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

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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.

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Darya V. Fedorova

Dmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: darya.v.fedorova@gmail.com
Russian Federation

Lili A. Khachatryan

Dmitry Rogachev Federal Research Centre of Paediatric Haematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Email: lili.2510@yandex.ru
Russian Federation

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Copyright (c) 2016 Fedorova D.V., Khachatryan L.A.

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