Clinicopathologic Features and Outcomes of Lymphoplasmacytic Lymphoma Patients With Monoclonal IgG or IgA Paraprotein Expression
Leukemia & Lymphoma
Lymphoplasmacytic lymphoma secreting IgG or IgA (non-IgM LPL) is rarely seen. Systematic studies of the clinical features and treatment outcomes are lacking in these patients. This study evaluated 17 patients with non-IgM LPL. The paraprotein secreted by these tumors was IgA (n=8; 47%) and IgG (n=9; 53%). The median serum level of paraprotein was 2,475 mg/dl (range=747-5260) for IgA and 2580 mg/dl (range=1900-7100) for IgG. The IgA-LPL group was more likely to present with B symptoms, a high beta2-microglobulin level and extramedullary involvement. Compared with patients with Waldenström macroglobulinemia (WM), patients with non-IgM LPL showed similar clinical and pathologic features, but a higher mortality within the first year after diagnosis (p<0.001) and worse overall survival (p=0.024), with no difference in progression-free survival and disease-specific survival. Rituximab alone or rituximab-based therapy was used frequently and was effective as either first-line or salvage therapy.
IgA, IgG, Lymphoplasmacytic lymphoma, Waldenström macroglobulinemia, non-IgM, prognosis, treatment
Cao X, Medeiros L, Xia Y, Wang X, Thomas SK, Loghavi S, Li X, Shah JJ, Gustafson SA, Weber DM, Miranda RN, Xu-Monette Z, Orlowski RZ, Young KH. Clinicopathologic Features and Outcomes of Lymphoplasmacytic Lymphoma Patients With Monoclonal IgG or IgA Paraprotein Expression. Leukemia & Lymphoma. 2016; 57(5). doi: 10.3109/10428194.2015.1096357.