Clinicopathologic Features and Outcomes of Lymphoplasmacytic Lymphoma Patients With Monoclonal IgG or IgA Paraprotein Expression

Authors

Xin Cao, The University of Texas MD Anderson Cancer Center
L. Jeffrey Medeiros, The University of Texas MD Anderson Cancer Center
Yi Xia, The University of Texas MD Anderson Cancer Center
Xiaoxiao Wang, The University of Texas MD Anderson Cancer Center
Sheeba K. Thomas, The University of Texas MD Anderson Cancer Center
Sanam Loghavi, The University of Texas MD Anderson Cancer Center
Xin Li, The University of Texas MD Anderson Cancer Center
Jatin J. Shah, The University of Texas MD Anderson Cancer Center
Steven A. Gustafson, Kansas City University
Donna M. Weber, The University of Texas MD Anderson Cancer Center
Roberto N. Miranda, The University of Texas MD Anderson Cancer CenterFollow
Zijun Xu-Monette, The University of Texas MD Anderson Cancer Center
Robert Z. Orlowski, The University of Texas MD Anderson Cancer Center
Ken H. Young, The University of Texas MD Anderson Cancer Center

Document Type

Article

Publication Title

Leukemia & Lymphoma

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.

DOI

10.3109/10428194.2015.1096357

Publication Date

5-2016

Keywords

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

ISSN

1029-2403

Recommended Citation

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.