|
KMID : 0438520070140010114
|
|
Journal of the Korean Society of Neonatology
2007 Volume.14 No. 1 p.114 ~ p.118
|
|
|
A Case of Kasabach-Merritt Syndrome Diagosed by Fetal Ultrsonography
|
|
Kim Ji-Eun
Lee Young-Ah
Chueh Hee-Won
Kwon Eun-Young
Kim Jung-Pyo
|
|
|
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/μL) 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. (J Korean Soc Neonatol 2007;14:114-118)
|
|
|
KEYWORD
|
|
|
Kasabach-Merritt syndrome, Interferon alfa, Pentoxyfylline
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
  
|