Atypical presentation of paroxysmal nocturnal hemoglobinuria treated by eculizumab: A case report.

Date publication

mars 2017

Journal

Medicine

Auteurs

Membres identifiés du Cancéropôle Est :
Pr DELMER Alain

Tous les auteurs :
Quinquenel A, Maestraggi Q, Lecoq-Lafon C, Peffault de Latour R, Delmer A, Servettaz A

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/28328837

Résumé

Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant acquired hematopoietic stem cell disease, which can be revealed by hemolytic anemia, thromboembolism, or bonemarrow failure. Thrombosis can occur at any site, but coronary thrombosis is extremely rare. Controlled trials have demonstrated that eculizimab, an inhibitor of the terminal complement cascade, was able to reduce both hemolysis and thrombosis, but its efficacy in cases of PNH with coronary thrombosis is unknown.

Mots clés

Aged, Antibodies, Monoclonal, Humanized, therapeutic use, Coronary Thrombosis, diagnosis, Coronary Vessels, diagnostic imaging, Delayed Diagnosis, Fatal Outcome, Hemoglobinuria, Paroxysmal, diagnosis, Humans, Immunologic Factors, therapeutic use, Male

Référence

Medicine (Baltimore). 2017 03;96(12):e6403