Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in an infant: treatment with interferon.

ABSTRACT

A 2-week-old infant developed respiratory failure due to a mediastinal Kaposiform hemangioendothelioma that was complicated by thrombocytopenia and consumptive coagulopathy. Initial surgery was unsuccessful at removing the tumorous infiltration of mediastinal structures. Multiple transfusions with fresh frozen plasma, platelets, and red blood cells were needed for the consumptive coagulopathy, and ventilatory support was required for 5 months. Therapy for the tumor included methylprednisolone, aminocaproic acid, and vincristine, but a sustained response was achieved only after the initiation of alpha interferon. The patient was monitored closely and did not develop neurologic toxicity. This case demonstrates that interferon can be used to treat infants with Kaposiform hemangioendothelioma in life-threatening situations that do not respond to other forms of treatment.