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Blastic plasmacytoid dendritic cell neoplasma ��BPDCN�� is a rare invasive lymphoma, which has been recognized as an independent entity in the 2008 World Health Organization ��WHO�� classification of cutaneous lymphomas. Cutaneous involvement often represents the first manifestation of this disease, and it may appear as bruise-like nodules or plaques. It has a predilection for the invasion of bone marrow and lymph nodes with an aggressive course and poor prognosis. The tumor cells , which have been regarded to derive from the precursors of plasmacytoid dendritic cells with special immunophenotype, are positive for CD4, CD56, plasmacytoid dendritic cell-associated antigens ��CD123, TCL1 and BDCA-2��, and partly positive to TdT and CD68. Currently, the aetiology of BPDCN remains unknown and there are no standard therapeutic schemes.

[1] Petrella T�� Bagot M�� Willemze R��et al. Blastic NK-cell lymphomas (agranular CD4+ CD56+ hematodermic neoplasms): a review. Am J Clin Pathol, 2005, 123(5): 662-675. [2] Jaffe ES��Chan JK��Su IJ��et al. Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas.Definitions, differential diagnosis, and epidemiology. Am J Surg Pathol, 1996, 20(1):103-111. [3] Jaffe ES�� Harris NL��Stein H��et a1. World health organization classification of tumours��Tumours of haematopoietic and lymphoid tissues��Lyon��IARC Press��2001��214-215�� [4] Willemze R��Jaffe ES��Burg G��et a1��WHO-EORTC classification for cutaneous lymphomas. Blood, 2005,105:3768-3785�� [5] Petrella T�� Meijer CJ��Dalacs��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):307-313. [6] Swerdlow SH��Campo E��Harris NL��et a1. WHO classification of tumours of heamatopioetics and lymphoid tissues. Lyon��IARC Press, 2008��145-147�� [7] Petrella T��Dalac S��Maynadie M��et a1. CD4+ CD56+ cutaneous neoplasms: a distinct hematological entity? Groupe Fran?ais d'Etude des Lymphomes Cutanés (GFELC). Am J Surg Pathol, 1999, 23(2):137-146. [8] Wiesner T, Obenauf AC, Cota C, et a1. Alterations of the Cell-Cycle Inhibitors p27(KIP1) and p16(INK4a) Are Frequent in Blastic Plasmacytoid Dendritic Cell Neoplasms. J Invest Dermatol, 2009 Nov 19. [Epub ahead of print]. [9] Cota C, Vale E, Viana I, et a1. Cutaneous Manifestations of Blastic Plasmacytoid Dendritic Cell Neoplasm-Morphologic and Phenotypic Variability in a Series of 33 Patients. Am J Surg Pathol, 2010, 34(1):75-87. [10] 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. [11] Ng AP, Lade S, Rutherford T, et a1. Primary cutaneous CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma): a report of five cases. Haematologica, 2006,91(1):143-144. [12] Shiman M, Marchione R, Ricotti C, et a1.CD4+/CD56+ Hematodermic neoplasm (plasmacytoid dendritic cell tumor). Dermatol Online J, 2008 , 14(11):5. [13] Bekkenk MW��Jansen PM,Meijer CJ, et a1��CD56+ hematological neoplasms presenting in the skin��a retrospective analysis of 23 new cafes and 130 cases from the literature��Ann Oncol, 2004, 15(7):1097-1108. [14] Dalle S, Beylot-Barry M, Bagot M, et a1. Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol, 2009 Jun 22. [Epub ahead of print] [15] Pi?a-Oviedo S, Herrera-Medina H, Coronado H, et a1. D4+/CD56+ Hematodermic Neoplasm: Presentation of 2 Cases and Review of the Concept of an Uncommon Tumor Originated in Plasmacytoid Dendritic Cells Expressing CD123 (IL-3 Receptor [alpha]. Appl Immunohistochem Mol Morphol, 2007,5(4):481-486. [16] Kaune KM, Baumgart M, Bertsch HP, et a1.Solitary cutaneous nodule of blastic plasmacytoid dendritic cell neoplasm progressing to overt leukemia cutis after chemotherapy: immunohistology and FISH analysis confirmed the diagnosis. Am J Dermatopathol, 2009 ,31(7):695-701. [17] Facchetti F, Pileri SA, Agostinelli C, et a1. Cytoplasmic nucleophosmin is not detected in blastic plasmacytoid dendritic cell neoplasm. Haematologica, 2009,94(2):285-288. [18] L?ffler H, Kosely F, Ho AD, et a1. Blastic plasmacytoid dendritic cell neoplasm - a rare differential diagnosis of neoplastic skin infiltrations associated with systemic symptoms. Dtsch Med Wochenschr, 2009 ,134(39):1927-1930. [19] López V, Martí N, Ferrández A, et a1. An atypical presentation of a blastic plasmacytoid dendritic cell tumors. J Cutan Pathol,2009 Jul 13. [Epub ahead of print] [20] Fontaine J, Thomas L, Balme B, et a1. Haematodermic CD4+CD56+ neoplasm: complete remission after methotrexate-asparaginase treatment. Clin Exp Dermatol,2009 ,34(5):e43-45.