• Xupai Zhang, Shihui Ren, Nan Zhang, Xiao Wang, Lin Qiu, Haoping Sun, Hai Yi, and Fangyi Fan.
• Department of Hematology, The General Hospital of Western Theater Command, Chengdu, China.
• Medicine (Baltimore). 2024 Feb 9; 103 (6): e37158e37158.

RationaleSplenic marginal zone lymphoma (SMZL), an indolent small B-cell lymphoma, is uncommon, and part of the patients exist plasmocytic differentiation and secrete monoclonal paraproteins including IgM predominantly. SMZL with monoclonal IgG is rarer.Patient ConcernsWe report a case of SMZL (49-year-old, male) with monoclonal IgG, MYD88L265P mutation and hepatitis B virus infection.DiagnosesThe patient was presented to our hospital with aggravating complaints of dizziness, fatigue, postprandial abdominal distension, and night sweats. The diagnosis was confirmed by clinical manifestations, immunophenotype, bone marrow pathology.InterventionsThe patient received rituximab-based chemotherapy and sequential ibrutinib in combination with entecavir.OutcomesAfter 1 year of follow-up, his blood routine examination had returned to normal with normal level of albumin and significantly lower globulin than before, and the spleen was of normal size.LessonsWe conclude that rituximab-based chemotherapy is the main treatment option for the patients with SMZL, and Bruton's tyrosine kinase inhibitor has also shown beneficial efficacy.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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