Autoimmune hemolytic anemia with both cold and warm autoantibodies
I. A. Shulman, D. R. Branch, J. M. Nelson, J. C. Thompson, S. Saxena and L. D. Petz
Of 144 patients with a positive direct antiglobulin test and having
autoimmune hemolytic anemia (AIHA), 12 (8.3%) satisfied diagnostic criteria
for both warm antibody AIHA and cold agglutinin syndrome. All 12 patients
had IgG and C3d sensitizing their erythrocytes, and samples of their serum
contained IgM cold autohemagglutinins optimally reactive at 4 degrees C,
but with a high thermal amplitude to 37 degrees C, and IgG warm
autoantibodies. All red blood cell eluates contained IgG warm
autoantibodies. The 12 patients had severe hemolytic anemia that responded
dramatically to corticosteroid therapy, with the mean hemoglobin level
increasing from 6.3 to 12.9 g/dL. Five patients (42%) had systemic lupus
erythematosus, one patient (8%) had a non-Hodgkin's lymphoma, and six
patients (50%) had idiopathic AIHA; four patients (33%) had concomitant
thrombocytopenia (Evans' syndrome). Nine patients (75%) were female. Four
patients had unexpected alloantibodies potentially capable of in vivo
hemolysis of transfused blood. Because of the severe hemolysis, the
serologic findings, and the dramatic initial clinical response to
corticosteroid therapy, these patients represent a distinct catagory of
AIHA and should be given corticosteroid therapy quickly.
