Kasabach-Merritt syndrome

Kasabach-Merritt syndrome (also known as haemangioma thrombocytopaenia syndrome) is a rare life-threatening disease found in infant in which a rapidly growing vascular tumour is responsible for thrombocytopaenia, microangiopathic haemolytic anemia and consumptive coagulopathy.

Pathology

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

Aetiology

Kasabach-Merritt syndrome is usually caused by kaposiform haemangioendothelioma or tufted angioma. Other vascular tumours may be responsible, such as infantile haemangioma.

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 - 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.

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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Article Information

rID: 1549
Section: Syndromes
Tags: cases, syndrome
Synonyms or Alternate Spellings:
  • Kasabach Merritt syndrome
  • Kasabach and Merritt syndrome
  • Hemangioma thrombocytopenia syndrome
  • Haemangioma thrombocytopenia syndrome
  • Haemangioma thrombocytopaenia syndrome
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