Blastic plasmacytoid dendritic cell neoplasm

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Abstract

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.

About the authors

T. T. Valiev

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian Federation;
Federal State Budgetary Educational Institution of Supplementary Vocational Education Russian Medical Academy of Continuous Professional Training of Ministry of Health of Russian Federation

Author for correspondence.
Email: timurvaliev@mail.ru
ORCID iD: 0000-0002-1469-2365

Correspondence: Timur T. Valiev, MD, PhD, senior research scientist, Department of Hemoblastoses Chemotherapy, Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian.

Address: 115478, Moscow, Kashirskoe sh., 24 Russian Federation

G. Z. Seregin

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

ORCID iD: 0000-0003-3880-4402
Moscow Russian Federation

I. N. Serebryakova

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

O. A. Chernyshova

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

N. A. Kupryshina

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

A. D. Palladina

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

E. N. Sholokhova

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

D. I. Chebotaryov

National Research Center for Hematology of Ministry of Health of Russian Federation

Moscow Russian Federation

Zh. V. Tracevskaya

National Research Center for Hematology of Ministry of Health of Russian Federation

Moscow Russian Federation

S. N. Mikhailova

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

ORCID iD: 0000-0002-9502-072X
Moscow Russian Federation

M. V. Doroshenko

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

N. N. Tupitsyn

Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

B. V. Kurdyukov

Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

Moscow Russian Federation

A. V. Popa

N.I. Pirogov Russian National Research Medical University;
Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian Federation

ORCID iD: 0000-0001-5318-8033
Moscow Russian Federation

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Copyright (c) 2019 Valiev T.T., Seregin G.Z., Serebryakova I.N., Chernyshova O.A., Kupryshina N.A., Palladina A.D., Sholokhova E.N., Chebotaryov D.I., Tracevskaya Z.V., Mikhailova S.N., Doroshenko M.V., Tupitsyn N.N., Kurdyukov B.V., Popa A.V.

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This work is licensed under a Creative Commons Attribution 4.0 International License.