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.
Original language | English (US) |
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Pages (from-to) | 1104-1113 |
Number of pages | 10 |
Journal | Leukemia and Lymphoma |
Volume | 57 |
Issue number | 5 |
DOIs | |
State | Published - May 3 2016 |
Keywords
- IgA
- IgG
- Lymphoplasmacytic lymphoma
- Waldenström macroglobulinemia
- non-IgM
- prognosis
- treatment
ASJC Scopus subject areas
- Hematology
- Oncology
- Cancer Research