Key Takeaways

Hemolysis may occur with or without anemia. Hemolysis without anemia is called compensated hemolysis.

Hemolytic markers include blood cells (reticulocytes), serum/plasma markers (LDH, haptoglobin, AST, bilirubin), and hemoglobinuria and/or hemosidinuria.

Some hemolytic markers derive directly from lysed red cells (LDH and bilirubin), while others are indirect markers of cell lysis, including haptoglobin and bilirubin.

The peripheral smear is helpful for narrowing the differential diagnosis of hemolysis. Schistocytes are observed in cases of thrombotic microangiopathy (for example, thrombotic thrombocytopenia purpura) or microangiopathic hemolytic anemia (for example foot strike anemia and valve hemolysis (we use MAHA to describe valve hemolysis, but a paravalvular leak is not really microvascular in nature).

Valve hemolysis is seen in patients who have had surgical or transcatheter implantation of an aortic or mitral valve, less commonly in the setting of valve repair, and rarely in the case of native stenotic valves.

Diagnosis of valve hemolysis is suggested by otherwise unexplainable anemia in a patient with valve replacement/repair whose labs demonstrate non-immune hemolysis (hemolytic indices) with or without anemia. Diagnosis is more or less confirmed by showing a paravalvular leak on echocardiogram.

Valve hemolysis may lead to renal dysfunction from the toxic effects of hemoglobinuria.

Treatment of valve hemolysis includes both medical and interventional (transcatheter or open surgery) approaches.

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