Multiple mucosa-associated lymphoid tissue organs involving marginal zone B cell lymphoma: Organ-specific relationships and the prognostic factors. Consortium for improving survival of lymphoma study

Sung Yong Oh, Won Seog Kim, Jin Seok Kim, Seok Jin Kim, Suee Lee, Dae Ho Lee, Jong Ho Won, In Gyu Hwang, Min Kyoung Kim, Soon Il Lee, Yee Soo Chae, Deok Hwan Yang, Hye Jin Kang, Chul Won Choi, Jinny Park, Hyo Jung Kim, Jung Hye Kwon, Ho Sup Lee, Gyeong Won Lee, Hyeon Seok EomJae Yong Kwak, Won Sik Lee, Cheolwon Suh, Hyo Jin Kim

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Abstract

According to a previous review, multiple mucosa-associated lymphoid tissue (MALT)-organs involving marginal zone B cell lymphomas (MZLs) are present in 10-30% of patients. However, the clinical features and specific relationships among involved organs are yet to be clearly identified. In this study, we conducted retrospective analyses of multiple MALT organs involving MZLs (MM-MZLs) to identify their clinical features, treatment, prognosis, and specific relationships among involved organs. For analysis, between June 1987 and June 2009, a total of 55 patients from 17 different institutions in Korea, all of whom were histologically diagnosed with MM-MZL, were included in this study. MM-MZL was defined as MZL involving more than 2 different MALT organs. Multiple involvements within one MALT organ (e.g. both side ocular lesions, multiple lung nodules, and multiple stomach lesions, etc.) were excluded from this study. The male/female ratio of the 55 patients was 41/14. The median age of our subjects was 59 years (range 30-82 years). MM-MZL without lymph node (LN) was detected only in 9 patients (36.2%). Bone marrow (BM) involvement was observed in 17 patients (30.9%). The most common site of involvement was the gastrointestinal (GI) tract (25 patients, 45.5%) followed by the lung (40%), Waldeyer's ring (WR) (27.3%), and ocular area (25.5%). Ocular MZLs were commonly accompanied with WR- or lung-MZLs. GI-MZLs were WR or GI-MZLs. Lung-MZLs were frequently observed with ocular and GI-MZLs. WR-MZLs were ocular or GI-MZLs. A total of 53 patients were treated, and 2 on watchful wait. As much as 48 patients received chemotherapy-based treatment. Among them, CR or PR was achieved in 38 patients (79.2%, 95% CI 67-91%). Median time to progression (TTP) was 2.3 years (95% CI 1.4-3.2 years). Cause-specific overall survival (OS) did not reach the median value. The 5-year OS rate was 84.9%. MM-MZLs tend to be an indolent disease, characterized by prolonged survival with frequent relapses. The majority of cases could be controlled effectively via chemotherapy-based treatment, and prolonged survival was achieved in those patients. The GI, lung, WR, and ocular area were commonly presented with other MALT site MZLs, and an organ-specific relationship appears to be relevant to MM-MZLs.

Original languageEnglish
Pages (from-to)510-517
Number of pages8
JournalInternational Journal of Hematology
Volume92
Issue number3
DOIs
Publication statusPublished - 2010 Oct

Keywords

  • B cell lymphoma
  • Marginal zone
  • Mucosa-associated lymphoid tissues
  • Multiple

ASJC Scopus subject areas

  • Hematology

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    Oh, S. Y., Kim, W. S., Kim, J. S., Kim, S. J., Lee, S., Lee, D. H., Won, J. H., Hwang, I. G., Kim, M. K., Lee, S. I., Chae, Y. S., Yang, D. H., Kang, H. J., Choi, C. W., Park, J., Kim, H. J., Kwon, J. H., Lee, H. S., Lee, G. W., ... Kim, H. J. (2010). Multiple mucosa-associated lymphoid tissue organs involving marginal zone B cell lymphoma: Organ-specific relationships and the prognostic factors. Consortium for improving survival of lymphoma study. International Journal of Hematology, 92(3), 510-517. https://doi.org/10.1007/s12185-010-0680-z