Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas

摘要: Abstract Objective: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is benign tumor that grows in infancy characterized by proliferation of endothelial cells regression during childhood. Although Kasabach-Merritt syndrome (KMS) frequently mentioned as possible complication hemangioma, our experience suggests the anatomic underlying thrombocytopenia not "true," classic, involuting type hemangioma Study design: We reviewed clinical hemostasis data response to treatment 22 cases KMS, we analyzed biopsy specimens 15 them. Results: Clinically none patients had classic hemangioma. There was no female preponderance. All severe (lowest platelet count=3000/mm 3 ) consumption fibrinogen. Histologically, typical "capillary," infancy: exhibited either tufted angioma or kaposiform hemangioendothelioma pattern; all also contained numerous abnormal lymphatic-like vessels; lymphatic malformation major component two patients. The infants heterogeneous number therapeutic regimens, noted other reports. Severe morbidity present; three died, one leg amputation. "Residua" were, fact, residual neoplasia, variable duration, stable fibrofatty residuum, involuted hemangioma; only hematologic phenomenon "cured" after period years. Conclusions: KMS distinctive disease infancy, but infancy. This different resemblance pathologically association vessels. Whether single entity cannot be definitely concluded from this study. need better understanding pathogenesis improve management. (J Pediatr 1997;130:631-40)