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Short survival and frequent transformation in extranodal marginal zone lymphoma with multiple mucosal sites presentation
Author(s) -
Alderuccio Juan Pablo,
Zhao Wei,
Desai Amrita,
Ramdial Jeremy,
Gallastegui Nicolas,
Kimble Erik,
Fuente Macarena I.,
Husnain Muhammad,
Rosenblatt Joseph D.,
Alencar Alvaro J.,
Schatz Jonathan H.,
Moskowitz Craig H.,
Chapman Jennifer R.,
Vega Francisco,
Reis Isildinha M.,
Lossos Izidore S.
Publication year - 2019
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.25446
Subject(s) - medicine , international prognostic index , gastroenterology , hazard ratio , follicular lymphoma , lymphoma , proportional hazards model , b symptoms , stage (stratigraphy) , malt lymphoma , pathology , diffuse large b cell lymphoma , confidence interval , paleontology , biology
Abstract Between 11 and 37% of extranodal marginal zone lymphoma (EMZL) patients present with disease involvement in multiple mucosal sites (MMS). We analyzed 405 EMZL patients seen between 1995 and 2017: 265 (65.4%) patients presented with stage I disease, 49 of 309 (15.8%) patients with bone marrow involvement, and 35 of 328 (10.7%) patients with monoclonal gammopathy (MG). Forty‐three (10.6%) patients had MMS presentation, which was more frequently seen in patients aged >60 years (55.8%). Five (17.9%) of 28 MMS patients had MG. MMS patients commonly exhibited the International Prognostic Index (IPI) >2 (79.1%), Follicular Lymphoma International Prognostic Index (FLIPI) >2 (39.5%), and Mucosa‐Associated Lymphoid Tissue Lymphoma International Prognostic Index (MALT‐IPI) 2‐3 (60.5%). Both MMS presentation and MG were associated with shorter survival univariately. In multivariable Cox regression models, shorter progression‐free survival (PFS) and overall survival (OS) were observed in patients with MMS (hazard ratio [HR] = 3.08 and 2.92, respectively), age ≥60 years (HR = 1.52 and 2.45, respectively), and in patients who failed to attain a complete remission following initial therapy (HR = 3.27 and 2.13, respectively). Elevated lactate dehydrogenase was associated with shorter PFS (HR = 1.92), while anemia (HR = 2.46) was associated with shortened OS. MALT‐IPI ≥2 (HR = 2.47 and 4.75), FLIPI >2 (HR = 1.65 and 2.09), and IPI >2 (HR = 2.09 and 1.73) were associated with shorter PFS and OS, respectively. Higher grade transformation (HGT) occurred in 11 (25.6%) MMS patients with a 5‐year cumulative incidence of 13.2% (95% CI 4.7‐26.1%). EMZL patients with MMS presentation represent a novel clinical subset associated with shorter PFS, OS, and higher incidence of HGT that needs novel therapeutic approaches.