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J Med Assoc Thai. 2012 May;95 Suppl 5:S181-5.

Outcome of Kasabach-Merritt phenomenon: the role of vincristine as monotherapy: report of a case.

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1
Division of Pediatric Hematology/Oncology, Department of Pediatrics, Phramongkutklao College of Medicine, Bangkok, Thailand. ctrivaree@yahoo.com

Abstract

Kasabach-Merrit phenomenon (KMP) is a rare disorder of pediatric hematological malignancies which is previously referred to Kasabach-Merrit syndrome (KMS). The disorder is characterized by prominent vascular mass resulting from abnormal proliferation of blood vessels, consumption coagulopathy and thrombocytopenia. The diagnosis is based upon three basic findings as above. The authors describe a 6-month-old girl with a huge ecchymotic mass at left buttock who is found to have thrombocytopenia and consumption coagulopathy. The clinical and imaging studies strongly suggested the diagnosis of KMP. Vincristine was administered after a trial of corticosteroids was failed to show clinical and laboratory improvement. After 2 weeks, the patient showed that the platelet count and fibrinogen level become to be normal without blood transfusion and gradually decreased in tumor size without any surgical procedure in 4 weeks and disappeared in 5 months without any complications. In the present report, the authors present the findings from successfully specific treatment with vincristine alone and supportive treatment for life-threatening hemorrhage with platelet concentrate and cryoprecipitate should be the best management in our situation.

PMID:
22934467
[Indexed for MEDLINE]

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