open access
The beneficial role of allogenic hematopoietic cell transplantation in blastic plasmacytoid dendritic cell neoplasm
Affiliations- Department of Haematology and Transplantology, University Clinical Centre in Gdańsk, Gdańsk, Poland
- Department of Laboratory Medicine, Medical University of Gdańsk, Gdańsk, Poland
- Department of Haematology and Transplantology, Medical University of Gdańsk, Gdańsk, Poland
open access
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.
Keywords
blastic plasmacytoid dendritic cell neoplasm; allogeneic hematopoietic stem cell transplantation; treatment; prognosis
About this articleTitle
The beneficial role of allogenic hematopoietic cell transplantation in blastic plasmacytoid dendritic cell neoplasm
Journal
Issue
Pages
236-244
Published online
2020-12-01
Page views
176
Article views/downloads
312
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