Santos Tayse Silva Dos, Tavares Renato Sampaio, Farias Danielle Leão Cordeiro de
Hospital das Clínicas, Universidade Federal de Goiás (UFG), Goiânia, GO, Brazil.
Hospital das Clínicas, Universidade Federal de Goiás (UFG), Goiânia, GO, Brazil.
Rev Bras Hematol Hemoter. 2017 Apr-Jun;39(2):146-154. doi: 10.1016/j.bjhh.2016.09.014. Epub 2016 Dec 22.
Splenic marginal zone lymphoma (SMZL) is a low-grade B-cell non-Hodgkin's lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes and indolent clinical course. As a rare disease, with no randomized prospective trials, there is no standard of care for SMZL so far. Splenectomy has been done for many years as an attempt to control disease, but nowadays it has not been encouraged as first line because of new advances in therapy as rituximab, that are as effective with minimal toxicity. Facing these controversies, this review highlights advances in the literature regarding diagnosis, prognostic factors, treatment indications and therapeutic options.
脾边缘区淋巴瘤（SMZL）是一种低度恶性B细胞非霍奇金淋巴瘤，其特征为脾肿大、淋巴细胞中度增多（伴有或不伴有绒毛状淋巴细胞）、外周淋巴结受累罕见以及临床病程进展缓慢。作为一种罕见疾病，由于缺乏随机前瞻性试验，目前尚无SMZL的标准治疗方案。脾切除术多年来一直作为控制疾病的一种尝试，但如今由于利妥昔单抗等治疗方法的新进展，因其疗效显著且毒性极小，脾切除术已不再被鼓励作为一线治疗方法。面对这些争议，本综述重点介绍了文献中关于诊断、预后因素、治疗指征和治疗选择的进展。
