PMID: 9108863Apr 1, 1997Paper

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas

The Journal of Pediatrics
O EnjolrasJ P Escande


In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a "true," classic, involuting type of hemangioma of infancy and childhood. We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical "capillary," involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The...Continue Reading


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