Diagnosis: Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP).
MICROSCOPIC FINDINGS AND CLINICAL COURSE
Hematoxylin-eosin–stained sections of the punch biopsy specimen showed a vascular proliferation within the dermis that was composed of spindled endothelial cells diffusely infiltrating the collagen fibers as well as areas with a more lobular architecture. Rare mitotic figures were seen.
Our patient received intravenous methylprednisolone and vincristine therapy in the hospital. Within 3 weeks, he was discharged with instructions to return for weekly infusions of vincristine and tapering doses of methylprednisolone. At his most recent follow-up, 5 months after initial presentation, he was clinically stable with no significant bleeding episodes. The tumor has showed partial reduction in size as a result of treatment.
DISCUSSION
Rarely, vascular tumors may be complicated by KMP, which is a consumptive coagulopathy that typically presents in the first few weeks of life (mean age at onset, 7 weeks). It is characterized by the triad of hemangioma, thrombocytopenia, and coagulopathy. The underlying hemangioma may be an obvious . . . [Full Text of this Article]
