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Research advances on the pathogenesis of Kaposi form hemangioendothelioma or tufted angioma and its concomitant Kasabach-Merritt phenomenon

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Author:: No author available

Journal Title:: Chinese Journal of Pediatric Surgery

Issue:: 9

DOI:: 10.3760/cma.j.cn421158-20201230-00780

Key Word:: 血小板;卡波西型血管内皮瘤;丛状血管瘤;Blood platelets;Kaposi form hemangioendothelioma;Tufted hemangioma

Abstract： Kasabach-Merritt phenomenon (KMP) is defined as Kaposiform hemangioendothelioma (KHE) or tufted angioma (TA) associated with fatal thrombocytopenia, together with hypofibrinogenemia and consumptive coagulopathy. With a young onset age and a high fatality rate, KMP progresses rapidly and the wellbeing of children are seriously endangered. The precipitating factors of KMP have remained elusive. The treatment outcomes are poor and there is no unified and high-quality treatment guidelines. This review summarized the pathogenesis of KHE or TA and its concomitant KMP to provide theoretical rationales for proper clinical treatments.

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