Sirolimus for Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon in a Neonate

Author:

Cabrera Trevor B.1,Speer Allison L.2,Greives Matthew R.3,Goff Donna A.4,Menon Neethu M.5,Reynolds Eric W.6

Affiliation:

1. Department of Pediatrics, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

2. Department of Pediatric Surgery, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

3. Department of Pediatric Plastic Surgery, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

4. Department of Pediatric Cardiology, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

5. Department of Pediatric Hematology, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

6. Department of Neonatal/Perinatal Medicine, University of Texas Health Science Center at Houston/McGovern Medical School, Houston, Texas

Abstract

AbstractWe present a case of a neonate born with kaposiform hemangioendothelioma (KHE), complicated by Kasabach-Merritt phenomenon (KMP) and other serious conditions, who was successfully treated with sirolimus. In addition to complications from thrombocytopenia and fluid overload, during the course of therapy, our patient experienced supratherapeutic drug levels at the commonly accepted starting dose of sirolimus. Patients with KHE and KMP should be closely monitored for potential complications of both the initial disease and unexpected side effects of treatments.

Publisher

Georg Thieme Verlag KG

Subject

Obstetrics and Gynaecology,Pediatrics, Perinatology, and Child Health

Reference30 articles.

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3. 2014 revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update;A C Merrow;Radiographics,2016

4. Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion;M A Walsh;J Pediatr Hematol Oncol,2008

5. Kasabach-Merritt syndrome;M el-Dessouky;J Pediatr Surg,1988

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