A Case of Kasabach-Merritt Syndrome Diagnosed by Fetal Ultrasonography.

Kim,  Ji Eun; Chueh,  Hee Won; Kwon,  Eun Young; Lee,  Young Ah; Kim,  Jung Pyo

Case Report

Journal of the Korean Society of Neonatology 2007;14(1):114-118.
Published online May 1, 2007.

A Case of Kasabach-Merritt Syndrome Diagnosed by Fetal Ultrasonography.

Ji Eun Kim, Hee Won Chueh, Eun Young Kwon, Young Ah Lee, Jung Pyo Kim

Department of Pediatrics, College of Medicine, Dong-A University, Busan, Korea. kjp30802002@yahoo.co.kr

산전 진찰에서 발견된 Kasabach-Merritt 증후군 치료 경험 1례

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Abstract

In 1940, Kasabach and Merritt first described the association of a large vascular tumor and thrombocytopenia and termed this Kasabach-Merritt syndrome. This is characterized by a rapidly enlarging hemangioma with thrombocytopenia and coagulopathy. We describe an infant with a large complex vascular lesion of the face that was diagnosed in utero and successfully treated interferon and pentoxyfylline. In a fetus at 36+0 weeks gestation, ultrasound demonstrated abnormality of the face and neck. she was delivered at 38+5 weeks gestation with a large facial hemangioma. She had severe thrombocytopenia in the first 24hr of life (platelets 22,000/microliter) and she was commenced on oral steroids(prednisolone 2 mg/kg/day). The hemangioma continued to grow and the baby required frequent platelet transfusions. When the infant was 17 days old, treatment with pentoxyfylline and subcutaneous alpha interferon(initially 60,000 units/m2/day) was started. From 1 month of age, the hemangioma gradually reduced in volume and the platelet count stared to rise. Recent, the hemangioma progressively diminished in size without any serious complication.

Key Words: Kasabach-Merritt syndrome; Interferon alfa; Pentoxyfylline