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Kasabach-Merritt syndrome, also known as hemangioma thrombocytopenia syndrome, is a rare life-threatening disease found mostly 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.
Kasabach-Merritt syndrome is most seen in infants with kaposiform hemangioendothelioma or tufted angioma. Nonetheless, other vascular tumors may be responsible, such as infantile hemangioma, and rare cases have also been described in adults with giant liver hemangiomas 5-6.
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, including systemic corticosteroids, radiation therapy, compression, embolization and pharmacotherapy (e.g. interferon, platelet aggregation inhibitors) 4.
Repetitive hemarthrosis can also occur, leading to a hemophilia-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 pediatricians who first described the condition in 1940.
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- 4. Hesselmann S, Micke O, Marquardt T et-al. Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha. Br J Radiol. 2002;75 (890): 180-4. Br J Radiol (full text) - Pubmed citation
- 5. Albitar H & Iyer V. Giant Liver Hemangioma with Kasabach-Merritt Syndrome. Am J Med. 2020;133(1):56-7. doi:10.1016/j.amjmed.2019.06.024 - Pubmed
- 6. EASL Clinical Practice Guidelines on the Management of Benign Liver Tumours. J Hepatol. 2016;65(2):386-98. doi:10.1016/j.jhep.2016.04.001 - Pubmed