Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children73010.24287/1726-1708-2023-22-2-142-151CLINICAL OBSERVATIONSКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯKaposiform lymphangiomatosis with Kasabach–Merritt phenomenonКапошиформный лимфангиоматоз с феноменом Казабаха–Мерриттhttps://orcid.org/0000-0001-7265-0414KhachatryanL. A.ХачатрянЛ. А.
Russian Federation

Lili A. Khachatryan, Cand. Med. Sci., Head of Unit

Hematology / Oncology Isolation Unit

117997

1 Samory Mashela St.

Moscow

Лили Альбертовна Хачатрян, канд. мед. наук, заведующая отделением

боксированное отделение гематологии / онкологии

117997

ул. Саморы Машела, 1

Москва

lili.2510@yandex.ruhttps://orcid.org/0000-0002-2322-5734NovichkovaG. A.НовичковаГ. А.
Russian Federation

Moscow

Москва

VasilievaM. S.ВасильеваМ. С.
Russian Federation

Moscow

Москва

KletskayaI. S.КлецкаяИ. С.
Russian Federation

Moscow

Москва

ScherbakovA. P.ЩербаковА. П.
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 России080720232221421512803202324052023Copyright ©; 2025, «D. Rogachev NMRCPHOI»Copyright ©; 2025, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2025«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/730

   Kaposiform lymphangiomatosis (KLA) is an aggressive lymphatic anomaly associated with bone involvement, serositis occurring at various sites, the development of Kasabach–Merritt phenomenon, and frequent infectious complications. The International Society for the Study of Vascular Anomalies classifies KLA as a subtype of generalized lymphatic anomaly. The mTOR-inhibitor rapamycin in combination with symptomatic treatment is the most common specific treatment. However, there are no standard approaches to the management of KLA. Even with modern diagnostic tools and combination therapy, the 5-year survival rate is 51 %, and the average life expectancy is 2.75 years. This article presents a classic case of KLA associated with Kasabach–Merritt phenomenon that was successfully managed with rapamycin and a liposomal form of doxorubicin as specific therapy. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.

   Капошиформный лимфангиоматоз (kaposiform lymphangiomatosis, KLA) – это агрессивная лимфатическая аномалия, сочетающаяся с поражением костей, серозитами различной локализации, развитием феномена Казабаха–Мерритт, а также высоким уровнем инфекционных осложнений. В настоящее время Международным обществом по изучению сосудистых аномалий (International Society for the Study of Vascular Anomalies) KLA рассматривается как подтип генерализованной лимфатической аномалии. В качестве специфической терапии чаще всего применяется mTOR-ингибитор рапамицин в комбинации с симптоматическим лечением. Однако единых стандартов ведения пациентов нет. Даже при современной диагностике и комплексной терапии 5-летняя выживаемость составляет 51 %, а средняя продолжительность жизни – 2,75 года. В данной статье представлен классический случай KLA, протекавший в сочетании с феноменом Казабаха–Мерритт, с успешным применением в качестве специфической терапии рапамицина и липосомальной формы доксорубицина. Родители пациента дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.

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