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Blastic plasmacytoid dendritic cell neoplasm: case report and literature review


Authors: M. Čerňan 1;  T. Szotkowski 1;  Z. Rusiňáková 1;  L. Raida 1;  M. Tichý 2;  M. Dušková 2
Authors‘ workplace: Hemato-onkologická klinika LF UP a FN Olomouc 1;  Ústav klinické a molekulární patologie LF UP a FN Olomouc 2
Published in: Transfuze Hematol. dnes,23, 2017, No. 3, p. 134-144.
Category: Comprehensive Reports, Original Papers, Case Reports

Overview

Blastic plasmacytoid dendritic cell neoplasm represents a rare hematologic malignancy of aggressive biology and unfavourable prognosis. This disease is characterised by primary skin lesions often followed by bone marrow and extramedullary involvement. Diagnosis is based on immunohistochemistry and flow cytometric demonstration of CD4 and CD56 co-expression with variable positivity of other markers specific to dendritic cells. The median age at diagnosis ranges from 60 to 70 years, with males affected 3 times more frequently. Knowledge of its treatment is limited to retrospective analyses of small patient cohorts. Despite generally favourable treatment response to chemotherapy, the disease tends to relapse in a few months with rapid systemic dissemination. The reported data suggest that induction therapy for acute lymphoblastic leukaemia followed by allogeneic hematopoietic cell transplantation is more effective than other induction or consolidation chemotherapy strategies.

The article reports the case of a 22-year old male with newly diagnosed blastic plasmacytoid dendritic cell neoplasm with skin-limited symptoms. The patient underwent induction and one cycle of consolidation chemotherapy accordingto the GMALL protocol for acute lymphoblastic leukaemia followed by allogeneic hematopoietic cell transplantation in the first complete remission. Chemotherapy and transplantation were not associated with serious complications and the patient remains in remission with a good quality of life, 22 months after allogeneic transplantation. This case details the successful treatment strategy in a disease with unfavourable prognosis. The article also presents a review of the literature regarding diagnosis, clinical presentation and treatment strategies in patients with blastic plasmacytoid dendritic cell neoplasm. The aim of the paper is also to provide information about this rare nosological unit and promote prompt diagnosis and treatment initiation necessary for disease control.

KEY WORDS:
blastic plasmacytoid dendritic cell neoplasm – BPDCN – induction chemotherapy – GMALL – bone marrow transplantation – immunophenotyping


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