open access

Vol 51, No 4 (2020)
Untitled
Published online: 2020-12-01
Submitted: 2019-12-03
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The beneficial role of allogenic hematopoietic cell transplantation in blastic plasmacytoid dendritic cell neoplasm

Emilia Ulenberg, Krzysztof Lewandowski, Maria Bieniaszewska, Jan Maciej Zaucha
DOI: 10.2478/ahp-2020-0041
·
Acta Haematol Pol 2020;51(4):236-244.

open access

Vol 51, No 4 (2020)
Untitled
Published online: 2020-12-01
Submitted: 2019-12-03

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.

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.

Get Citation

Keywords

blastic plasmacytoid dendritic cell neoplasm; allogeneic hematopoietic stem cell transplantation; treatment; prognosis

About this article
Title

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

Journal

Acta Haematologica Polonica

Issue

Vol 51, No 4 (2020)

Pages

236-244

Published online

2020-12-01

DOI

10.2478/ahp-2020-0041

Bibliographic record

Acta Haematol Pol 2020;51(4):236-244.

Keywords

blastic plasmacytoid dendritic cell neoplasm
allogeneic hematopoietic stem cell transplantation
treatment
prognosis

Authors

Emilia Ulenberg
Krzysztof Lewandowski
Maria Bieniaszewska
Jan Maciej Zaucha

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