[1484] Human Blastic Plasmacytoid Dendritic Cell Neoplasms. Characterization of a Series of 45 Cases with Clinical Correlation

Alejandro Parra Cubillos, Azahara Martinez Lopez, Waleska Salcedo Mercado, Raquel Pajares, Raquel Fuentes-Mateos, Maria E Castillo, Renato Franco, Miguel A Piris, Santiago Montes-Moreno, Carlos Barrionuevo-Cornejo, Sandro Casavilca-Zambrano, Shirley Quintana-Truyenque, Cesar Samanez-Figari. HUMV, Santander, Spain; CNIO, Madrid, Spain; FJD, Madrid, Spain; INEN, Lima, Peru; Instituto dei Tumori, Napoli, Italy; Médica INEN

Background: Human Blastic Plasmacytoid Dendritic Cell Neoplasm (HBPDCN) is an uncommon agressive leukemia except for pediatric patients treated with ALL-type regimens (AG Jegalian et al. Hematologica 2010). The spectrum of cutaneous manifestations and histopathological pattens is variable (C Cota et al. Am J Surg Pathol 2010). Significant phenotypic diversity exists with panels including CD4, CD56, TCL1 and CD123.
Design: The aim of this study was to characterize the clinical, pathological, immunohistochemical features of a series of 45 patients diagnosed of BPDCN.
Results: Most cases presented with systemic disease with LN and/or BM infiltration (21 cases 77,78%). In cases with disease limited to the skin, 5 (18,52%) cases had multifocal skin involvement (>pT2cN0M0) and 1 single case (3,7%) had localized skin disease (7 mitosis per HPF) were related with worse OS (p =0.07). Cases with mature morphology and low mitotic count had a overall survival of 70%.
Conclusions: HBPDCN usually present with systemic involvement. Bone marrow infiltration, atypical cytology with blastic cell morphology and high mitotic count seem to be related with worse outcome. Age is a major determinant of better overall survival. Significant phenotypic diversity exists and new specific markers are required for diagnosis.
Category: Hematopathology

Wednesday, March 6, 2013 9:30 AM

Poster Session V # 234, Wednesday Morning

 

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