[Hemolytic and uremic syndrome and related thrombotic microangiopathies: Epidemiology, pathophysiology and clinics]

Rev Med Interne. 2017 Dec;38(12):817-824. doi: 10.1016/j.revmed.2017.06.004. Epub 2017 Jul 12.
[Article in French]


Thrombotic microangiopathies (TMA) represent an eclectic group of conditions, which share hemolytic anemia and thrombocytopenia as a common defining basis. Remarkable breakthroughs in the physiopathological setting have allowed for a thorough recomposition of the disparate syndromes, which form the constellation of TMA. In this view, clinicians now discriminate thrombocytopenic thrombotic purpura (TTP) defined by a severe deficiency in ADAMTS13, which is rarely associated with a severe renal involvement and the hemolytic and uremic syndrome (HUS) in which renal impairment is the most prominent clinical feature. HUS can result from toxins stemming from bacterial infections of the digestive tract, alternate complement pathway abnormalities, metabolic or coagulation disorders or, lastly, drug and various toxic compounds. The diverse forms of HUS reflect the insights gained in the understanding of the pathophysiological mechanisms underpinning TMA. In this first part, a broad overview of the epidemiological, physiopathological and clinical aspects of HUS and related TMA syndromes is presented.

Keywords: Acute kidney injury; Enterohemorrhagic Escherichia coli; Escherichia coli entérohémorragique; Hemolytic and uremic syndrome; Insuffisance rénale aiguë; Microangiopathie thrombotique; Shiga toxin; Shigatoxine; Syndrome hémolytique et urémique; Thrombotic microangiopathy.

Publication types

  • Review

MeSH terms

  • Acute Kidney Injury / diagnosis
  • Acute Kidney Injury / epidemiology
  • Acute Kidney Injury / etiology
  • Atypical Hemolytic Uremic Syndrome / complications
  • Atypical Hemolytic Uremic Syndrome / epidemiology
  • Hemolytic-Uremic Syndrome* / complications
  • Hemolytic-Uremic Syndrome* / diagnosis
  • Hemolytic-Uremic Syndrome* / epidemiology
  • Humans
  • Purpura, Thrombotic Thrombocytopenic / complications
  • Purpura, Thrombotic Thrombocytopenic / diagnosis
  • Purpura, Thrombotic Thrombocytopenic / epidemiology
  • Thrombotic Microangiopathies* / diagnosis
  • Thrombotic Microangiopathies* / epidemiology
  • Thrombotic Microangiopathies* / etiology