Blastic plasmacytoid dendritic cell neoplasm
- Authors: Valiev T.T.1,2, Seregin G.Z.1, Serebryakova I.N.3, Chernyshova O.A.3, Kupryshina N.A.3, Palladina A.D.3, Sholokhova E.N.3, Chebotaryov D.I.4, Tracevskaya Z.V.4, Mikhailova S.N.1, Doroshenko M.V.1, Tupitsyn N.N.5, Kurdyukov B.V.5, Popa A.V.6,1
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Affiliations:
- 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
- Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation
- National Research Center for Hematology of Ministry of Health of Russian Federation
- Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian Federation
- N.I. Pirogov Russian National Research Medical University
- Issue: Vol 18, No 4 (2019)
- Pages: 79-89
- Section: CLINICAL OBSERVATIONS
- Submitted: 30.12.2019
- Accepted: 30.12.2019
- Published: 30.12.2019
- URL: https://hemoncim.com/jour/article/view/288
- DOI: https://doi.org/10.24287/1726-1708-2019-18-4-79-89
- ID: 288
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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 FederationG. 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 FederationMoscow Russian Federation
O. A. Chernyshova
Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow Russian Federation
N. A. Kupryshina
Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow Russian Federation
A. D. Palladina
Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow Russian Federation
E. N. Sholokhova
Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow Russian Federation
D. I. Chebotaryov
National Research Center for Hematology of Ministry of Health of Russian FederationMoscow Russian Federation
Zh. V. Tracevskaya
National Research Center for Hematology of Ministry of Health of Russian FederationMoscow 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 FederationMoscow Russian Federation
N. N. Tupitsyn
Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow Russian Federation
B. V. Kurdyukov
Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationMoscow 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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