Future development of lymphoproliferative disorders in patients with autoimmune hemolytic anemia

The association between autoimmune hemolytic anemia (AIHA) and subsequent a ppearance of lymphoproliferative disorders (LPDs) has not been properly add ressed in large-scale studies. We evaluated 107 patients with idiopathic (6 7 patients) or underlying (40 patients) immune disorders diagnosed with AIH A between 1992 and 1999. The following variables were examined in univariat e and multivariate analysis: age; sex; type of AIHA (warm- or cold-active a ntibodies); presence of underlying immune disorders; and serum monoclonal p rotein, Of the 107 patients, 19 (18%) developed malignant LPDs. The median time to develop malignancy was 26.5 months (range, 9-76 months), At multiva riate analysis, advanced age (P = 0.005), underlying autoimmune diseases (P = 0.002), and the presence of serum gammopathy (P = 0.045) were risk facto rs for future development of LPDs in these patients. Also, serum monoclonal IgM protein was a significant predictor (P = 0.0001) for the appearance of LPDs in patients with AIHA. The present study provides evidence that AIHA in some patients should be considered as a precursor of malignant LPDs, Kno wledge of certain characteristics may help identify patients at risk for th is transformation; periodic clinical and laboratory assessment of these pat ients is warranted.