Fifty-seven consecutive patients with Waldenstrom's Macroglobuliemia were s
tudied retrospectively for autoimmune manifestations. 28 patients or 51% (1
6 women and 13 men) had clinical and/or serological autoimmune manifestatio
ns, two or more of these being concomitant in 20 (12 women and 8 men). The
predominant findings were Coombs' positive autoimmune hemolytic anemia (16%
), seropositive rheumatoid arthritis (16%), inflammatory gastric ulcer with
parietal cell autoantibodies (12%), and IgM-cardiolipin syndrome (11%). 40
% of the autoimmune manifestations were present at the time of diagnosis of
the Waldenstrom's Macroglobulinaemia and 60% were observed over a mean per
iod of 4.7 years. All patients had an IgM M-component. There was no correla
tion between autoimmunity and the size of the M-component or the degree of
hypo-IgG and hypo-IgA gammaglobulinemia. The only correlation between autoi
mmunity and infection was found in patients with gastric ulcer and parietal
cell autoantibodies, in whom the infection was caused by Helicobacter pylo
ri.