M. Witzens et al., A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test, ANN HEMATOL, 77(5), 1998, pp. 231-234
We report here for the first time a patient with mu heavy-chain disease (HC
D), hyperimmunoglobulinemia, and a positive direct antiglobulin test (DAT,
Coombs test). The heavy-chain diseases involve the proliferation of lymphop
lasma cells of B cell origin and are characterized by the production of inc
omplete heavy chains devoid of light chains. The association of mu heavy-ch
ain disease with either hyperglobulinemia or a positive DAT has not been re
ported in the literature to date. In this patient, immunofixation of serum
proteins with monospecific antisera to alpha-, gamma-, mu,- or delta-chains
and to kappa- and lambda-chains revealed a precipitation band with antibod
y to IgM, but not with kappa and lambda light-chain antibodies, indicating
mu heavy-chain disease. Hyperglobulinemia was present, which is very uncomm
on for HCD. A DAT of the patient's red blood cells (RBC) was found to be st
rongly positive for anti-IgG but negative for anti-IgM, IgA, -C3c, and -C3d
. However, when the eluate from the patient's red blood cells was investiga
ted with nephelometry, it was found to contain antigens reactive with anti-
gamma as well with anti-mu-antiserum, When a DAT was performed with a rando
mly chosen test cell incubated with the eluate, the antibody-containing elu
ate was shown to react with anti-IgG as well as with anti-IgM-antiserum. In
summary, the eluate from the patient's RBCs contained IgG and an immunoglo
bulin structure reactive with anti-IgM in an RBC agglutination assay as wel
l as with anti-mu antiserum in a nephelometric investigation. Whether this
IgM on the patient's erythrocytes is penta- or oligomeric, complete IgM, or
the heavy chain cannot be concluded from these observations.