| Literature DB >> 26421453 |
Xin Cao1, L Jeffrey Medeiros1, Yi Xia1, Xiaoxiao Wang1, Sheeba K Thomas2, Sanam Loghavi1, Xin Li1, Jatin J Shah2, Steven A Gustafson3, Donna M Weber2, Roberto N Miranda1, Zijun Y Xu-Monette1, Robert Z Orlowski3, Ken H Young1,4.
Abstract
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.Entities:
Keywords: IgA; IgG; Lymphoplasmacytic lymphoma; Waldenström macroglobulinemia; non-IgM; prognosis; treatment
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Year: 2015 PMID： 26421453 DOI： 10.3109/10428194.2015.1096357
Source DB: PubMed Journal: Leuk Lymphoma ISSN： 1026-8022