Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematopoietic neoplasm, which in the past was also known variously as blastic NK cell lymphoma, agranular CD4+ natural killer cell leukemia, and CD4+/CD56+ hematodermic neoplasm. BPDCN is now believed to arise from plasmacytoid dendritic cells, but its exact etiology is still unknown. We report here on the cerebrospinal fluid (CSF) cytology of a BPDCN, a hypercellular specimen comprised of malignant, singly dispersed cells with scant to moderate amounts of pale blue, agranular cytoplasm, and uniform round to oval nuclei, fine chromatin, prominent nucleoli, occasional cytoplasmic microvacuoles, and pseudopodia. Neither mitoses nor karyorrhexis were identified. Flow cytometry of the CSF demonstrated that the malignant cells expressed bright CD45, HLA-DR and CD33, dim CD4, heterogeneous CD56, and partial CD123. The importance of clinical, histopathological, and phenotypic correlation is emphasized. Clinical and histopathological correlation and a literature review are also presented. The poor clinical outcome makes it important to accurately report this rare tumor in a CSF specimen.

Facchetti F, Jones DM, Petrella T: Blastic plasmacytoid dendritic cell neoplasm; in Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds): WHO Classification of Tumors of Haematopoietic and Lymphoid Tissue. Lyon, IARC, 2008, pp 145–147.
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;127:687–700.
Chaperot L, Bendriss N, Manches O, Gressin R, Maynadie M, Trimoreau F, Orfeuvre H, Corront B, Feuillard J, Sotto JJ, Bensa JC, Briere F, Plumas J, Jacob MC: Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood 2001;97:3210–3217.
Chaperot L, Perrot I, Jacob MC, Blanchard D, Salaun V, Deneys V, Lebecque S, Briere F, Bensa JC, Plumas J: Leukemic plasmacytoid dendritic cells share phenotypic and functional features with their normal counterparts. Eur J Immunol 2004;34:418–426.
Dalle S, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P, Dompmartin A, d’Incan M, Maubec E, Grange F, Dereure O, Prey S, Barete S, Wetterwald M, Fraitag S, Petrella T: Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol 2009;162:74–79.
Petrella T, Comeau MR, Maynadie M, Couillault G, De Muret A, Maliszewski CR, Dalac S, Durlach A, Galibert L: ‘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;26:852–862.
Petrella T, Facchetti F: Tumoral aspects of plasmacytoid dendritic cells: what do we know in 2009? Autoimmunity 2010;43:210–214.
Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L, Metze D, Fink-Puches R, Wiesner T, Cerroni L: Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol 2010;34:75–87.
Feuillard J, Jacob MC, Valensi F, Maynadie M, Gressin R, Chaperot L, Arnoulet C, Brignole-Baudouin F, Drenou B, Duchayne E, Falkenrodt A, Garand R, Homolle E, Husson B, Kuhlein E, Le Calvez G, Sainty D, Sotto MF, Trimoreau F, Bene MC: Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood 2002;99:1556–1563.
Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, Meijer CJ, Courville P, Joly P, Grange F, De Muret A, Machet L, Dompmartin A, Bosq J, Durlach A, Bernard P, Dalac S, Dechelotte P, D’Incan M, Wechsler J, Teitell MA: Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review. Am J Clin Pathol 2005;123:662–675.
Colonna M, Trinchieri F, Liu YJ: Plasmacytoid dendritic cells in immunity. Nat Immunol 2004;5:1219–1226.
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;128:445–453.
Garnache-Ottou F, Chaperot L, Biichle S, Ferrand C, Remy-Martin JP, Deconinck E, de Tailly PD, Bulabois B, Poulet J, Kuhlein E, Jacob MC, Salaun V, Arock M, Drenou B, Schillinger F, Seilles E, Tiberghien P, Bensa JC, Plumas J, Saas P: Expression of the myeloid-associated marker CD33 is not an exclusive factor for leukemic plasmacytoid dendritic cells. Blood 2005;105:1256–1264.
Reimer P, Rudiger T, Kraemer D, Kunzmann V, Weissinger F, Zettl A, Konrad Muller-Hermelink H, Wilhelm M: What is CD4+CD56+ malignancy and how should it be treated? Bone Marrow Transplant 2003;32:637–646.
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