非IgM型淋巴浆细胞淋巴瘤临床及生物学特征

OBJECTIVE: The study aims to explore the clinical and biological characteristics of patients with non-IgM lymphoplasmacytic lymphoma（LPL）. METHODS: The clinical data of 340 patients with LPL admitted to the Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College were collected retrospectively, including 23 cases of the non-IgM LPL and 317 cases of the Waldenström's macroglobulinemia（WM）, from July 1993 to August 2020. The clinical and biological characteristics of the two groups were compared. RESULTS: Among 23 patients with the non-IgM type LPL, two patients secreted monoclonal IgA, 14 patients secreted monoclonal IgG, and seven patients did not secrete monoclonal immunoglobulin. The median age of the non-IgM LPL and WM were both 62（35−81）years old. Compared with the WM group, the proportion of women（56.5％ vs 27.3％, P＝0.007）, the proportion of splenomegaly（60.1％ vs 43.8％, P＝0.100）, and the proportion of extranodal invasion（21.7％ vs 12.3％, P＝0.672）in non-IgM LPL group were higher. Eighteen patients were tested for MYD88 gene mutation, and the overall mutation rate of MYD88 was 55.6％. In the non-IgM LPL group, a total of 17 patients received treatment, which had a comparable proportion（94.4％ vs 92.7％, P＝0.488）to the WM group. Sixteen patients were evaluated for efficacy, and the overall remission rate of the first-line treatment was 87.5％. The median follow-up time was 33.9（3.5–125.1）months, and the median PFS and OS were both not reached. The 3-year PFS and OS rates were 71.4％ and 68.9％, respectively. In the WM group, the median PFS was 66.2 months and the median OS was 78.1 months. Compared with the WM group, in the non-IgM group no significant differences in PFS（P＝0.340）and OS（P＝0.544）were seen. CONCLUSION: The clinical and biological characteristics of the non-IgM LPL and WM patients were similar. However, the proportion of women and extranodal involvement were higher in the non-IgM LPL group. The survival and prognosis of the non-IgM LPL patients were similar to those of the WM patients.