Hemolytic anemia
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Synopsis

- Inherited hemolytic anemias – Generally due to abnormal hemoglobin molecules as seen in sickle cell anemia or thalassemia, or due to abnormalities of the RBC membranes resulting in excess splenic sequestration.
- Acquired hemolytic anemias – These can be due to exposure to certain drugs, certain infections (bacterial or viral), autoimmune etiologies, hematologic malignancies, mechanical destruction (due to mechanical heart valves, including potential leakage around the site of insertion), or sequestration in the setting of hypersplenism.
Intravascular hemolysis occurs within the vessel due to either mechanical trauma (from damaged endothelium) or complement fixation / activation on cell surface, or from an infectious agent. Extravascular hemolysis occurs outside of blood vessels in the spleen or liver when RBCs are removed or destroyed due to membrane surface defects or surface antibodies. Autoantibodies that lead to extravascular hemolysis may be categorized as "warm" (usually immunoglobulin G [IgG] antibodies), which bind to RBCs at body temperature, or "cold" (usually IgM antibodies), which bind to RBCs at below body temperature, producing cold agglutinins (ie, cold agglutinin disease).
Specific types of hemolytic anemia have their own incidence, demographics, and unique risk factors.
Types of hemolytic anemias include but are not limited to:
- Acute hemolytic transfusion reaction
- Disseminated intravascular coagulation
- Thrombotic microangiopathy
- RBC parasites (babesia, malaria)
- Drug-induced hemolysis (see drug-induced anemia)
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Autoimmune hemolytic anemia
- Thalassemia (alpha and beta)
- Sickle cell disease
- Hereditary spherocytosis
- Hereditary elliptocytosis
- Mechanical hemolytic anemia due to mechanical aortic valves
- Spur cell hemolytic anemia (SCHA)
Codes
ICD10CM:D59.9 – Acquired hemolytic anemia, unspecified
SNOMEDCT:
61261009 – Hemolytic Anemia
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Lymphoma / leukemia
- Myeloproliferative disease
- Paroxysmal nocturnal hemoglobinuria
- Lead poisoning
- Porphyria (eg, porphyria cutanea tarda)
- Anemia of chronic disease
- Aplastic anemia
- Iron deficiency anemia
- Megaloblastic anemia (vitamin B12 deficiency, folate deficiency)
- Myelodysplastic syndrome
- Biliary obstruction (cirrhosis, cholangitis, hepatobiliary malignancy)
- HELLP syndrome
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:08/13/2019
Last Updated:02/23/2022
Last Updated:02/23/2022