Clinicopathologic features and outcomes of lymphoplasmacytic lymphoma patients with monoclonal IgG or IgA paraprotein expression

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.

Keywords:

• Lymphoplasmacytic lymphoma
• Waldenström macroglobulinemia
• non-IgM
• prognosis
• treatment
• IgA
• IgG

Acknowledgments

This work was supported by The University of Texas MD Anderson Cancer Center Lymphoma Moonshot Program, Institutional Research Grant Award, an MD Anderson Lymphoma Specialized Programs of Research Excellence (SPORE) Research Developmental Program Award, an MD Anderson Myeloma SPORE Research Developmental Program Award and the National Cancer Institute and the National Institutes of Health (R01CA138688 and R01CA187415 to KHY). X.C. is the recipient of the Hematology/Oncology Scholarship Award. This work was also partially supported by grants from the National Cancer Institute and National Institutes of Health (P50CA136411 and P50CA142509 to R.Z.O) and by the MD Anderson Cancer Center Support Grant (CA016672). KHY also receives research support from Roche Molecular System, Gilead Sciences Pharmaceutical, Seattle Genetics, Dai Sanyo Pharmaceutical, Adaptive Biotechnology and HTG Molecular Diagnostics.

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article at http://dx.doi.org/10.3109/10428194.2015.1096357