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Pediatric blood & cancer · Dec 2018
Case ReportsKaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus.
- Jamie Cashell, Gayle M Smink, Klaus Helm, and Frederico Xavier.
- Department of Pediatric Hematology/Oncology, Penn State Health Children's Hospital, Hershey, Pennsylvania.
- Pediatr Blood Cancer. 2018 Dec 1; 65 (12): e27305.
AbstractA full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach-Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.© 2018 Wiley Periodicals, Inc.
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