Kasabach-Merritt Syndrome Is Not Associated with Typical Hemangiomas

Kasabach-Merritt syndrome, in which platelet trapping leads to life-threatening thrombocytopenia, is considered a complication of hemangioma. This study of 22 children with Kasabach-Merritt syndrome shows that the vascular lesion associated with this syndrome is not the classic type of infantile hemangioma.

The children had violaceous, painful, aggressively infiltrative masses that appeared between birth and 13 months of age and grew rapidly. All patients had severe thrombocytopenia and consumptive coagulopathy. The mean duration of the thrombocytopenia was three years. When platelet consumption ended, the tumor mass decreased markedly in size but a vascular lesion persisted. What remained was residual vascular neoplasia, not the fibrofatty …