J Korean Soc Neonatol.  2007 May;14(1):114-118.

A Case of Kasabach-Merritt Syndrome Diagnosed by Fetal Ultrasonography

• Kim JE
• Chueh HW
• Kwon EY
• Lee YA
• Kim JP

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.

Keyword

Kasabach-Merritt syndrome; Interferon alfa; Pentoxyfylline

MeSH Terms

Fetus
Hemangioma
Humans
Infant
Interferon-alpha
Interferons
Kasabach-Merritt Syndrome*
Neck
Platelet Count
Platelet Transfusion
Pregnancy
Thrombocytopenia
Ultrasonography
Ultrasonography, Prenatal*
Interferon-alpha
Interferons