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

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

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Complement Inactivating Agents / therapeutic use
  • Female
  • Humans
  • Laparoscopy / adverse effects*
  • Postoperative Hemorrhage / complications*
  • Rare Diseases
  • Thrombotic Microangiopathies / diagnosis
  • Thrombotic Microangiopathies / drug therapy*
  • Thrombotic Microangiopathies / etiology
  • Uterine Myomectomy / adverse effects*

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement Inactivating Agents
  • eculizumab