Kasabach-Merritt syndrome, also known as hemangioma thrombocytopenia syndrome, is a rare life-threatening disease found in infants in which a rapidly growing vascular tumor is responsible for thrombocytopenia, microangiopathic hemolytic anemia and consumptive coagulopathy.
Vascular tumors may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation.
The radiographic features are specific to each vascular tumor. They can be found in multiple locations, more frequently subcutaneous, musculoskeletal, splenic and hepatic in location.
Treatment and prognosis
Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolization and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) .
Repetitive hemarthrosis can also occur, leading to a hemophilia-like arthropathy . Other complications include congestive heart failure and gastrointestinal bleeding .
History and etymology
The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.
- kongenitale vaskuläre Malformationen
- infantiles Leberhämangioendotheliom