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Classification of autoimmune hemolytic anemia

Warm-reactive AIHA

Primary (idiopathic)*

Secondary^¶

Autoimmune or inflammatory disorders (eg, systemic lupus erythematosus, Sjögren syndrome, scleroderma, juvenile idiopathic arthritis, dermatomyositis, vitiligo, ulcerative colitis, autoimmune hepatitis, type 1 diabetes mellitus, autoimmune thyroiditis)
Evans syndrome
Primary immunodeficiency (eg, common variable immune deficiency, Wiskott-Aldrich syndrome)
Acquired immunodeficiency (eg, HIV)
Malignancy (eg, acute leukemia, lymphoma)
Infection^Δ
Posttransplant

Cold agglutinin disease

Primary (idiopathic)*

Secondary^¶

Infection (eg, Mycoplasma pneumoniae, EBV)^Δ
Malignancy (eg, lymphoma)

Paroxysmal cold hemoglobinuria

Primary (idiopathic)

Post-infectious^Δ

AIHA: autoimmune hemolytic anemia; EBV: Epstein-Barr virus.
* Primary AIHA refers to disorders with only immune-mediated hemolytic anemia and no evidence of an underlying systemic disorder.
¶ Secondary AIHA refers to disorders with immune-mediated hemolytic anemia in the presence of another condition. Common secondary causes are listed in this table. For further details, refer to UpToDate content on AIHA in children.
Δ Infectious and post-infectious causes of AIHA are most commonly associated with cold agglutinins disease and paroxysmal cold hemoglobinuria.

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