Autoimmune hemolytic anemia

1. Autoimmune hemolytic anemia
Warm agglutinin AIHA Cold agglutinin AIHA
Etiology • Drugs (eg. penicillin)
• Viral infections
• Autoimmune (eg. SLE)
• Immunodeficiency states
• Lymphoproliferative (eg. CLL)
• Infections (eg. Mycoplasma
pneumoniae infection & infectious
mononucleosis)
• Lymphoproliferative diseases
Clinical
presentation
• Asymptomatic to life-
threatening anemia
• Direct Coombs’ positive with
anti-IgG, anti-C3, or both
• Symptoms of anemia
• Livedo reticularis & acral cyanosis
with cold exposure that disappear
with warming
• Direct Coombs’ positive with anti-
C3 or anti-IgM, but usually not IgG
Treatment • Corticosteroids
• Splenectomy for refractory
disease
• Avoidance of cold temperatures
• Rituximab +/- fludarabine
Complications • Venous thromboembolism
• Lymphoproliferative disorders
• Ischemia & peripheral gangrene
• Lymphoproliferative disorders