Clinico-Pathologic Characteristics, Patterns of Treatment and Outcome of Newly Diagnosed Waldenstroms Macroglobulinemia- A Single Center Real World Retrospective Analysis
摘要
Waldenström macroglobulinemia (WM) is a rare indolent neoplasm characterized by presence of ≥ 10% lymphoid cells in the BM exhibiting plasmacytoid or plasma-cell differentiation, with secretion of an IgM monoclonal protein. This is a retrospective analysis of 89 patients with WM that describes the clinical and laboratory characteristics, treatment patterns and outcome of patients with WM. The median age of the entire cohort was 66 years with male predominance (67.4%). Most common presentations were symptoms pertaining to anemia (77.5%) and constitutional symptoms (33.7%). Median bone marrow lymphoplasmacytic cells were 41%. Positivity for MYD88 and CXCR4 mutations was seen in 81.8% of 77 and 2.4% of 41 tested patients, respectively. BR was the most common regimen used (52.8%). The overall response rate was 87.8%. Median overall survival, progression-free survival and time to next treatment were 8.49, 2.15 and 3.88 years, respectively. BR was associated with the longest observed progression-free survival in this cohort.