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

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Cao, X; Medeiros, LJ; Xia, Y; Wang, X; Thomas, SK; Loghavi, S; Li, X; Shah, JJ; Gustafson, SA; Weber, DM; Miranda, RN; Xu-Monette, ZY; Orlowski, RZ; Young, KH

Published Date

  • May 2016

Published In

Volume / Issue

  • 57 / 5

Start / End Page

  • 1104 - 1113

PubMed ID

  • 26421453

Pubmed Central ID

  • 26421453

Electronic International Standard Serial Number (EISSN)

  • 1029-2403

International Standard Serial Number (ISSN)

  • 1042-8194

Digital Object Identifier (DOI)

  • 10.3109/10428194.2015.1096357

Language

  • eng