A Case of Kasabach-Merritt Syndrome Diagnosed by Fetal Ultrasonography.
- Author:
Ji Eun KIM
1
;
Hee Won CHUEH
;
Eun Young KWON
;
Young Ah LEE
;
Jung Pyo KIM
Author Information
1. Department of Pediatrics, College of Medicine, Dong-A University, Busan, Korea. kjp30802002@yahoo.co.kr
- Publication Type:Case Report
- Keywords:
Kasabach-Merritt syndrome;
Interferon alfa;
Pentoxyfylline
- MeSH:
Fetus;
Hemangioma;
Humans;
Infant;
Interferon-alpha;
Interferons;
Kasabach-Merritt Syndrome*;
Neck;
Platelet Count;
Platelet Transfusion;
Pregnancy;
Thrombocytopenia;
Ultrasonography;
Ultrasonography, Prenatal*
- From:Journal of the Korean Society of Neonatology
2007;14(1):114-118
- CountryRepublic of Korea
- Language:Korean
-
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.
