Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children54610.24287/1726-1708-2021-20-3-74-91ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИKaposiform hemangioendothelioma/Kasabach–Merritt syndrome. Сlinical and laboratory characteristics. Analysis of clinical casesКапошиформная гемангиоэндотелиома/синдром Казабаха–Мерритт: клиниколабораторная характеристика. Анализ клинических случаевhttps://orcid.org/0000-0001-7265-0414KhachatryanL. A.ХачатрянЛ. А.
Russian Federation

Lili A. Khachatryan, cand. med. sci., Head of Box Department of Hematology/Oncology

1 Samory Mashela St., Moscow 117997 

Хачатрян Лили Альбертовна, канд. мед. наук, заведующая боксированным отделением гематологии/онкологии

117997, Москва, ул. Саморы Машела, 1 

lili.2510@yandex.ruKletskayaI. S.КлецкаяИ. С.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-1918-0841RemizovA. N.РемизовА. Н.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2322-5734NovichkovaG. A.НовичковаГ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-0016-6698MaschanA. A.МасчанА. А.
Russian Federation

Moscow

Москва

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России0810202120374910810202108102021Copyright ©; 2021, «D. Rogachev NMRCPHOI»Copyright ©; 2021, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2021«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/546

Kaposiform hemangioendothelioma (KHE) is a rare, usually congenital vascular tumor. It resembles Kaposi sarcoma histologically, but etiologically it is not associated with herpes simplex virus type 8. KHE refers to tumors of intermediate malignancy degree. The most severe complication is the addition of thrombocytopenia and consumption coagulopathy, i.e. development of the Kasabach–Merritt syndrome/phenomenon (KMS), which determines the high mortality rate (up to 30%) in this histological variant. The frequency of occurrence of KMS is unknown. Over Patients with KHE/KMS have clear clinical and laboratory characteristics, which in most cases allow make to diagnose without histological confirmation. Over 7-year follow-up period 32 patients with KHE were registered in our center; in 90.6% of cases it was complicated by the development of KMS. The study was approved by the Independent Ethics Committee and Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In the most of patients the tumor was detected from birth (84%), in half of the cases (52%) hematological complications were diagnosed simultaneously with the detection of the tumor. Сommon local complications include joint contractures, destruction of bone tissue, and invasion of neighboring organs. The half of the patients had changes in the heart function: from minor cardiac pathology to congenital defects. In addition, there were clinical and instrumental changes associated with volume overload: an increase in liver size, myocardial hypertrophy. Despite the presence of clear clinical and laboratory characteristics of KMS, some cases require differential diagnosis with other vascular anomalies accompanied by thrombocytopenia and consumption coagulopathy – with congenital hemangiomas (rapidly involuting congenital hemangioma), multifocal lymphangioendotheliomatosis with thrombocytopenia, kaposiform lymphangiomatosis, venous malformations. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.

Капошиформная гемангиоэндотелиома (КГЭ) – это редкая, как правило, врожденная сосудистая опухоль. Гистологически напоминает саркому Капоши, но этиологически она не ассоциирована с вирусом герпеса 8-го типа. КГЭ относится к опухолям промежуточной степени злокачественности. Самым грозным и тяжелым осложнением является присоединение тромбоцитопении и коагулопатии потребления, т. е. развитие синдрома/феномена Казабаха–Мерритт (СКМ), который и определяет высокую летальность (до 30%) при данном гистологическом варианте. Частота встречаемости СКМ неизвестна. Пациенты с КГЭ/СКМ имеют четкие клинические и лабораторные характеристики, которые в большинстве случаев позволяют поставить диагноз, не подтверждая его гистологически. За 7-милетний период наблюдения в нашем Центре были зарегистрированы 32 пациента с КГЭ, у 90,6% она осложнилась развитием СКМ. Исследование поддержано независимым этическим комитетом и утверждено решением ученого совета ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России. У большинства пациентов (72%) опухоль выявлялась с рождения, в половине случаев (59%) гематологические осложнения диагностировались одновременно с выявлением опухоли. К частым локальным осложнениям относятся контрактуры суставов, деструкция костной ткани, прорастание в соседние органы. У половины пациентов отмечались также изменения со стороны сердца: от малой патологии до врожденных пороков. Кроме того, выявлялся ряд клинико-инструментальных изменений, связанных с перегрузкой объемом: увеличение размеров печени, гипертрофия миокарда. Несмотря на наличие четких клинико-лабораторных характеристик СКМ, некоторые случаи требуют проведения дифференциального диагноза с другими сосудистыми аномалиями, сопровождающимися тромбоцитопенией и коагулопатией потребления, а именно с врожденными гемангиомами (rapidly involuting congenital hemangioma), мультифокальным лимфангиоэндотелиоматозом с тромбоцитопенией, капошиформным лимфангиоматозом, венозными мальформациями. Родители пациентов дали согласие на использование информации, в том числе фотографий детей, в научных исследованиях и публикациях.

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