Short survival and frequent transformation in extranodal marginal zone lymphoma with multiple mucosal sites presentation

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.

Trends of Incidence and Survival Rrates of Mucosa-associated Lymphoid Tissue Lymphoma in the Korean Population: Analysis of the Korea Central Cancer Registry Database

“…The other sites of MALT-lymphoma include the lung (14%), head and neck (14%), skin (11%), thyroid gland (4%), salivary gland (6%), and eye (12%). 27 34 A single center study in Korea estimated that gastric MALT-lymphoma accounts for 50% of all MALT-lymphoma cases. 35 The incidence of gastric MALT-lymphoma has been increasing in the last few decades and accounts for approximately 2%–8% of all gastric neoplasms.…”

Marginal zone lymphoma: present status and future perspectives

“…Single-agent rituximab (with short or protracted administration) has become the preferred approach in most patients, so that splenectomy or chemoimmunotherapy is reserved for patients not responding to single-agent treatment. 65 Recent single institution data showed that CD5 expression, although rare in MZL, was associated with a lower ORR following rituximab monotherapy but not bendamustine and rituximab, suggesting the latter combination might be preferred if systemic therapy is required. 66…”

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

“…EMZL is diagnosed at an early stage of the disease in 60% to 80% of cases and remains localized to mucosa environment for prolonged periods of time [12][13][14]. This unique biological characteristic makes local treatment, such as eradication of H. pylori infection from the stomach or radiation therapy (RT) in other locations, the preferred approach.…”