Presents with acute or subacute development of fatigue or jaundice, and may include orthostasis and mild splenomegaly.
Anemia, reticulocytosis, low haptoglobin, high LDH, and high indirect bilirubin suggest hemolysis.
Direct antiglobulin test (Coombs) is important for differentiating immune from nonimmune etiologies. Peripheral smear review is important in identifying underlying cause.
Corticosteroids are often first-line therapy in immune-mediated cases. Discontinuation of offending agents (e.g., suspected drugs) and supportive care are the mainstay of treatment for many subtypes.
Early vaccination against encapsulated organisms is important if splenectomy is possible.
Hemolytic anemia encompasses a number of conditions that result in the premature destruction of RBCs. Common causes include autoantibodies, medications, and underlying malignancy, but the condition can also result from a number of hereditary conditions, such as hemoglobinopathies.
History and exam
- autoimmune disorders
- lymphoproliferative disorders
- prosthetic heart valve
- family origin in Mediterranean, Middle East, Sub-Saharan Africa, or Southeast Asia
- family history of hemoglobinopathy or RBC membrane defects
- paroxysmal nocturnal hemoglobinuria
- recent exposure to cephalosporins, penicillins, quinine derivatives, or NSAIDs
- recent exposure to naphthalene or fava beans
- thermal injury
- exceptional exertion
- recent exposure to nitrites, dapsone, ribavirin, or phenazopyridine
- recent paraquat ingestion
- Clostridium perfringens infection
- Haemophilus influenzae type B infection
- liver disease
Associate Professor of Clinical Medicine
Department of Medicine
University of Virginia
JD declares that he has no competing interests.
Dr John Densmore would like to gratefully acknowledge Dr Michelle Loch, a previous contributor to this monograph. ML declares that she has no competing interests.
PN declares that he has no competing interests.
Hematologic Oncology and Blood Disorders
Cleveland Clinic Lerner College of Medicine
AL declares that he has no competing interests.
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