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Nodal marginal zone B-cell lymphoma: analysis of 36 cases. Clinical presentation and treatment outcomes of nodal marginal zone B-cell lymphoma
Authors:Sung Yong Oh  Baek-Yeol Ryoo  Won Seog Kim  Kihyun Kim  Jeeyun Lee  Hyun Jung Kim  Jung Mi Kwon  Hyo Rak Lee  Young Hye Ko  Suk Joong Oh  Keon Woo Park  Hyo-Jin Kim  Hyuk-Chan Kwon  Eunmi Nam  Jung Han Kim  Yeon Hee Park  Seung-Sook Lee  Ho Young Kim  Keunchil Park
Affiliation:(1) Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Kangnam-gu, Seoul, 135-710, South Korea;(2) Department of Pathology, Samsung Medical Center, Seoul, South Korea;(3) Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, South Korea;(4) Department of Internal Medicine, Dankook University College of Medicine, Cheonan, South Korea;(5) Department of Internal Medicine, Dong-A University College of Medicine, Busan, South Korea;(6) Department of Internal Medicine, College of Medicine, Inje University, Sanggye Park Hospital, Seoul, Korea;(7) Department of Internal Medicine, Gachon Medical School, Incheon, South Korea;(8) Department of Internal Medicine, Hallym University College of Medicine, Chuncheon, Gangwondo, South Korea;(9) Department of Hematology and Oncology, Korea Institute of Radiological & Medical Sciences, Seoul, South Korea;(10) Department of Pathology, Korea Institute of Radiological & Medical Sciences, Seoul, South Korea;(11) Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Gangwondo, South Korea
Abstract:Nodal marginal zone B-cell lymphoma (NMZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and the optimal treatment modality have not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of NMZL. Thirty-six patients who were histologically diagnosed as NMZL were included in the analysis. Fifty-three percent of the patients had localized disease (stages I and II), and 21.2% (7/33) had bone marrow involvement at presentation. B symptom was present in only three patients (8.3%). Most patients were categorized as low or low-intermediate risk group by international prognostic index (IPI) (77.1%). Majority (94.4%) of the patients with localized disease achieved complete remission (CR) after the initial treatment. Of the seven patients with disseminated disease, who were treated with anthracycline-based chemotherapy, four patients achieved CR. Of the seven patients who received nonanthracycline-based chemotherapy, no patient achieved CR. After the median follow-up duration of 36 months, the median progression-free survival (PFS) was 3.9 (95% CI; 2.9–5.6) years, and the estimated 5-year PFS and overall survival rates were 47.2 and 82.7%, respectively. The significant predictive factors for PFS were performance status, advanced stage, and follicular lymphoma IPI (FLIPI) in this study. This clinical feature is similar to FL rather than to MZL-MALT type.
Keywords:Nodal  Marginal zone B-cell lymphoma
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