Hemoglobin-specific antibody in a multiply transfused patient with sickle
cell disease
PA Noronha, LN Vida, CL Park and GR Honig
Department of Pediatrics, University of Illinois College of Medicine,
Chicago 60612, USA.
Human hemoglobins (Hbs) are known to be immunogenic, and both normal and
variant forms of Hb have been shown to stimulate antibody formation in a
variety of animal species. In patients who are homozygous for the sickle Hb
(HbS) mutation, transfusion of normal, HbA-containing erythrocytes provides
a potential stimulus for HbA alloimmunization. We tested serum samples for
the presence of anti-Hb antibody by a solid- phase enzyme-linked
immunosorbent assay (ELISA) using Hb-coated polystyrene microtiter plates.
Hb-bound antibody was identified using an antihuman IgG antibody. Serum
samples from 89 patients with sickle cell disease were initially tested for
evidence of Hb antibody. The serum from three individuals exhibited
antibody activity against HbA with little or no activity against HbS. Only
one of them, a multiply transfused adult with HbSS, was available for
further study. When this patient's antibody was tested against a variety of
normal and mutant Hbs using antibody either to human IgG or to kappa
chains, the anti-Hb antibody demonstrated specificity for the region of the
Hb beta chain corresponding to the site of the amino acid substitution of
HbS. The level of activity of the patient's anti-HbA showed no significant
change over 1.5 years of observation. The transfusion of erythrocytes
containing Hb structurally different from that of the recipient appeared to
be capable of stimulating the production of Hb-specific alloimmune
antibody.
Volume 89,
Issue 6,
pp. 2155-2158,
03/15/1997
Copyright © 1997 by The American Society of Hematology