Pediatric Hematology/Oncology and Immunopathology

Вопросы гематологии/онкологии и иммунопатологии в педиатрии

1726-17082414-9314

Fund Doctors, Innovations, Science for Children

288

10.24287/1726-1708-2019-18-4-79-89

CLINICAL OBSERVATIONS

КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ

Blastic plasmacytoid dendritic cell neoplasm

Опухоль из бластных плазмоцитоидных дендритических клеток

https://orcid.org/0000-0002-1469-2365

Valiev

T. T.

Валиев

Т. Т.

Russian Federation

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

Контактная информация: Валиев Тимур Теймуразович, д-р мед. наук, старший научный сотрудник отделения химиотерапии гемобластозов, НИИ детской онкологии и гематологии Национального медицинского исследовательского центра онкологии им. Н.Н. Блохина Минздрава России.

Адрес: 115478 Москва, Каширское шоссе, 24timurvaliev@mail.ru

https://orcid.org/0000-0003-3880-4402

Seregin

G. Z.

Серегин

Г. З.

Russian Federation

Moscow

Москва

Serebryakova

I. N.

Серебрякова

И. Н.

Russian Federation

Moscow

Москва

Chernyshova

O. A.

Чернышева

О. А.

Russian Federation

Moscow

Москва

Kupryshina

N. A.

Купрышина

Н. А.

Russian Federation

Moscow

Москва

Palladina

A. D.

Палладина

А. Д.

Russian Federation

Moscow

Москва

Sholokhova

E. N.

Шолохова

Е. Н.

Russian Federation

Moscow

Москва

Chebotaryov

D. I.

Чеботарев

Д. И.

Russian Federation

Moscow

Москва

Tracevskaya

Zh. V.

Трацевская

Ж. В.

Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-9502-072X

Mikhailova

S. N.

Михайлова

С. Н.

Russian Federation

Moscow

Москва

Doroshenko

M. V.

Дорошенко

М. Б.

Russian Federation

Moscow

Москва

Tupitsyn

N. N.

Тупицын

Н. Н.

Russian Federation

Moscow

Москва

Kurdyukov

B. V.

Курдюков

Б. В.

Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-5318-8033

Popa

A. V.

Попа

А. В.

Russian Federation

Moscow

Москва

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian FederationНИИ детской онкологии и гематологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России

Federal State Budgetary Educational Institution of Supplementary Vocational Education Russian Medical Academy of Continuous Professional Training of Ministry of Health of Russian FederationФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationНИИ клинической онкологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России

Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России

National Research Center for Hematology of Ministry of Health of Russian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России

Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationНИИ клинической онкологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России

Clinical Oncology Research Institute of N.N.Blokhin National Research Cancer Center of Ministry of Health of Russian FederationНИИ детской онкологии и гематологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России

N.I. Pirogov Russian National Research Medical UniversityНИИ детской онкологии и гематологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России

Pediatric Oncology and Hematology Research Institute of N.N. Blokhin National Research Cancer Center of Ministry of Health of Russian FederationФГБОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России

31122019

184

79893012201930122019

2019

«D. Rogachev NMRCPHOI»

ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России

https://creativecommons.org/licenses/by/4.0

https://hemoncim.com/jour/article/view/288

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.

Опухоль из бластных плазмоцитоидных дендритических клеток (ОБПДК) – крайне редкий вариант гемобластоза. Представления о его клеточном происхождении драматически менялись по мере появления новых данных о молекулярно-биологических и иммунологических характеристиках опухоли. В работе рассмотрены клинические особенности ОБПДК, цитологические, морфо-иммунологические и молекулярно-генетические критерии диагностики ОБПДК. С учетом редкой встречаемости ОБПДК, весьма сложной диагностики, требующей постановки расширенной диагностической панели антител, стандартные терапевтические подходы не разработаны. Используются протоколы химиотерапии острого лимфобластного и острого миелоидного лейкоза с/без последующей аутологичной/аллогенной трансплантации костного мозга, но результаты остаются неудовлетворительными. Впервые в отечественной литературе приводится описание ОБПДК у ребенка 14 лет. Представлена детальная клиническая характеристика этой редкой опухоли, результаты дерматоскопии, гистологические, иммунологические и ее молекулярные особенности с дифференциально-диагностических позиций. Родители пациентов дали согласие на использование информации о ребенке в научных исследованиях и публикациях.

blastic plasmacytoid dendritic cell neoplasmdiagnosistreatment

опухоль из бластных плазмоцитоидных дендритических клетокдиагностикалечение

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