Abstract
A 57-year-old man became aware of left supraclavicular lymph node swelling, which was subsequently diagnosed as Langerhans cell sarcoma, based on a positive immunophenotype for CD1a, S-100 protein, and langerin, and histologically bizarre pleomorphism. The tumor became leukemic 3 months later. Despite intensive chemotherapy, he died of disease progression 7 months after the initial diagnosis. Tumor cells in the leukemic phase expressed CD5, CD7, CD13, CD33, CD34, CD68, and CD123. These findings suggested leukemic transformation from Langerhans cell sarcoma. Leukemic transformation may be a clinical manifestation of advanced Langerhans cell sarcoma, and should be differentiated from acute myelogenous leukemia.
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References
Katz SI, Tamaki K, Sachs DH. Epidermal Langerhans cells are derived from cells originating in bone marrow. Nature. 1979;282:324–6.
Caux C, Dezutter-Dambuyant C, Schmitt D, Banchereau J. GM-CSF and TNF-alpha cooperate in the generation of dendritic Langerhans cells. Nature. 1992;360:258–61.
Weiss LM, Grogan TM, Pileri SA, et al. Langerhans cell sarcoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization classification of tumours. Pathology and genetics: tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2001. p. 283.
Bohn OL, Ruiz-Arguelles G, Navarro L et al. Cutaneous Langerhans cell sarcoma: a case report and review of the literature. Int J Hematol. 2007;85:116–20.
Pileri SA, Grogan TM, Harris NL 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;41:1–29.
Lee JS, Ko GH, Kim HC et al. Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report. J Korean Med Sci. 2006;21:577–80.
Caux C, Vanbervliet B, Massacrier C et al. Interleukin-3 cooperates with tumor necrosis factor alpha for the development of human dendritic/Langerhans cells from cord blood CD34+ hematopoietic progenitor cells. Blood. 1996;87:2376–85.
Ito T, Inaba M, Inaba K et al. A CD1a+/CD11c+ subset of human blood dendritic cells is a direct precursor of Langerhans cells. J Immunol. 1999;163:1409–19.
Caux C, Vanbervliet B, Massacrier C et al. CD34+ hematopoietic progenitors from human cord blood differentiate along two independent dendritic cell pathways in response to GM-CSF+TNF alpha. J Exp Med. 1996;184:695–6.
Imamura M, Sakamoto S, Hanazono H. Malignant histiocytosis: a case of generalized histiocytosis with infiltration of Langerhans’ granule-containing histiocytes. Cancer. 1972;28:467–75.
Henderson DW, Sage RE. Malignant histiocytosis with eosinophilia. Cancer. 1973;32:1421–8.
Kawase T, Hamazaki M, Ogura M et al. CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases. Int J Hematol. 2005;81:323–9.
Greenberger JS, Crocker AC, Vawter G, Jaffe N, Cassady JR. Results of treatment of 127 patients with systemic histiocytosis. Medicine (Baltimore). 1981;60:311–38.
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We are grateful to Hidenori Tsukiji for technical assistance.
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Sumida, K., Yoshidomi, Y., Koga, H. et al. Leukemic transformation of Langerhans cell sarcoma. Int J Hematol 87, 527–531 (2008). https://doi.org/10.1007/s12185-008-0063-x
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DOI: https://doi.org/10.1007/s12185-008-0063-x