Atypical Presentation of Pregnancy-Related Hemolytic Uremic Syndrome

Am J Kidney Dis. 2018 Sep;72(3):451-456. doi: 10.1053/j.ajkd.2017.11.006. Epub 2018 Jan 10.

Abstract

The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI. We use this as an opportunity to review the clinical presentation and pathophysiology of preeclampsia, eclampsia, and the TMAs. We focus on diagnostic challenges, especially because many patients with TMA do not present with thrombocytopenia, which can delay diagnosis. We additionally review the clinical settings in which administration of eculizumab, a C5 membrane attack complex inhibitor, is appropriate.

Keywords: Acute kidney injury (AKI); differential diagnosis; eclampsia; hemolytic uremic syndrome (HUS); kidney biopsy; postpartum period; pre-eclampsia; pregnancy; thrombotic microangiopathy (TMA).

Publication types

  • Case Reports

MeSH terms

  • Atypical Hemolytic Uremic Syndrome / blood*
  • Atypical Hemolytic Uremic Syndrome / diagnostic imaging*
  • Atypical Hemolytic Uremic Syndrome / therapy
  • Female
  • Humans
  • Plasma Exchange / methods
  • Postnatal Care / methods
  • Pregnancy
  • Pregnancy Complications / blood*
  • Pregnancy Complications / diagnostic imaging*
  • Pregnancy Complications / therapy
  • Young Adult