The Japanese Journal of Pediatric Hematology
Online ISSN : 1884-4723
Print ISSN : 0913-8706
ISSN-L : 0913-8706
Successful Methylprednisolone High-Dose Therapy with Irradiation in a Case of Kasabach-Merritt Syndrome
Takashi SHINOZAWANobuo NAKAMURANaohiro DANYAKaiko OKAZAKIAtsushi SHIBUYA
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1990 Volume 4 Issue 1 Pages 69-73

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Abstract
Combination therapy with high-dose methylprednisolone (m-PSL) and irradiation was performed in an infant with Kasabach-Merritt syndrome. She was a two-month-old infant with a giant hemangioma-29 cm in diameter-around her left thigh. After open biopsy in the center of the tumor, the size of the tumor increased up to 40 cm in diameter. Abnormal findings of blood coagulation data and thrombocytopenia appeared at the same time and showed DIC. Initial treatment for DIC and hemangioma consisted of heparin, gabexate mesilate (FOY), antithrombin III (AT-III) and irradiation however, all proved ineffective : the size of the hemangioma remained the same and frequent transfusions including platelet and fresh frozen plasma (FFP) were necessary. There was quick response to high-dose m-PSL therapy combined with irradiation as the second treatment. The tumor decreased and no transfusion was necessary. The combination of high-dose m-PSL and irradiation therapy thus proved to be a useful treatment for life-threatening DIC complicated with bleeding after performing the biopsy in the hemangioma.
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