A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test

Citation
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
Citations number
16
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
ANNALS OF HEMATOLOGY
ISSN journal
09395555 → ACNP
Volume
77
Issue
5
Year of publication
1998
Pages
231 - 234
Database
ISI
SICI code
0939-5555(199811)77:5<231:ACOMHD>2.0.ZU;2-#
Abstract
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.