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Eculizumab for Severe Thrombotic Microangiopathy Secondary to Surgical Invasive Stress and Bleeding

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare condition caused by an excessive activation of the complement pathway based on genetic or acquired dysfunctions in complement regulation, leading to thrombotic microangiopathy (TMA). A complement-amplifying condition (CAC) can trigger aH...

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Detalles Bibliográficos
Autores principales: Fujita, Yoko, Terashita, Maho, Yazawa, Masahiko, Yamasaki, Yukitaka, Imamura, Tomonori, Kibayashi, Junichiro, Sawai, Toshihiro, Hidaka, Yoshihiko, Ohtani, Katsuki, Inoue, Norimitsu, Shibagaki, Yugo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6995713/
https://www.ncbi.nlm.nih.gov/pubmed/31902910
http://dx.doi.org/10.2169/internalmedicine.3315-19
Descripción
Sumario:Atypical hemolytic uremic syndrome (aHUS) is an extremely rare condition caused by an excessive activation of the complement pathway based on genetic or acquired dysfunctions in complement regulation, leading to thrombotic microangiopathy (TMA). A complement-amplifying condition (CAC) can trigger aHUS occurrence along with complement abnormality. We herein report a case of severe TMA after laparoscopic myomectomy in a healthy woman. This case was eventually diagnosed as complement-mediated TMA secondary to surgical invasive stress as a CAC, with no definitive diagnosis of aHUS despite a genetic test. The patient fully recovered after several eculizumab administrations.