Scleroderma and hemolytic anemia in a patient with deficiency of IgA and C4: a hitherto undescribed association.
We describe a 66-year-old women with CREST variant of scleroderma who developed autoimmune hemolytic anemia responsive to prednisone and demonstrated a deficiency of IgA and the C4 component of complement. This association of clinical, immunologic and genetic findings has not previously been reported. The literature relating to these findings is reviewed and possible mechanisms are discussed.
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D003181:Complement C4 / Q000172:deficiency*; D004406:Dysgammaglobulinemia / Q000150:complications*; D005260:Female; D006801:Humans; D017098:IgA Deficiency; D012595:Scleroderma, Systemic / Q000150:complications*; D013577:Syndrome
D003181:Complement C4