The Clinical Study Of Lymphoplasmacytic Lymphoma/Waldenstr(?)m Macroglobulinemia

Objective:To investigate the clinical manifestation, therapy and response of the lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (LPL/WM), and to find the factors that influence response rate.Method:The clinical data of 12 patients firstly diagnosed as LPL/WM were reviewed, including 1 typical case. The factors influencing response were analyzed statistical methods.Results:12 patients including 6 male and 6 female were investigated, with a median age of 61 years. The clinical manifestations included:fatigue, bleeding, neuropathy, psychiatric symptoms, hyperviscosity syndrome and so on. The mono-clone cells of LPL/WM infiltrated other organs, with 12 cases of bone marrow infiltration,5 of splenomegaly,4 of lymphadenectasis,1 of hepatomegaly,1 of pulmonary infiltration. Complete blood count revealed anemia in 11 patients and thrombocytopenia in 3 ones. Bone marrow examination showed abnormal lymphoid plasma cells infiltration, and abnormal small B cells in biopsy and immunophenotype.5 cases were detected immunoglobulin heavy chain (IgH) gene rearrangement, and 1 of T cell receptor (TCR) gene rearrangement. In 12 patients,1 patient did not adopt any of the therapies after diagnosed, other 11 case were treated. And 5 cases received partial response,5 of stable disease,1 of progressive disease. Overall response rate was 45.5%.2 cases with treatment of alkylating agents all received partial response, and 2 cases with rituximab received partial response.6 cases received combination chemotherapy based on alkylating agents,1 of partial response,4 of stable disease, and 1 of progressive disease. 2 cases received combination chemotherapy based on rituximab,1 of partial response, and 1 of stable disease. Serum monoclonal immunoglobulin levels decreased after therapies. Among 10 cases of IgM type, obviously, the levels decreased 50.5%(P＜0.01). The serum free light chain levels reduced. Among 7 cases ofκlight chain type, apparently, the levels reduced 41.1%(P<0.01). There is no obvious difference between the levels of urine free light chain before and after therapy. The response rate of therapy in those with hemoglobin>100g/L and<2 extra nodal lesions'patients were significantly higher than those with hemoglobin<100g/L and>2 extra nodal lesions (P<0.05). All patients were followed-up for ranging for 2-63 months, with a median of 27 months. All the patients were survived till now.Conclusion:With variations of clinical manifestations, LPL/WM is smouldering with a long duration free from progression. Treatment for LPL/WM always includes alkylating agent, rituximab, and combination chemotherapy that bases on the two agents above. Hemoglobin level and numbers of extra nodal lesions are associated with the response rate.