis with perforin gene mutations. Hum Immunol. 2013;74(12):1579–85.

    Article  CAS  PubMed  Google Scholar 

  157. Ohkuma K, Saraya T, Sada M, Kawai S. Evidence for cytomegalovirus-induced haemophagocytic syndrome in a young patient with AIDS. BMJ Case Rep. 2013; 2013.

    Google Scholar 

  158. Ito Y, Kawamura Y, Iwata S, Kawada J, Yoshikawa T, Kimura H. Demonstration of type II latency in T lymphocytes of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2013;60(2):326–8.

    Article  PubMed  Google Scholar 

  159. Ahn JS, Rew SY, Shin MG, Kim HR, Kim HR, Yang DH, et al. Clinical significance of clonality and Epstein-Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2010;85:719–22.

    Article  PubMed  Google Scholar 

  160. Smith MC, Cohen DN, Greig B, Yenamandra A, Vnencak-Jones C, Thompson MA, et al. The ambiguous boundary between EBV-related hemophagocytic lymphohistiocytosis and systemic EBV-driven T cell lymphoproliferative disorder. Int J Clin Exp Pathol. 2014;7(9):5738–49.

    PubMed  PubMed Central  Google Scholar 

  161. Draper NL, Morgan MB. Dermatologic perivascular hemophagocytosis: a report of two cases. Am J Dermatopathol. 2007;29(5):467–9.

    Article  PubMed  Google Scholar 

  162. Valentín SM, Montalván E, Sánchez JL. Perivascular hemophagocytosis: report of 2 cases and review of the literature. Am J Dermatopathol. 2010;32(7):716–9.

    Article  PubMed  Google Scholar 

  163. 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 

  164. Sroa N, Zirwas MJ, Bechtel M. Multicentric reticulohistiocytosis: a case report and review of the literature. Cutis. 2010;85(3):153–5.

    PubMed  Google Scholar 

  165. Baek IW, Yoo SH, Yang H, Park J, Kim KJ, Cho CS. A case of multicentric reticulohistiocytosis. Mod Rheumatol. 2014:1–4. [Epub ahead of print] PMID: 25211404.

    Google Scholar 

  166. Kandiah DA. Multicentric reticulohistiocytosis. Mayo Clin Proc. 2014;89(8):e73. doi:10.1016/j.mayocp.2013.10.036.

    Article  PubMed  Google Scholar 

  167. Lu YY, Lu CC, Wu CH. Leonine facies in the cutaneous form of multicentric reticulohistiocytosis. Intern Med. 2012;51(15):2069–70.

    Article  PubMed  Google Scholar 

  168. West KL, Sporn T, Puri PK. Multicentric reticulohistiocytosis: a unique case with pulmonary fibrosis. Arch Dermatol. 2012;148(2):228–32.

    Article  PubMed  Google Scholar 

  169. Yang HJ, Ding YQ, Deng YJ. Multicentric reticulohistiocytosis with lungs and liver involved. Clin Exp Dermatol. 2009;34(2):183–5.

    Article  PubMed  Google Scholar 

  170. Webb-Detiege T, Sasken H, Kaur P. Infiltration of histiocytes and multinucleated giant cells in the myocardium of a patient with multicentric reticulohistiocytosis. J Clin Rheumatol. 2009;15(1):25–6.

    Article  PubMed  Google Scholar 

  171. Taniguchi T, Asano Y, Okada A, Sugaya M, Sato S. Ultraviolet light-induced Köbner phenomenon contributes to the development of skin eruptions in multicentric reticulohistiocytosis. Acta Derm Venereol. 2011;91(2):160–3.

    Article  PubMed  Google Scholar 

  172. Arai S, Katsuoka K, Ishikawa A. Multicentric reticulohistiocytosis presenting with the cutaneous features of photosensitivity dermatitis. J Dermatol. 2012;39(2):180–1.

    Article  PubMed  Google Scholar 

  173. Bennàssar A, Mas A, Guilabert A, Julià M, Mascaró-Galy JM, Herrero C. Multicentric reticulohistiocytosis with elevated cytokine serum levels. J Dermatol. 2011;38(9):905–10.

    PubMed  Google Scholar 

  174. Kovach BT, Calamia KT, Walsh JS, Ginsburg WW. Treatment of multicentric reticulohistiocytosis with etanercept. Arch Dermatol. 2004;140(8):919–21.

    Article  PubMed  Google Scholar 

  175. Lovelace K, Loyd A, Adelson D, Crowson N, Taylor JR, Cornelison R. Etanercept and the treatment of multicentric reticulohistiocytosis. Arch Dermatol. 2005;141(9):1167–8.

    Article  PubMed  Google Scholar 

  176. Iwata H, Okumura Y, Seishima M, Aoyama Y. Overexpression of monocyte chemoattractant protein-1 in the overlying epidermis of multicentric reticulohistiocytosis lesions: a case report. Int J Dermatol. 2012;51(4):492–4.

    Article  PubMed  Google Scholar 

  177. Codriansky KA, Rünger TM, Bhawan J, Kantarci A, Kissin EY. Multicentric reticulohistiocytosis: a systemic osteoclastic disease? Arthritis Rheum. 2008;59(3):444–8. doi:10.1002/art.23320.

    Article  PubMed  Google Scholar 

  178. Nicol C, Quereux G, Renaut JJ, Renac F, Dreno B. Paraneoplastic multicentric reticulohistiocytosis. Ann Dermatol Venereol. 2011;138(5):405–8.

    Article  CAS  PubMed  Google Scholar 

  179. Ben Abdelghani K, Mahmoud I, Chatelus E, Sordet C, Gottenberg JE, Sibilia J. Multicentric reticulohistiocytosis: an autoimmune systemic disease? Case report of an association with erosive rheumatoid arthritis and systemic Sjogren syndrome. Joint Bone Spine. 2010;77(3):274–6.

    Article  PubMed  Google Scholar 

  180. Macía-Villa CC, Zea-Mendoza A. Multicentric reticulohistiocytosis: case report with response to infliximab and review of treatment options. Clin Rheumatol. 2016;35(2):527–34.

    Google Scholar 

  181. Zelger B, Cerio R, Soyer HP, Misch K, Orchard G, Wilson-Jones E. Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. Am J Dermatopathol. 1994;16(6):577–84.

    Article  CAS  PubMed  Google Scholar 

  182. Miettinen M, Fetsch JF. Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases. Am J Surg Pathol. 2006;30(4):521–8.

    Article  PubMed  Google Scholar 

  183. Bialynicki-Birula R, Sebastian-Rusin A, Maj J, Wozniak Z, Baran E, Dziegiel P. Multicentric reticulohistiocytosis with S100 protein positive staining: a case report. Acta Dermatovenerol Croat. 2010;18(1):35–7.

    PubMed  Google Scholar 

  184. Perrin C, Lacour JP, Michiels JF, Flory P, Ziegler G, Ortonne JP. Multicentric reticulohistiocytosis. Immunohistological and ultrastructural study: a pathology of dendritic cell lineage. Am J Dermatopathol. 1992;14(5):418–25.

    Article  CAS  PubMed  Google Scholar 

  185. Tashiro A, Takeuchi S, Nakahara T, Oba J, Tsujita J, Fukushi J, et al. Aberrant expression of CD10 in ground-glass-like multinucleated giant cells of multicentric reticulohistiocytosis. J Dermatol. 2010;37(11):995–7.

    Article  PubMed  Google Scholar 

  186. Tan BH, Barry CI, Wick MR, White KP, Brown JG, Lee A, et al. Multicentric reticulohistiocytosis and urologic carcinomas: a possible paraneoplastic association. J Cutan Pathol. 2011;38(1):43–8.

    Article  PubMed  Google Scholar 

  187. Krause ML, Lehman JS, Warrington KJ. Multicentric reticulohistiocytosis can mimic rheumatoid arthritis. J Rheumatol. 2014;41(4):780–1.

    Article  PubMed  Google Scholar 

  188. Trotta F, Colina M. Multicentric reticulohistiocytosis and fibroblastic rheumatism. Best Pract Res Clin Rheumatol. 2012;26(4):543–57.

    Article  CAS  PubMed  Google Scholar 

  189. Mun JH, Ko HC, Kim MB. Multicentric reticulohistiocytosis masquerading as dermatomyositis: similar and different features. J Dermatol. 2012;39(1):104–7.

    Article  PubMed  Google Scholar 

  190. Fett N, Liu RH. Multicentric reticulohistiocytosis with dermatomyositis-like features: a more common disease presentation than previously thought. Dermatology. 2011;222(2):102–8.

    Article  PubMed  Google Scholar 

  191. Spadaro A, Riccieri V, Sili-Scavalli A, Innocenzi D, Pranteda G, Taccari E. Value of cytological analysis of the synovial fluid in multicentric reticulohistiocytosis. A case. Rev Rhum Ed Fr. 1994;61(7–8):554–7.

    CAS  PubMed  Google Scholar 

  192. Zak FG. Reticulohistiocytoma (“ganglioneuroma”) of the skin. Br J Dermatol Syph. 1950;62(9):351–5.

    Article  CAS  PubMed  Google Scholar 

  193. Rabkin MS, Vukmer T. Granular cell variant of epithelioid cell histiocytoma. Am J Dermatopathol. 2012;34(7):766–9.

    Article  PubMed  Google Scholar 

  194. Caltabiano R, Magro G, Vecchio GM, Lanzafame S. Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma? J Cutan Pathol. 2010;37(2):287–91.

    Article  PubMed  Google Scholar 

  195. Jedrych J, Nikiforova M, Kennedy TF, Ho J. Epithelioid cell histiocytoma of the skin with clonal ALK gene rearrangement resulting in VCL-ALK and SQSTM1-ALK gene fusions. Br J Dermatol. 2015;37(7):539–43.

    Google Scholar 

  196. Miettinen M, Fernandez M, Franssila K, Gatalica Z, Lasota J, Sarlomo-Rikala M. Microphthalmia transcription factor in the immunohistochemical diagnosis of metastatic melanoma: comparison with four other melanoma markers. Am J Surg Pathol. 2001;25(2):205–11.

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  198. Brenn T, Calonje E, Granter SR, Leonard N, Grayson W, Fletcher CD, et al. Cutaneous rosai-dorfman disease is a distinct clinical entity. Am J Dermatopathol. 2002;24(5):385–91.

    Article  PubMed  Google Scholar 

  199. Zhu F, Zhang JT, Xing XW, Wang DJ, Zhu RY, Zhang Q, et al. Rosai-Dorfman disease: a retrospective analysis of 13 cases. Am J Med Sci. 2013;345:200–10.

    Article  PubMed  Google Scholar 

  200. Frater JL, Maddox JS, Obadiah JM, Hurley MY. Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg. 2006;10(6):281–90.

    Article  PubMed  Google Scholar 

  201. Fernandez-Vega I, Santos-Juanes J, Ramsay A. Rosai-Dorfman disease following a classical Hodgkin lymphoma, nodular sclerosis subtype. Am J Dermatopathol. 2014;36(3):280–1.

    Article  PubMed  Google Scholar 

  202. Llamas-Velasco M, Cannata J, Dominguez I, García-Noblejas A, Aragües 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 

  203. Venkataraman G, McClain KL, Pittaluga S, Rao VK, Jaffe ES. Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease. Am J Surg Pathol. 2010;34(4):589–94.

    Article  PubMed  PubMed Central  Google Scholar 

  204. Arakaki N, Gallo G, Majluf R, Diez B, Arias E, Riudavets MA, et al. Extranodal Rosai-Dorfman disease presenting as a solitary mass with human herpesvirus 6 detection in a pediatric patient. Pediatr Dev Pathol. 2012;15(4):324–8.

    Article  PubMed  Google Scholar 

  205. Al-Daraji W, Anandan A, Klassen-Fischer M, Auerbach A, Marwaha JS, Fanburg-Smith JC. Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann Diagn Pathol. 2010;14(5):309–16.

    Article  PubMed  Google Scholar 

  206. Salva KA, Stenstrom M, Breadon JY, Odland PB, Bennett D, Longley J, et al. Possible association of cutaneous Rosai-Dorfman disease and chronic Crohn disease: a case series report. JAMA Dermatol. 2014;150(2):177–81.

    Article  PubMed  PubMed Central  Google Scholar 

  207. Zhang X, Hyjek E, Vardiman J. A subset of Rosai-Dorfman disease exhibits features of IgG4-related disease. Am J Clin Pathol. 2013;139(5):622–32.

    Article  CAS  PubMed  Google Scholar 

  208. 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 

  209. 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 

  210. Vuong V, Moulonguet I, Cordoliani F, Crickx B, Bezier M, Vignon-Pennamen MD, et al. Cutaneous revelation of Rosai-Dorfman disease: 7 cases. Ann Dermatol Venereol. 2013;140(2):83–90.

    Article  CAS  PubMed  Google Scholar 

  211. Cangelosi JJ, Prieto VG, Ivan D. Cutaneous Rosai-Dorfman disease with increased number of eosinophils: coincidence or histologic variant? Arch Pathol Lab Med. 2011;135(12):1597–600.

    Article  PubMed  Google Scholar 

  212. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry. Cancer. 1984;54:1834–40.

    Article  CAS  PubMed  Google Scholar 

  213. Sasaki K, Pemmaraju N, Westin JR, Wang WL, Khoury JD, Podoloff DA, et al. A single case of Rosai-Dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine. Front Oncol. 2014;4:297.

    Article  PubMed  PubMed Central  Google Scholar 

  214. Zheng M, Bi R, Li W, Landeck L, Chen JQ, Lao LM, et al. Generalized pure cutaneous Rosai-Dorfman disease: a link between inflammation and cancer not associated with mitochondrial DNA and SLC29A3 gene mutation? Discov Med. 2013;16(89):193–200.

    PubMed  Google Scholar 

  215. Avitan-Hersh E, Mandel H, Indelman M, Bar-Joseph G, Zlotogorski A, Bergman R. A case of H syndrome showing immunophenotye similarities to Rosai-Dorfman disease. Am J Dermatopathol. 2011;33(1):47–51.

    Article  PubMed  Google Scholar 

  216. 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 

  217. Molho-Pessach V, Ramot Y, Camille F, Doviner V, Babay S, Luis SJ, et al. H syndrome: the first 79 patients. J Am Acad Dermatol. 2014;70(1):80–8.

    Article  PubMed  Google Scholar 

  218. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn. 1972;35:379–80.

    Google Scholar 

  219. Kim TY, Ha KS, Kim Y, Lee J, Lee K, Lee J. Characteristics of Kikuchi-Fujimoto disease in children compared with adults. Eur J Pediatr. 2014;173(1):111–6.

    Article  PubMed  Google Scholar 

  220. Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. 2004;122(1):141–52.

    Article  PubMed  Google Scholar 

  221. Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi-Fujimoto disease: analysis of 244 cases. Clin Rheumatol. 2007;26(1):50–4.

    Article  PubMed  Google Scholar 

  222. Cheng CY, Sheng WH, Lo YC, Chung CS, Chen YC, Chang SC. Clinical presentations, laboratory results and outcomes of patients with Kikuchi’s disease: emphasis on the association between recurrent Kikuchi’s disease and autoimmune diseases. J Microbiol Immunol Infect. 2010;43(5):366–71.

    Article  PubMed  Google Scholar 

  223. Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi’s disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol. 2008;59(1):130–6.

    Article  PubMed  Google Scholar 

  224. Kim JH, Kim YB, In SI, Kim YC, Han JH. The cutaneous lesions of Kikuchi’s disease: a comprehensive analysis of 16 cases based on the clinicopathologic, immunohistochemical, and immunofluorescence studies with an emphasis on the differential diagnosis. Hum Pathol. 2010;41(9):1245–54.

    Article  PubMed  Google Scholar 

  225. Kuo TT. Kikuchi’s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol. 1995;19(7):798–809.

    Article  CAS  PubMed  Google Scholar 

  226. Nomura Y, Takeuchi M, Yoshida S, Sugita Y, Niino D, Kimura Y, et al. Phenotype for activated tissue macrophages in histiocytic necrotizing lymphadenitis. Pathol Int. 2009;59(9):631–5.

    Article  CAS  PubMed  Google Scholar 

  227. Pilichowska ME, Pinkus JL, Pinkus GS. Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease): lesional cells exhibit an immature dendritic cell phenotype. Am J Clin Pathol. 2009;131(2):174–82.

    Article  PubMed  Google Scholar 

  228. Ohshima K, Karube K, Hamasaki M, Makimoto Y, Fujii A, Kawano R, et al. Apoptosis- and cell cycle-associated gene expression profiling of histiocytic necrotising lymphadenitis. Eur J Haematol. 2004;72(5):322–9.

    Article  CAS  PubMed  Google Scholar 

  229. Ishimura M, Yamamoto H, Mizuno Y, Takada H, Goto M, Doi T, et al. A non-invasive diagnosis of histiocytic necrotizing lymphadenitis by means of gene expression profile analysis of peripheral blood mononuclear cells. J Clin Immunol. 2013;33(5):1018–26.

    Article  CAS  PubMed  Google Scholar 

  230. Robertson KE, Forsyth PD, Batstone PJ, Levison DA, Goodlad JR. Kikuchi’s disease displaying a t(2:16) chromosomal translocation. J Clin Pathol. 2007;60(4):433–5.

    Article  PubMed  PubMed Central  Google Scholar 

  231. Requena L, El-Shabrawi-Caelen L, Walsh SN, Segura S, Ziemer M, Hurt MA, et al. Intralymphatic histiocytosis: a clinicopathologic study of 16 cases. Am J Dermatopathol. 2009;31:140–51.

    Article  PubMed  Google Scholar 

  232. Bakr F, Webber N, Fassihi H, Swale V, Lewis F, Rytina E, et al. Primary and secondary intralymphatic histiocytosis. J Am Acad Dermatol. 2014;70(5):927–33.

    Article  PubMed  Google Scholar 

  233. Watanabe T, Yamada N, Yoshida Y, Yamamoto O. Intralymphatic histiocytosis with granuloma formation associated with orthopedic metal implants. Br J Dermatol. 2008;158:402–4.

    CAS  PubMed  Google Scholar 

  234. O’Grady JT, Shahidullah H, Doherty VR, Al-Nafussi A. Intravascular histiocytosis. Histopathology. 1994;24:265–8.

    Article  PubMed  Google Scholar 

  235. Rossari S, Scatena C, Gori A, Grazzini M, Corciova S, Scarfì F, et al. Intralymphatic histiocytosis: cutaneous nodules and metal implants. J Cutan Pathol. 2011;38(6):534–5.

    Article  PubMed  Google Scholar 

  236. Echeverrıa-Garcıa B, Botella-Estrada R, Requena C, Guillen C. Intralymphatic histiocytosis and cancer of the colon. Actas Dermosifiliogr. 2010;101:257–62.

    Article  PubMed  Google Scholar 

  237. Park YJ, Kwon JE, Han JH, Kim CH, Kang HY. Intralymphatic histiocytosis mimicking oral lymphangioma circumscriptum. Am J Dermatopathol. 2014;36(9):759–61.

    Article  PubMed  Google Scholar 

  238. Rieger E, Soyer HP, Leboit PE, Metze D, Slovak R, Kerl H. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation. Br J Dermatol. 1999;140(3):497–504.

    Article  CAS  PubMed  Google Scholar 

  239. Piccolo V, Ruocco E, Russo T, Baroni A. A possible relationship between metal implant-induced intralymphatic histiocytosis and the concept of the immunocompromised district. Int J Dermatol. 2014;53(7), e365.

    Article  PubMed  Google Scholar 

  240. Ruocco V, Brunetti G, Puca RV, Ruocco E. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol. 2009;23(12):1364–73.

    Article  CAS  PubMed  Google Scholar 

  241. Franz R, Andres C. Cheilitis granulomatosa and Melkersson-Rosenthal syndrome. Intralymphatic histiocytosis as valuable diagnostic indication. Pathologe. 2014;35(2):177–81.

    Article  CAS  PubMed  Google Scholar 

  242. Asagoe K, Torigoe R, Ofuji R, Iwatsuki K. Reactive intravascular histiocytosis associated with tonsillitis. Br J Dermatol. 2006;154:560–2.

    Article  CAS  PubMed  Google Scholar 

  243. Pouryazdanparast P, Yu L, Dalton VK, Haefner HK, Brincat C, Mandell SH, et al. Intravascular histiocytosis presenting with extensive vulvar necrosis. J Cutan Pathol. 2009;36 Suppl 1:1–7.

    Article  PubMed  Google Scholar 

  244. Rongioletti F, Rebora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol. 2003;49(5):887–96.

    Article  PubMed  Google Scholar 

  245. Cathébras P, Laurent H, Guichard I. Generalized telangiectasia revealing intravascular large B-cell lymphoma. Br J Haematol. 2013;161(2):155.

    Article  PubMed  Google Scholar 

  246. Riveiro-Falkenbach E, Fernández-Figueras MT, Rodríguez-Peralto JL. Benign atypical intravascular CD30(+) T-cell proliferation: a reactive condition mimicking intravascular lymphoma. Am J Dermatopathol. 2013;35(2):143–50.

    Article  PubMed  Google Scholar 

  247. Adachi Y, Kosami K, Mizuta N, Ito M, Matsuoka Y, Kanata M, et al. Benefits of skin biopsy of senile hemangioma in intravascular large B-cell lymphoma: a case report and review of the literature. Oncol Lett. 2014;7(6):2003–6.

    PubMed  PubMed Central  Google Scholar 

  248. Aung PP, Ballester LY, Goldberg LJ, Bhawan J. Incidental simultaneous finding of intravascular histiocytosis and reactive angioendotheliomatosis: a case report. Am J Dermatopathol. 2015;37(5):401–4.

    Google Scholar 

  249. Grogan TM, Pileri SA, Chan JKC, Weiss LM, Fletcher CDM. Histiocytic sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, et al., editors. World Health Organization Classification of Tumours, WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2008. p. 356–7.

    Google Scholar 

  250. 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 

  251. Ralfkiaer E, Delsol G, O'Connor NT, Brandtzaeg P, Brousset P, Vejlsgaard GL, et al. Malignant lymphomas of true histiocytic origin. A clinical, histological, immunophenotypic and genotypic study. J Pathol. 1990;160(1):9–17.

    Article  CAS  PubMed  Google Scholar 

  252. Kamel OW, Gocke CD, Kell DL, Cleary ML, Warnke RA. True histiocytic lymphoma: a study of 12 cases based on current definition. Leuk Lymphoma. 1995;18(1–2):81–6.

    Article  CAS  PubMed  Google Scholar 

  253. Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004;28(9):1133–44.

    Article  PubMed  Google Scholar 

  254. Nguyen TT, Schwartz EJ, West RB, Warnke RA, Arber DA, Natkunam Y. Expression of CD163 (hemoglobin scavenger receptor) in normal tissues, lymphomas, carcinomas, and sarcomas is largely restricted to the monocyte/macrophage lineage. Am J Surg Pathol. 2005;29(5):617–24.

    Article  PubMed  Google Scholar 

  255. Lau SK, Chu PG, Weiss LM. CD163: a specific marker of macrophages in paraffin-embedded tissue samples. Am J Clin Pathol. 2004;122(5):794–801.

    Article  PubMed  Google Scholar 

  256. Vos JA, Abbondanzo SL, Barekman CL, Andriko JW, Miettinen M, Aguilera NS. Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163. Mod Pathol. 2005;18(5):693–704.

    Article  PubMed  Google Scholar 

  257. Soslow RA, Davis RE, Warnke RA, Cleary ML, Kamel OW. True histiocytic lymphoma following therapy for lymphoblastic neoplasms. Blood. 1996;87(12):5207–12.

    CAS  PubMed  Google Scholar 

  258. Feldman AL, Minniti C, Santi M, Downing JR, Raffeld M, Jaffe ES. Histiocytic sarcoma after acute lymphoblastic leukaemia: a common clonal origin. Lancet Oncol. 2004;5(4):248–50.

    Article  PubMed  Google Scholar 

  259. Chen W, Lau SK, Fong D, Wang J, Wang E, Arber DA, et al. High frequency of clonal immunoglobulin receptor gene rearrangements in sporadic histiocytic/dendritic cell sarcomas. Am J Surg Pathol. 2009;33(6):863–73.

    Article  PubMed  Google Scholar 

  260. McClure R, Khoury J, Feldman A, Ketterling R. Clonal relationship between precursor B-cell acute lymphoblastic leukemia and histiocytic sarcoma: a case report and discussion in the context of similar cases. Leuk Res. 2010;34(2):71–3.

    Article  Google Scholar 

  261. Bouabdallah R, Abéna P, Chetaille B, Aurran-Schleinitz T, Sainty D, Dubus P, et al. True histiocytic lymphoma following B-acute lymphoblastic leukaemia: case report with evidence for a common clonal origin in both neoplasms. Br J Haematol. 2001;113(4):1047–50.

    Article  CAS  PubMed  Google Scholar 

  262. 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 p16INK4A. Pediatr Blood Cancer. 2011;56(2):307–10.

    Article  PubMed  Google Scholar 

  263. Hure MC, Elco CP, Ward D, Hutchinson L, Meng X, Dorfman DM, et al. Histiocytic sarcoma arising from clonally related mantle cell lymphoma. J Clin Oncol. 2012;30(5):49–53.

    Article  Google Scholar 

  264. 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 

  265. Pletneva MA, Smith LB. Anaplastic large cell lymphoma: features presenting diagnostic challenges. Arch Pathol Lab Med. 2014;138(10):1290–4.

    Article  PubMed  Google Scholar 

  266. Wu J, Qin D, Burton GF, Szakal AK, Tew JG. Follicular dendritic cell-derived antigen and accessory activity in initiation of memory IgG responses in vitro. J Immunol. 1996;157(8):3404–11.

    CAS  PubMed  Google Scholar 

  267. Yamada K, Yamakawa M, Imai Y, Tsukamoto M. Expression of cytokine receptors on follicular dendritic cells. Blood. 1997;90(12):4832–41.

    CAS  PubMed  Google Scholar 

  268. Steinman RM, Pack M, Inaba K. Dendritic cells in the T-cell areas of lymphoid organs. Immunol Rev. 1997;156:25–37.

    Article  CAS  PubMed  Google Scholar 

  269. Chan JKC, Pileri SA, Delsol G, Fletcher CDM, Weis LM, Grogg KL. Follicular dendritic cell sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2008. p. 363–4.

    Google Scholar 

  270. Meijs M, Mekkes J, van Noesel C, Nijhuis E, Leeksma O, Jonkman M, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma without Castleman's disease; treatment with rituximab. Int J Dermatol. 2008;47(6):632–4.

    Article  PubMed  Google Scholar 

  271. Lee IJ, Kim SC, Kim HS, Bang D, Yang WI, Jung WH, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman’s tumor. J Am Acad Dermatol. 1999;40:294–7.

    Article  CAS  PubMed  Google Scholar 

  272. Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer. 1997;79(2):294–313.

    Article  CAS  PubMed  Google Scholar 

  273. Cokelaere K, Debiec-Rychter M, De Wolf-Peeters C, Hagemeijer A, Sciot R. Hyaline vascular Castleman’s disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis. Am J Surg Pathol. 2002;26(5):662–9.

    Article  CAS  PubMed  Google Scholar 

  274. Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N. Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series. Crit Rev Oncol Hematol. 2013;88(2):253–71.

    Article  PubMed  Google Scholar 

  275. Weiss LM, Grogan TM, Chan JKC. Interdigitating dendritic cell sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2008. p. 361–2.

    Google Scholar 

  276. Fonseca R, Yamakawa M, Nakamura S, van Heerde P, Miettinen M, Shek TW, et al. Follicular dendritic cell sarcoma and interdigitating reticulum cell sarcoma: a review. Am J Hematol. 1998;59(2):161–7.

    Article  CAS  PubMed  Google Scholar 

  277. Grogg KL, Macon WR, Kurtin PJ, Nascimento AG. A survey of clusterin and fascin expression in sarcomas and spindle cell neoplasms: strong clusterin immunostaining is highly specific for follicular dendritic cell tumor. Mod Pathol. 2005;18(2):260–6.

    Article  CAS  PubMed  Google Scholar 

  278. Grogg KL, Lae ME, Kurtin PJ, Macon WR. Clusterin expression distinguishes follicular dendritic cell tumors from other dendritic cell neoplasms: report of a novel follicular dendritic cell marker and clinicopathologic data on 12 additional follicular dendritic cell tumors and 6 additional interdigitating dendritic cell tumors. Am J Surg Pathol. 2004;28(8):988–98.

    Article  PubMed  Google Scholar 

  279. Shao H, Xi L, Raffeld M, Feldman AL, Feldman AL, Ketterling RP, 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 

  280. Feldman AL, Arber DA, Pittaluga S, Martinez A, Burke JS, Raffeld M, et al. Clonally related follicular lymphomas and histiocytic/dendritic cell sarcomas: evidence for transdifferentiation of the follicular lymphoma clone. Blood. 2008;111(12):5433–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  281. Fraser CR, Wang W, Gomez M, Zhang T, Mathew S, Furman RR, et al. Transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma to interdigitating dendritic cell sarcoma: evidence for transdifferentiation of the lymphoma clone. Am J Clin Pathol. 2009;132(6):928–39.

    Article  CAS  PubMed  Google Scholar 

  282. Xie Q, Chen L, Fu K, Harter J, Young KH, Sunkara J, et al. Podoplanin (d2-40): a new immunohistochemical marker for reactive follicular dendritic cells and follicular dendritic cell sarcomas. Int J Clin Exp Pathol. 2008;1(3):276–84.

    CAS  PubMed  PubMed Central  Google Scholar 

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  1. Department of Pathology, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Barcelona, 08916, Spain

    Maria Teresa Fernández Figueras M.D., Gustavo Tapia M.D., José Luis Mate M.D. & Aurelio Ariza M.D.

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  1. Maria Teresa Fernández Figueras M.D.
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    Jose A. Plaza

  2. Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

    Victor G. Prieto

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Figueras, M.T.F., Tapia, G., Mate, J.L., Ariza, A. (2016). Immunohistology of Leukemia Cutis and Histiocytic Tumors. In: Plaza, J., Prieto, V. (eds) Applied Immunohistochemistry in the Evaluation of Skin Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-30590-5_11

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