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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.
Afiliação
  • Duan L; Departments of Paediatrics.
  • Renzi S; Paediatrics, Division of Haematology/Oncology.
  • Weidman D; Paediatrics, Division of Haematology/Oncology.
  • Waespe N; Paediatrics, Division of Haematology/Oncology.
  • Chami R; Swiss Childhood Cancer Registry, Institute of Social and Preventive Medicine, University of Bern, Switzerland.
  • Manson D; Paediatric Laboratory Medicine.
  • Cada M; Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada.
  • Carcao M; Paediatrics, Division of Haematology/Oncology.
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.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Derrame Pericárdico / Sarcoma de Kaposi / Derrame Pleural Maligno / Sirolimo / Síndrome de Kasabach-Merritt / Hemangioendotelioma Tipo de estudo: Diagnostic_studies / Guideline Limite: Humans / Male / Newborn Idioma: En Revista: J Pediatr Hematol Oncol Assunto da revista: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Ano de publicação: 2020 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Derrame Pericárdico / Sarcoma de Kaposi / Derrame Pleural Maligno / Sirolimo / Síndrome de Kasabach-Merritt / Hemangioendotelioma Tipo de estudo: Diagnostic_studies / Guideline Limite: Humans / Male / Newborn Idioma: En Revista: J Pediatr Hematol Oncol Assunto da revista: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Ano de publicação: 2020 Tipo de documento: Article