Sirolimus Treatment of an Infant With Intrathoracic Kaposiform Hemangioendothelioma Complicated by Life-threatening Pleural and Pericardial Effusions.

Duan, Lucy; Renzi, Samuele; Weidman, Danielle; Waespe, Nicolas; Chami, Rose; Manson, David; Cada, Michaela; Carcao, Manuel (2018). Sirolimus Treatment of an Infant With Intrathoracic Kaposiform Hemangioendothelioma Complicated by Life-threatening Pleural and Pericardial Effusions. (In Press). Journal of pediatric hematology, oncology Lippincott Williams & Wilkins 10.1097/MPH.0000000000001268

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Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that may be associated with Kasabach-Merritt Phenomenon (KMP), which is a consumptive coagulopathy with potentially life-threatening thrombocytopenia. Management of KHE and KMP is challenging, and currently, there are no standardized validated treatment protocols. Mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of KHE. We describe a term male who presented as a diagnostic dilemma with life-threatening pleural and pericardial effusions and severe thrombocytopenia. After extensive work-up the etiology for his condition was determined to be KHE with KMP. The patient was commenced on sirolimus and responded well to therapy with resolution of KMP.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine

UniBE Contributor:

Waespe, Nicolas

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

1077-4114

Publisher:

Lippincott Williams & Wilkins

Language:

English

Submitter:

Tanya Karrer

Date Deposited:

28 Aug 2018 14:00

Last Modified:

23 Oct 2019 13:25

Publisher DOI:

10.1097/MPH.0000000000001268

PubMed ID:

30044355

BORIS DOI:

10.7892/boris.119341

URI:

https://boris.unibe.ch/id/eprint/119341

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