bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.18821/0234-5730-2016-61-2-110-112bloodjour-50Research ArticleОБЗОРЫ ЛИТЕРАТУРЫREVIEWS OF LITERATUREНовые критерии диагностики лейкемического системного мастоцитоза и миеломастоцитозной лейкемииNew diagnostic criteria of leukemic systemic mastocytosis and myelomastocytic leukemiahttps://orcid.org/0000-0002-1171-2746БудневскийА. В.BudnevskiyA. V.https://orcid.org/0000-0002-8545-6255ОвсянниковЕ. С.OvsyannikovE. S.

Овсянников Евгений Сергеевич, кандидат мед. наук, доцент кафедры факультетской терапии

394000, г. Воронеж

Ovsyannikov Evgeny V., MD, PhD, associate professor

Voronezh, 394000

ovses@yandex.ruhttps://orcid.org/0000-0002-6809-9743ЖусинаЮ. Г.ZhusinaYu. G.

394000, г. Воронеж

Voronezh, 394000

ГБОУ ВПО «Воронежский государственный медицинский университет им. Н.Н. Бурденко» Минздрава РоссииРоссияVoronezh State Medical University n.a. N.N. BurdenkoRussian Federation201601032019612110112Copyright © Будневский А.В., Овсянников Е.С., Жусина Ю.Г., 20192019Будневский А.В., Овсянников Е.С., Жусина Ю.Г.Budnevskiy A.V., Ovsyannikov E.S., Zhusina Y.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/50

Лейкоз из тучных клеток – лейкемический системный мастоцитоз как проявление системного мастоцитоза характеризуется пролиферацией и накоплением незрелых тучных клеток в костном мозге и других внутренних органах. Наибольшие трудности существуют в дифференциальной диагностике лейкемического системного мастоцитоза и миеломастоцитарного лейкоза. Несмотря на имеющиеся в обоих случаях опубликованные критерии диагностики, некоторые вопросы терминологии остаются открытыми. Данная проблема обсуждалась консенсус-группой по мастоцитозу в 2011 и 2013 гг. (EU/US-consensus group and the European Competence Network on Mastocytosis – ECNM). Диагноз миеломастоцитарного лейкоза как миелоидной опухоли с большим числом тучных клеток было предложено считать правомочным при отсутствии критериев, необходимых для диагностики мастоцитоза. Кроме того, было рекомендовано на основании наличия или отсутствия кожных проявлений разделить лейкемический системный мастоцитоз на острый и хронический. Первичную форму тучноклеточного лейкоза нужно дифференцировать с вторичной, которая, как правило, развивается на фоне установленного агрессивного системного мастоцитоза или саркомы тучных клеток. Подчеркнута неизбежность стадии предлейкоза для лейкемического системного мастоцитоза, который часто дебютирует в виде агрессивного системного мастоцитоза с быстрым прогрессированием и появлением от 5 до 19% тучных клеток в мазках костного мозга. Подобное состояние рекомендовано называть агрессивным системным мастоцитозом с трансформацией в лейкоз из тучных клеток. Расширение текущей классификации ВОЗ путем включения в нее различных вариантов тучноклеточного лейкоза позволит оптимизировать отбор пациентов для клинических испытаний.

Mast cell leukemia is characterized by proliferation and accumulation of immature mast cells in the bone marrow and other organs. The difficulties exist in the differential diagnosis of leukemic systemic mastocytosis and myelomastocytic leukemia. Although in both cases diagnostic criteria are reported, some questions about the terms remain to be open. This problem was discussed by the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) in 2011--2013. The diagnosis of myelomastocytic leukemia as myeloid tumor with a large number of mast cells was proposed as eligible in the absence of diagnostic criteria of mastocytosis. It was also recommended to divide leukemic systemic mastocytosis into acute and chronic forms based on the presence or absence of cutaneous manifestations. The primary form of the mast cell leukemia must be differentiated from the secondary, which usually develops in the set of aggressive systemic mastocytosis and mast cell sarcoma. The authors put emphasis on the inevitability of the prephase or leukemic systemic mastocytosis, which is often debut as aggressive systemic mastocytosis with rapid progression and the appearance of from 5 to 19% of mast cells in bone marrow smears. This condition is recommended to call the aggressive systemic mastocytosis in conversion to mast cells leukemia. The expansion of the current WHO classification by incorporating different variants of mast cell leukemia, will optimize the selection of patients for clinical trials.

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The authors declare that there are no conflicts of interest present.