The Clinical Features and Prognosis of Lymphoplasmacytic Lymphoma/ Waldenström Macroglobulinemia: A Large Series Analysis from a Single Center

Background: Lymphoplasmacytic Lymphoma/Waldenströmmacroglobulinemia (LPL/WM) is a rare B-cell proliferative malignancy characterized by immunoglobulin M monoclonal gammopathy and /or bone marrow infiltration by lymphocyte, lymphoplasmacytic and/or plasma cells. The clinical features and prognosis of WM/LPL have less been investigated in Chinese patients. Moreover, few studies had reported the prognostic value of cytogenetic aberrations in Chinese WM/LPL patients.

Methods: We systematically reviewed the records of 233 LPL/WM patients, who had been diagnosed at institute of Hematology&Blood Disease Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College.

Results: The median age of 233 patients was 62 years (range 32-87) and the male to female ratio was 2.76 (171/62). The most common symptoms at diagnosis were fatigue (59.1%) and bleeding (11.2%). The patients in low, intermediate and higher risk group of the international prognostic scoring system for WM (ISSWM) occupied 14.9%, 32.6% and 52.6%, respectively. 90.4% and 16% patients showed an elevated serum β2-microglobulinand LDH. The percentages of hepatomegaly, splenomegaly, peripheral lymphadenopathy, B symptom were 14.9%, 34.5%, 43% and 29.3%, respectively. The degree of splenomegaly was always mild or moderate, only 19.1% patients with giant spleen. The karyotypes abnormalities were not common in LPL/WM patients. Only 8.9% (14/157) patients had abnormal chromosome karyotypes. The most common cytogenetic aberration detected by fluorescence in situ hybridization (FISH) were deletion of 17q13 (9/120, 7.5%), trisomy 12(5/58, 8.6%). Other aberrations include 13q14 deletion in 3 of 128 patients (2.3%), 11q22 deletion in2 of 90 patients (2.2%). Univariate analysis showed that age>62 years, leukocytopenia, thrombocytopenia, anemia, cytopenias ≥ 2, elevated LDH, abnormal chromosome karyotypes, P53 deletion and high risk of ISSWM were adverse risk factors for OS. But only clinical effect not reaching partial response (p=0.006), elevated LDH (p=0.049) and leukocytopenia (p=0.048) were the independent prognostic factors at multivariate analysis.

Conclusion: Distinct characteristics exist in WM in China compared with the Western country. Elevated LDH and leukocytopenia were the independent adviser factors for OS in this series patient.