Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children65510.24287/1726-1708-2022-21-3-90-99CLINICAL OBSERVATIONSКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯMultifocal lymphangioendotheliomatosis with thrombocytopenia/cutaneovisceral angiomatosis with thrombocytopeniaМультифокальный лимфангиоэндотелиоматоз с тромбоцитопенией/кожновисцеральный ангиоматоз с тромбоцитопениейhttps://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., 117997, Moscow, Russia

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

ScherbakovA. P.ЩербаковА. П.
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 России1510202221390991510202215102022Copyright ©; 2022, «D. Rogachev NMRCPHOI»Copyright ©; 2022, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2022«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/655

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)/cutaneovisceral angiomatosis with thrombocytopenia, first reported as a new nosological entity in 2004, is an extremely rare vascular pathology of the neonatal period without hereditary nature. This disease has an extremely poor prognosis. According to the previously published data, this vascular pathology is characterized by involvement of the skin and internal organs in the pathological process. Lesions of the gastrointestinal tract are almost obligatory in this disease. MLT is associated with high mortality, which, according to some authors, can reach more than 50%. Fulminant gastrointestinal bleedings due to specific lesions of the intestinal mucosa, severe thrombocytopenia and consumption coagulopathy, are the leading cause of death in these patients. Currently, there are no standard treatment options for MLT. The data about the efficacy of glucocorticosteroids, vincristine, thalidomide, propranolol, and rapamycin in the treatment of MLT, are controversial. Here we present a case report of a patient with MLT with a classic clinical spectrum of this disease. We first report on the efficacy of a polychemotherapeutic regimen with liposomal doxorubicin in the treatment of this disease: the patient achieved a complete hematological response after 2 months from the treatment start. A durable clinical response was documented after 6 months of treatment. This disease represents challenges, both from a diagnostic and therapeutic point of view. Further studies are required to find the optimal treatment regimens for this condition. The patient's parents have consented to the use of de-identified clinical information and photos of the patient in scientific research and publications.

Мультифокальный лимфангиоэндотелиоматоз с тромбоцитопенией (МЛТ)/кожно-висцеральный ангиоматоз с тромбоцитопенией, описанный в 2004 г. в качестве новой нозологической единицы, представляет собой крайне редкую сосудистую патологию неонатального периода без доказанного наследственного характера с очень плохим прогнозом. По данным ранее описанных случаев, для этой сосудистой патологии, которая относится к так называемым гемангио-лимфангиогенным сосудистым аномалиям, характерно вовлечение в патологический процесс кожи и внутренних органов с практически обязательным поражением желудочно-кишечного тракта. Летальность, которая, по данным некоторых публикаций, может достигать более 50%, обусловлена преимущественно фульминантными желудочно-кишечными кровотечениями в результате специфического поражения слизистой на фоне выраженной тромбоцитопении и коагулопатии потребления. В настоящее время не существует стандартов терапии МЛТ. Эффективность применяемых лекарственных препаратов, таких как глюкокортикостероиды, винкристин, талидомид, пропранолол, рапамицин, неоднозначна. В данной статье приведен пример МЛТ с классическим спектром клинических проявлений этой редкой нозологии. Впервые при лечении этого заболевания была применена полихимиотерапия с использованием липосомальной формы доксорубицина, которая позволила через 2 мес от начала терапии достичь полного гематологического ответа и через 6 мес – стойкого клинического ответа. Безусловно, этот сложный как с диагностической, так и с терапевтической точки зрения диагноз требует дальнейшего изучения в целях поиска оптимальных режимов этиологически и патогенетически обоснованной терапии. Родители пациента дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.

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