Autoimmune disorders (SLE, RA, scleroderma, cold agglutinin disease).

Lymphoproliferative disorders (CLL, NHL).

Prosthetic heart valves (ball-and-cage, and bileaflet valves). Usually subclinical, but can be severe in up to 15% of pts.

Family history of hemoglobinopathies or RBC membrane defects (thalassemia, sickle cell disease, G6PD deficiency, spherocytosis).

Exposure to drugs (cephalosporins, penicillins, NSAIDs) or other chemicals (naphthalene, fava beans).

Infection ( Clostridium perfringens , Haemophilus influenza type B, malaria, HIV).

Wilson disease (due to toxic effect of copper ions in circulation).

Anemia, hypoxia.

Underlying CV compromise.

Splenomegaly in pts with extravascular hemolysis (within the reticuloendothelial system). Splenectomy is a common surgical procedure in pts with sickle cell disease due to hemolysis and sickling.

Renal failure due to massive hemolysis (e.g., cold agglutinin hemolysis, sickling, drug reaction)

Varying levels of liver disease depending on type of hemolytic anemia. Synthetic function of liver is usually normal, but in severe cases can be compromised.

Anemia, hypoxia.

Underlying CV compromise.

Splenomegaly in pts with extravascular hemolysis (within the reticuloendothelial system). Splenectomy is a common surgical procedure in pts with sickle cell disease due to hemolysis and sickling.

Renal failure due to massive hemolysis (e.g., cold agglutinin hemolysis, sickling, drug reaction)

Varying levels of liver disease depending on type of hemolytic anemia. Synthetic function of liver is usually normal, but in severe cases can be compromised.

Uncompensated anemia in pts with subacute hemolysis

Periop hemolysis and/or hypoxia

Need for transfusion and/or fluids

Pts with hemolytic anemia may present with any of the following: fatigue, angina, SOB, tachypnea, tachycardia, or jaundice. The hemolysis can lead to changes in blood viscosity, gallstone production, splenomegaly, and renal failure in severe cases. Many pts will be both iron and folate deficient.

Epidemiology varies by pt population. For example, G6PD is an X-linked condition and its prevalence is near 50% in Kurdish Jews, but around 1:1000 in North American and European populations.

Other things to consider incl monitoring periodic Hct levels, and administering prophylactic antibiotics/vaccinations to pts who have had a splenectomy.