Waldenstrom macroglobulinemia - Development of diagnostic criteria and identification of prognostic factors

To establish whether a combination of morphologic and immunophenotypic crit eria could be developed to more precisely define Waldenstrom macroglobuline mia (WM) and prognostic factors, we retrospectively assessed the clinical a nd laboratory, features of 111 cases of WM. Bone marrow infiltration by sma ll lymphocytes was documented in each case; and diffuse, interstitial, nodu lar and paratrabecular patterns of infiltration were documented in 58%, 32% , 6%, and 4% of cases, respectively. Ninety percent were characterized by a surface immunoglobulin-positive, CDl9+CD20+CD5-CDI0-CD23- immunophenotype. The median overall survival from diagnosis was 60 months; univariate analy sis revealed the following adverse prognostic factors: older than 60 years, performance status more than 1. platelet count less than 100 x 10(3)/muL ( < 100 x 10(9)/L), pancytopenia, and diffuse bone marrow infiltration. Assoc iated median survival it-as 40, 38, 46, 28, and 59 months, respectively. Mu ltivariate analysis revealed age, performance status, and platelet count as prognostically significant, but stratification of patients according to th e International Prognostic Index had limited value. We suggest defining WM by the following criteria: IgM monoclonal gammopathy; bone marrow infiltrat ion by small lymphocytes, plasmacytoid cells, and plasma cells in a diffuse , interstitial, or nodular pattern; and a surface immunoglobulin-positive, CD19+CD20+CD5-CD10-CD23- immunophenotype.