Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive tumor derived from the precursors of palsmacytoid dendritic cells with a high frequency of cutaneous and bone marrow involvement and leukaemic dissemination. The prognosis is poor, and an optimal treatment approach has not been defined. We describe a case of BPDCN that has been successfully treated with acute leukaemia-type induction (hyperCVAD) followed by allogeneic stem cell transplantation (ASCT).
Purpose: To evaluate the efficacy of hyperCVAD and ASCT in treatment of BPDCN.
Method: A case report and literature review. Result: An African American male, aged 49 years, presented with firm violaceous nodules on the left calf, back, and shoulders, accompanied by 40 lb weight loss. Patient did not experience night sweats, fevers, and chill. Complete blood cell count and serum lactate dehydrogenase were normal. A skin biopsy revealed dense dermal infiltrates consisting of intermediate-sized cells with high N:C ratio. The tumor cells were strongly positive for CD4, CD56; partially positive for TdT; weakly positive for CD45 and CD43; and negative for CD3, CD20, CD30, MPO, CD34, CD117. Bone marrow biopsy and aspirate smear showed hypercellular marrow with predominant blastic cells with high nuclear-cytoplasmic ratio, finely chromatin, and prominent nucleoli. Flow cytometric analysis demonstrated 85% blasts that were positive for HLA-DR, CD4, CD56, CD38, and TdT (partial); but negative for CD34, CD117, CD33, CD13, CD14, CD15, CD2, CD3, CD5, CD11c, CD7, CD19, CD10, CD20, CD22, CD24, Kappa, Lambda, CD25, CD52, and MPO. Cyotogenetic analysis reported an abnormal complex chromosome abnormality: 46, XY, add (7)(q22), add (8)(p11.2), add (9)(q13), psu dic(13:6)(p12;q16), del(13)(q12q22), -17, +21, +mar[9]. The peripheral blood smear revealed rare blasts. The patient responded well to hyperCVAD chemo followed by ASCT. He has remained disease free for > 5 years.
Conclusions: Aggressive chemotherapy followed by ASCT is a favorable treatment plan for BPDCN.
