Autoimmune hemolytic anemia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2]

Classification

Category	Features	Subtypes
Warm-antibody type	Accounts for ~75% of cases of autoimmune hemolytic anemia Due to IgG autoantibody Binding occurs optimally at 37 degrees Celsius Extravascular hemolysis (reticuloendothelial system)	Idiopathic Systemic lupus erythematosus Evans' syndrome Chronic lymphocytic leukemia Non-Hodgkin lymphoma Drugs (methyldopa)
Cold-antibody type	Accounts for ~25% of cases of autoimmune hemolytic anemia Due to IgM autoantibody Binding occurs optimally at 4 degrees Celsius Intravascular hemolysis Recognition by the reticuloendothelial system Destruction by macrophages Associated with cold agglutinin disease Associated with paroxysmal cold hemoglobinuria Associated with Mycoplasma infection Associated with malignancy
Mixed warm-antibody and cold-antibody type	Due to IgM subtype Mixed features

Intravascular haemolysis
Red blood cell lysis occurs in the circulation as a result of activation of the complement system cascade.

Extravascular haemolysis
Red Blood Cells that are coated with antibodies are specifically recognised in the reticuloendothelial system and destroyed by macrophages.

Subtypes

• Warm antibody autoimmune hemolytic anemia

• Idiopathic
• Systemic lupus erythematosus
• Evans' syndrome (antiplatelet antibodies and haemolytic antibodies)
• Chronic lymphocytic leukemia
• Drugs (methyldopa)

• Cold antibody autoimmune hemolytic anemia

• Idiopathic cold hemagglutinin syndrome
• Infectious mononucleosis
• Paroxysmal cold hemoglobinuria (rare)
• Lymphoma

• Mixed-type autoimmune hemolytic anemia

References

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