Vol 51, No 4 (2020)
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Published online: 2020-12-01

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The beneficial role of allogenic hematopoietic cell transplantation in blastic plasmacytoid dendritic cell neoplasm

Emilia Ulenberg1, Krzysztof Lewandowski2, Maria Bieniaszewska13, Jan Maciej Zaucha13
DOI: 10.2478/ahp-2020-0041
Acta Haematol Pol 2020;51(4):236-244.

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare aggressive hematopoietic malignancy. The median survival is approximately 12 months, and for patients >65 years the survival rate is 7 months, when only chemotherapy is administered. Clinically, it is characterized by skin involvement and most often bone marrow lesions accompanied by lymphadenopathy and in some cases hepato- and/or splenomegaly. The diagnosis is based on histopathological examination of the skin or bone marrow lesions and tumor cell immunophenotyping. The etiopathogenesis of the disease is not fully understood. Therapeutic decisions are based only on the results of a few retrospective analyses and case reports. This article presents the important role of allogeneic hematopoietic cell transplantation in the treatment of BPDCN.

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