This site is targeted at medical and radiology professionals, contains user contributed content, and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Kasabach-Merritt syndrome

Kasabach-Merritt syndrome (also known as hemangioma thrombocytopenia syndrome) is a rare life-threatening disease found in infant in which a rapidly growing vascular tumor is responsible for thrombocytopenia, microangiopathic hemolytic anemia and consumptive coagulopathy.

Aetiology

Kasabach-Merrit syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma.

Pathophysiology

Vascular tumors may grow very rapidly, which can induce severe thrombocytopenia and severe consumptive coagulopathy. In severe case, there may be disseminated intravascular coagulation.

Radiographic features

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

Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolisation and/or 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.

Etymology

The syndrome is named after Haig Haigouni Kasabach and Katharine Krom Merritt, the two pediatricians who first described the condition in 1940.

Updating… Please wait.
Loadinganimation

Alert_accept

Error Unable to process the form. Check for errors and try again.

Alert_accept Thank you for updating your details.