Применение пропранолола в лечении синдрома Казабаха-Мерритт у детей

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Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2016; 15: 28-33

Применение пропранолола в лечении синдрома Казабаха-Мерритт у детей

Федорова Дарья Викторовна, Хачатрян Лили Альбертовна

https://doi.org/10.24287/1726-1708-2016-15-4-28-33

Аннотация

Синдром Казабаха-Мерритт (СКМ) - это состояние, характеризующееся наличием сосудистой опухоли, коагулопатии и тромбоцитопении потребления. Нарушения в системе гемостаза приводят к развитию синдрома диссеминированного внутрисосудистого свертывания. СКм ассоциирован с капошиформной гемангиоэндотелиомой и «пучковой» ангиомой. СКМ характеризуется высокой летальностью до 30%, связанной как с геморрагическими осложнениями, так и с инвазивным ростом опухоли и сдавлением ею жизненно важных структур. Поиск эффективного и безопасного метода лечения СКМ остается насущной проблемой. В последние годы в качестве одного из потенциальных терапевтических агентов рассматривается неселективный бета-блокатор - пропранолол. В качестве возможных механизмов действия рассматриваются вазоконстрикция (в том числе опосредованно подавлением экспрессии эндотелиальной NO-синтетазы), подавление VEGF (фактор роста эндотелия сосудов) и bFGF (основной фактор роста фибробластов) -индуцированной пролиферации клеток эндотелия, индукция апоптоза, а также блокирование матриксных металлопротеиназ (ММPs) и ИЛ-6 (проангиогенный цитокин). Эффективность пропранолола доказана лишь при лечении инфантильных гемангиом.

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Pediatric Hematology/Oncology and Immunopathology. 2016; 15: 28-33

Use of propranolol in management of Kasabach-Merritt syndrome in children

Fedorova Darya V., Khachatryan Lili A.

https://doi.org/10.24287/1726-1708-2016-15-4-28-33

Abstract

Kasabach-Merritt syndrome (KMS) is a condition characterised by the presence of a vascular tumour, consumptive thrombocytopenia and coagulopathy. An impaired system of haemostasis results in development of disseminated intravascular coagulation syndrome. KMS is associated with Kaposiform haemangioendothelioma and «tufted» angioma. KMS is characterized by high lethality to 30% related to both haemorrhagic complications and invasive growth of tumour and its compression of vital structures. A search for an effective and safe method of management of KMS remains an urgent problem. In the past years, propranolol - a nonselective beta-blocker - is considered as one of potential therapeutic agents. The feasible mechanisms of action are considered to be vasoconstriction (including indirect suppression of expression of endothelial NO synthetase), suppression of VEGF (vascular endothelial growth factor) and bFGF (basic fibroblast growth factor) - induced proliferation of endothelial cells, induction of apoptosis, and also blockage of matrix metalloproteinases (ММPs) and IL-6 (proangiogenic cytokine). The effectiveness of propranolol has been proven only for treatment of infantile haemangiomas.

References