Snapshot A 30-year-old man presents to urgent care for a week duration of fatigue, weakness, and nausea. He endorsed several episodes of nonbloody diarrhea. He also reports a rash on his lower extremities. On physical exam, he is noted to have nonblanching red papules scattered on his lower extremities. Laboratory examination show thrombocytopenia as well as anemia. His creatinine is mildly elevated. A peripheral blood smear shows fragmented red blood cells. Concerned, his physician sends a specific laboratory test for ADAMTS13 activity. He is treated with plasma exchange therapy. Introduction Overview fragmentation hemolysis is an example of a non-immune mediated extrinsic hemolytic anemia due to mechanical destruction of the red blood cells (RBCs) often associated with microvascular abnormalities, hence the term microangiopathic hemolytic anemia Epidemiology Risk factors and associated conditions prosthetic heart valve Waring blender syndrome hemolytic anemia in the setting of prosthetic heart valves HELLP syndrome disseminated intravascular coagulopathy (DIC) thrombotic thrombocytopenic purpura (TTP) hemolytic uremic syndrome (HUS) drug-induced thrombotic microangiopathy (DITMA) quinine, oxymorphone, and ecstasy malignancy malignant hypertension scleroderma renal crisis Kasabach-Merritt phenomenon rare consumptive coagulopathy associated with vascular tumors ETIOLOGY Pathogenesis mechanism RBCs are often sheared by a variety of factors, including fibrin strand formation or foreign body such as mechanical valves Presentation Symptoms common symptoms pallor lightheadedness jaundice other signs of underlying conditions petechiae or purpura dark urine bleeding splenomegaly Studies Serum labs hemolysis labs increased indirect bilirubin increased lactate dehydrogenase (LDH) decreased haptoglobin Peripheral blood smear schistocytes, also known as helmet cells small and irregular triangular or crescent-shaped cells, with lack of central pallor Differential Acute hemolytic reaction (ABO incompatibility) key distinguishing factors often occurs acutely during transfusion presents with fevers, chills, and hypotension Treatment Medical treat underlying medication condition Complications Renal failure Shock