Treatment outcomes of IgG4-producing marginal zone B-cell lymphoma: a retrospective case series
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ORIGINAL ARTICLE
Treatment outcomes of IgG4‑producing marginal zone B‑cell lymphoma: a retrospective case series Yuichi Sumii1 · Noboru Asada1 · Yasuharu Sato2,8 · Koh‑ichi Ohshima3 · Masanori Makita4 · Yusuke Yoshimoto5 · Yuka Sogabe6 · Kenji Imajo4 · Yusuke Meguri1 · Daisuke Ennishi1 · Hisakazu Nishimori1 · Nobuharu Fujii7 · Ken‑ichi Matsuoka1 · Tadashi Yoshino8 · Yoshinobu Maeda1 Received: 22 May 2020 / Revised: 4 August 2020 / Accepted: 7 August 2020 © Japanese Society of Hematology 2020
Abstract IgG4-producing marginal zone B-cell lymphomas (MZLs) have been recently proposed as a subtype of MZLs. Despite the abundant literature on pathophysiological features of this type of lymphoma, only a few retrospective studies pertaining to the treatment outcomes have been reported, and its prognosis remains unclear. We retrospectively analyzed seven patients with IgG4-producing MZLs diagnosed at our institute, with specific reference to treatment and outcomes. The median age was 69.0 years (55–79), and all were males. The median follow-up period was 66.6 months (8–121). All patients had localized disease; four patients had tumors of the ocular adnexa, whereas two had retroperitoneal tumors. Five patients were treated with irradiation (30 Gy/15 fr) (n = 4) or surgery (n = 1), resulting in tumor reduction. Two patients were treated by chemotherapy or irradiation. Among them, one commenced rituximab monotherapy, which led to an inadequate reduction of the tumor. Subsequent irradiation induced complete response (CR). The other patient experienced repeated relapses during follow-up and finally achieved CR by combination chemotherapy. Treatment was well tolerated in all cases, and none of the patients showed disease progression at the last follow-up visit. Our results indicate that the standard treatments for MZLs are generally appropriate for IgG4-producing MZL. Keywords IgG4 · Extranodal marginal zone B-cell lymphoma · IgG4-related disease
* Noboru Asada nasada@okayama‑u.ac.jp 1
Department of Hematology and Oncology, Okayama University Hospital, 2‑5‑1, Shikata‑cho Kita‑ku, Okayama 700‑8558, Japan
2
Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, Japan
3
Department of Ophthalmology, National Hospital Organization Okayama Medical Center, Okayama, Japan
4
Department of Hematology/Oncology, Okayama City Hospital, Okayama, Japan
5
Department of Neurosurgery, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan
6
Department of Ophthalmology, Mitoyo General Hospital, Kagawa, Japan
7
Department of Transfusion Medicine, Okayama University Hospital, Okayama, Japan
8
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
Introduction Marginal zone B-cell lymphoma (MZL) is characterized by lymphoma cells derived from the post-germinal center memory B-cells, residing in the marginal zones of the reactive B-cell follicles and extending into the interfollicular region as well
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