title: Distinctive clinical and histological features of Waldenström's macroglobulinemia and splenic marginal zone lymphoma.
abstract: We studied 122 patients with Waldenström's macroglobulinemia (WM) and 98 with splenic marginal zone lymphoma (SMZL); 29 SMZL patients (30%) had a serum MC (IgM in 17 patients). SMZL differed from WM for female prevalence, abdominal and superficial adenopathy, spleen and liver involvement, positive HCV. The median MC level was 2.0 g/dL in WM and 0.95 g/dL in SMZL (P<.001). On BM histology, SMZL was characterized by sinusoidal infiltration (70% of cases) and by a more frequent nodular pattern (P<.01) while WM had a higher incidence of interstitial BM localization. After a median follow-up of 5.3 years, median OS was not reached for SMZL and was 12 years for WM (P=.23; 14 years for asymptomatic WM, 8 years for symptomatic WM). In conclusion, despite similar outcomes of these 2 entities, SMZL appears as a disease with distinct clinical features and BM histology and a peculiar association with HCV infection.
authors: Division of Hematology, Department of Oncohematology, University of Pavia Medical School, Fondazione IRCCS Policlinico San Matteo, Italy. luca.arcaini@unipv.it
authors: Luca
authors: L
authors: Arcaini
authors: Marzia
authors: M
authors: Varettoni
authors: Emanuela
authors: E
authors: Boveri
authors: Ester
authors: E
authors: Orlandi
authors: Sara
authors: S
authors: Rattotti
authors: Silvia
authors: S
authors: Zibellini
authors: Michele
authors: M
authors: Merli
authors: Marco
authors: M
authors: Lucioni
authors: Silvia
authors: S
authors: Rizzi
authors: Manuel
authors: M
authors: Gotti
authors: Lucia
authors: L
authors: Morello
authors: Cristiana
authors: C
authors: Pascutto
authors: Marco
authors: M
authors: Paulli
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000473:pathology*; D016019:Survival Analysis; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis / Q000473:pathology*