Kasabach-Merritt syndrome, also known as haemangioma thrombocytopenia syndrome, is a rare life-threatening disease found mostly in infants in which a rapidly growing vascular tumour is responsible for thrombocytopenia, microangiopathic haemolytic anaemia, and consumptive coagulopathy.
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Pathology
Vascular tumours may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe cases, there may be disseminated intravascular coagulation.
Aetiology
Kasabach-Merritt syndrome is most seen in infants with kaposiform haemangioendothelioma or tufted angioma. Nonetheless, other vascular tumours may be responsible, such as infantile haemangioma, and rare cases have also been described in adults with giant liver haemangiomas 5-6.
Radiographic features
The radiographic features are specific to each vascular tumour. 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, including systemic corticosteroids, radiation therapy, compression, embolisation and pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) 4.
Complications
Repetitive haemarthrosis can also occur, leading to a haemophilia-like arthropathy 1. Other complications include congestive heart failure and gastrointestinal bleeding 3.
History and etymology
The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two paediatricians who first described the condition in 1940.
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