title: 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.
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Xin
authors.initials: X
authors.lastname: Cao
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Yi
authors.initials: Y
authors.lastname: Xia
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Xiaoxiao
authors.initials: X
authors.lastname: Wang
authors.affiliation: b Department of Lymphoma and Myeloma , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Sheeba K
authors.initials: SK
authors.lastname: Thomas
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Sanam
authors.initials: S
authors.lastname: Loghavi
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Xin
authors.initials: X
authors.lastname: Li
authors.affiliation: b Department of Lymphoma and Myeloma , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Jatin J
authors.initials: JJ
authors.lastname: Shah
authors.affiliation: c Department of Pathology , Kansas City University of Medicine and Biosciences , Kansas City , MO , USA ;
authors.forename: Steven A
authors.initials: SA
authors.lastname: Gustafson
authors.affiliation: b Department of Lymphoma and Myeloma , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Donna M
authors.initials: DM
authors.lastname: Weber
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;
authors.forename: Zijun Y
authors.initials: ZY
authors.lastname: Xu-Monette
authors.affiliation: c Department of Pathology , Kansas City University of Medicine and Biosciences , Kansas City , MO , USA ;
authors.forename: Robert Z
authors.initials: RZ
authors.lastname: Orlowski
authors.affiliation: a Department of Hematopathology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA ;|d The University of Texas School of Medicine, Graduate School of Biomedical Sciences , Houston , TX , USA.
authors.forename: Ken H
authors.initials: KH
authors.lastname: Young
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D001853:Bone Marrow / Q000473:pathology; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007074:Immunoglobulin G / Q000097:blood*; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000097:blood / Q000401:mortality; D010266:Paraproteins; D011379:Prognosis; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy
chemical_list: D015415:Biomarkers; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D010266:Paraproteins
keywords: IgA; IgG; Lymphoplasmacytic lymphoma; Waldenström macroglobulinemia; non-IgM; prognosis; treatment