Sirolimus Treatment of an Infant With Intrathoracic Kaposiform Hemangioendothelioma Complicated by Life-threatening Pleural and Pericardial Effusions.
J Pediatr Hematol Oncol
; 42(1): 74-78, 2020 01.
Article
em En
| MEDLINE
| ID: mdl-30044355
ABSTRACT
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Derrame Pericárdico
/
Sarcoma de Kaposi
/
Derrame Pleural Maligno
/
Sirolimo
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Síndrome de Kasabach-Merritt
/
Hemangioendotelioma
Tipo de estudo:
Diagnostic_studies
/
Guideline
Limite:
Humans
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Male
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Newborn
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article