A new pathological perspective on thrombotic microangiopathy

Abstract

Thrombotic microangiopathy (TMA) refers to a condition caused by microvascular injury that includes thrombosis, hemolytic anemia, and thrombocytopenia. There are two classic TMAs, hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura, as well as an atypical HUS (aHUS). aHUS includes a broad spectrum of disorders with diverse etiologies and shares clinical manifestations with classic TMA; however, it frequently lacks typical clinical and laboratory findings. These traits can confuse clinicians and pathologists in terms of renal pathologic diagnosis, especially in cases where TMA is associated with other glomerulopathies or hypertensive renal disease. In this review, new paradigms for classifying TMA and the diversity of histopathologic changes including associated renal diseases are discussed. Renal biopsy is an important and useful diagnostic tool for diagnosing TMA and identifying TMA changes in other renal diseases, including hypertension. Adopting the term “TMA features” for TMA-like changes in glomerulus or artery/arteriole in addition to the pathological diagnosis of glomerulopathy would be informative to clinicians for a prompt diagnosis and treatment of aHUS.

Keywords

• atypical hemolytic uremic syndrome
• classification
• complement c4d
• pathology
• thrombotic microangiopathies