Kaposiform hemangioendothelioma with Kasabach–Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus. Issue 12 (1st August 2018)

• Record Type: Journal Article
• Authors: Cashell, Jamie; Smink, Gayle M.; Helm, Klaus; Xavier, Frederico
• Is Part Of: Pediatric blood & cancer. Volume 65:Issue 12(2018)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2018-08-01
• Subjects: kaposiform hemangioendothelioma -- Kasabach–Merritt phenomenon -- sirolimus; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.27305
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: A 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.