BACKGROUND: The prognostic importance of extramedullary involvement in @entity1 with @entity8057 ( @entity8057 ) at diagnosis and treatment options for these @entity1 has not been well evaluated. In this study, we investigated the clinical manifestations, biological features, and effect of first-line therapy on the outcome of @entity8057 @entity1 diagnosed with extramedullary involvement (EMWM) vs those with only bone marrow involvement (BMWM). METHODS: We analyzed the clinical data of 312 @entity8057 @entity1 diagnosed with EMWM (n   =   106) and BMWM (n   =   206) at The University of Texas @entity601 Anderson @entity5 Center from 1994 to 2014. EMWM was confirmed by biopsy, positron emission tomography-computed tomography, or magnetic resonance imaging, and clinical laboratory analyses. RESULTS: Characteristics associated with EMWM were male sex (P   =   0.027), age younger than 65  years (P   =   0.048), presence of B symptoms (P   <   0.001), high serum @entity2263 macroglobulin (P   <   0.001) level, low serum @entity2313 level (P   =   0.036), and @entity2008 (P   =   0.010). Kaplan-Meier survival analysis results showed that EMWM @entity1 had a significantly shorter median overall survival (P   <   0.001) and progression-free survival (PFS) (P   <   0.001) than did BMWM @entity1 . Chemotherapy combined with targeted therapy improved PFS for BMWM @entity1 (P   =   0.004) but not for EMWM @entity1 . Additionally, initial treatment with rituximab significantly improved the PFS of BMWM @entity1 (P   =   0.012) but had no effect on EMWM @entity1 . However, EMWM @entity1 treated with @entity8742 analogs attained a better PFS than those who did not (P   =   0.021). CONCLUSIONS: We show that extramedullary involvement at diagnosis is an adverse prognostic factor in @entity8057 @entity1 and that first-line therapy with @entity8742 analogs improved PFS for @entity1 with EMWM. The study provides unique clinical and treatment observations in subtypes of @entity8057 @entity1 .

XXXX with extramedullary involvement at initial diagnosis portends a poorer prognosis.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2313 :: ('213', 'Gene') :: ['albumin'] @entity2008 :: ('MESH:D002869', 'Disease') :: ['cytogenetic abnormalities'] @entity5 :: ('MESH:D009369', 'Disease') :: ['Cancer'] @entity8742 :: ('D009705', 'Chemical') :: ['nucleoside'] @entity601 :: ('MESH:D009436', 'Disease') :: ['MD'] @entity2263 :: ('10242', 'Gene') :: ['beta-2'] @entity8057 :: ('MESH:D008258', 'Disease') :: ['WM', 'Waldenstr  m macroglobulinemia']
@entity8057 :: (MESH:D008258,Disease) :: ['Waldenstr  m macroglobulinemia']
