Idiopathic Atypical Hemolytic Uremic Syndrome: A Case Report

Abstract

Thrombotic microangiopathies (TMAs) are a spectrum of disorders characterized by hemolytic anemia, thrombocytopenia, and formation of microthrombi in blood vessels, resulting in tissue injury due to compromised blood flow. This classical presentation of TMA is characterized by varying degrees of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and ischemic damage to the brain, kidneys, heart, lungs and GI tract. TMA can be classified into different types based upon the etiology and pathogenesis like thrombotic thrombocytopenic purpura (TTP), Complement mediated TMA (atypical Hemolytic uremic syndrome [aHUS]), infection associated TMA (shiga toxin producing E.coli, streptococcus pneumoniae) secondary to systemic causes like malignancy, autoimmune diseases and medications. In this article, we discuss a case of a female diagnosed with aHUS with acute kidney injury complicated by hypertension and severe anemia. As aHUS is known to be associated with high mortality and morbidity, prompt recognition of aHUS is crucial for efficient treatment. Here we have discussed the clinical presentation, histopathological hallmarks, and the diagnostic approach for TMA.