Open AccessBlastic plasmacytoid dendritic cell neoplasm
Author(s) -
Т. Т. Валиев,
Г. З. Серегин,
И. Н. Серебрякова,
О. А. Чернышева,
Н. А. Купрышина,
А. Д. Палладина,
Е. Н. Шолохова,
DI Chebotarev,
Ж. В. Трацевская,
С. Н. Михайлова,
М. Б. Дорошенко,
Н. Н. Тупицын,
Б. В. Курдюков,
Alexander Popa
Publication year - 2019
Publication title -
voprosy gematologii/onkologii i immunopatologii v pediatrii
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.108
H-Index - 3
eISSN - 2414-9314
pISSN - 1726-1708
DOI - 10.24287/1726-1708-2019-18-4-79-89
Subject(s) - medicine , malignancy , myeloid , bone marrow , cancer , differential diagnosis , leukemia , pathology , neoplasm , oncology , cancer research , immunology
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare hematologic malignancy. Our view of the cellular origins of this kind of tumor has been changing dramatically with the emergence of new data on the molecular biological and immunological characteristics of the tumor. This article discusses the clinical features of BPDCN, as well as the cytological, morphological-immunological and molecular genetic criteria for BPDCN diagnosis. Taking into account the rare incidence of BPDCN, as well as its rather complex diagnostic procedure, which requires an extended diagnostic antibody panel, standard methods of therapy have not been developed. Chemotherapy protocols for acute lymphoblastic leukemia and acute myeloid leukemia are used, with/without subsequent autologous/allogeneic bone marrow transplantation, but the results remain unsatisfactory. For the first time in Russian cancer research, this article provides a description of BPDCN in a 14-year-old child. A detailed clinical analysis of this rare tumor is provided, as well as dermatoscopy results and a description of the histological, immunological and molecular features of BPDCN, from the point of view of differential diagnosis. Parents patients agreed to use personal data in research and publications.