Successful exclusive propranolol therapy in an infant with life-threatening Kasabach–Merritt syndrome

摘要

We describe the successful treatment of an infant with life-threatening Kasabach–Merritt syndrome (KMS) who received propranolol therapy. A full-term, 6-week-old female infant, in poor general condition, was admitted with a large vascular tumor of right buttock, perineal region, extending to a lumbar region and the right thigh. There was a gray-blue, tense, shiny, warm swelling over the site of vascular tumor. The child had platelet count of 20,000?cells/mm. d -dimer was positive and suggestive of consumptive coagulopathy. A diagnosis of KMS was made. Computer tomography (CT) imaging was suggestive of a vascular tumor – Kaposiform hemangioendothelioma. The baby was initially treated with platelet, fresh frozen plasma and blood transfusions. There was temporary rise in platelets to 118,000?cells/mm but after transfusions the platelets dropped to 20,000?cells/mm and the baby developed purpuric swelling over right thigh and right buttock. Platelet transfusions were discontinued and propranolol treatment was started. From the 4th day, the swelling started regressing and the platelet count rose to 73,000?cells/mm. At the age of 2 years, the tumor has nearly completely involuted. In our case propranolol proved to be effective first-line treatment for Kasabach–Merritt syndrome associated with a vascular tumor – Kaposiform hemangioendothelioma. Minor side effect was poor weight gain after prolonged treatment.