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Intern Med. 2020;59(1):93-99. doi: 10.2169/internalmedicine.3315-19. Epub 2020 Jan 1.

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

Author information

1
Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Japan.
2
Department of Infectious Disease, St. Marianna University School of Medicine, Japan.
3
Department of Emergency and Critical Care Medicine, Tokyo Metropolitan Police Hospital, Japan.
4
Department of Obstetrics and Gynecology, Yokohama General Hospital, Japan.
5
Department of Pediatrics, Shiga University of Medical Science, Japan.
6
Department of Molecular Genetics, Wakayama Medical University, Japan.
7
The Japanese Association for Complement Research, Japan.
8
Department of Food and Health Sciences, Rakuno Gakuen University, Japan.

Abstract

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.

KEYWORDS:

TMA; aHUS; atypical hemolytic uremic syndrome; complement amplifying condition; eculizumab; thrombotic microangiopathy

PMID:
31902910
PMCID:
PMC6995713
DOI:
10.2169/internalmedicine.3315-19
[Indexed for MEDLINE]
Free PMC Article

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