Pharmacological Treatment Leading to Complete Resolution in Kasabach-Merritt Phenomenon-Case Report
Kasabach-Merritt syndrome is a rare vascular tumor usually seen in infancy. It is locally aggressive and infiltrates the skin, subcutaneous tissue, and muscles. It is characterized by coagulopathy with thrombocytopenia, microangiopathic hemolytic anemia, and raised d-dimer levels. In small infants,...
| Published in: | Journal of Pediatric Research |
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| Main Authors: | , , , , , |
| Format: | Article |
| Language: | English |
| Published: |
Ege University, Faculty of Medicine, Department of Pediatrics and Ege Children Foundation
2025-03-01
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| Subjects: | |
| Online Access: | https://jpedres.org/articles/pharmacological-treatment-leading-to-complete-resolution-in-kasabach-merritt-phenomenon-case-report/doi/jpr.galenos.2024.20270 |
| Summary: | Kasabach-Merritt syndrome is a rare vascular tumor usually seen in infancy. It is locally aggressive and infiltrates the skin, subcutaneous tissue, and muscles. It is characterized by coagulopathy with thrombocytopenia, microangiopathic hemolytic anemia, and raised d-dimer levels. In small infants, this can cause life-threatening bleeding and can be fatal. We report on a two-month-old female child who presented to us with a rapidly enlarging purplish swelling on the right arm. It was also associated with petechial spots all over the body. A clinical diagnosis of hemangioma with Kasabach-Merritt phenomenon was made and was further confirmed by hematological investigations which showed anemia, and thrombocytopenia with hypofibrinogenemia. Imaging of the limb confirmed the diagnosis. After taking parental consent, the baby was started on injections of vincristine weekly with oral prednisolone. There was a significant reduction in the tumor’s size and improved blood parameters. After 6 weeks of steroid therapy, the medication was tapered and the child was changed to single agent sirolimus with monitoring of serum levels. There was a remarkable response to sirolimus with complete resolution of the tumour and Kasabach-Merritt phenomenon. Kasabach-Merritt syndrome can be a life-threatening complication in infants. Appropriate pharmacological therapy with stringent monitoring can bring complete resolution. |
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| ISSN: | 2147-9445 2587-2478 |