Università degli studi di Sassari, scienze mediche chirurgiche e sperimentali.
Medical Oncology and Hematology Unit, Humanitas Clinical and Research Center - IRCCS, Humanitas Cancer Center, Rozzano.
Anticancer Drugs. 2022 Jan 1;33(1):e36-e42. doi: 10.1097/CAD.0000000000001165.
Splenic marginal zone lymphoma (SMZL) is a rare lymphoproliferative disease involving B-cells and affecting elderly patients. SMZL plague peripheral blood and bone marrow, spleen. Lymph nodes are generally spared. SMZL is due to a protracted antigen stimulation of B lymphocytes and of microenvironment leading B-cell to polyclonal and then oligoclonal/monoclonal growth, promoting lymphoproliferation. Integration of the NOTCH2 and NFk-B signaling has been recently identified as the primary mechanism of neoplastic proliferation in SMZL. In total 20% of cases carry mutations in NOTCH2. Although SMZL has an indolent course, progression to diffuse large B-cell lymphoma occurs in about 10-15% of patients. Establishing the prognosis is a key step in disease management, depending on both individual risk and patients' health status. This review discusses tailored treatment of SMZL patients. Progression risk factors include nodal and extra-nodal involvement, peripheral lymphocytosis, anemia and thrombocytopenia. Patients with two or more score points have a median survival of <5 years. Watch and wait strategy is appropriate in low-risk and asymptomatic patients, whereas treatment of symptomatic patients ranges from splenectomy to rituximab monotherapy or associated with chemotherapy.
脾边缘区淋巴瘤（SMZL）是一种罕见的累及 B 细胞的淋巴增殖性疾病，影响老年患者。SMZL 累及外周血和骨髓、脾脏。淋巴结通常不受累。SMZL 是由于 B 淋巴细胞的持续抗原刺激和微环境导致 B 细胞的多克隆和寡克隆/单克隆生长，促进淋巴增殖。NOTCH2 和 NFkB 信号通路的整合最近被确定为 SMZL 中肿瘤增殖的主要机制。大约 20%的病例存在 NOTCH2 突变。尽管 SMZL 呈惰性病程，但约 10-15%的患者会进展为弥漫性大 B 细胞淋巴瘤。确定预后是疾病管理的关键步骤，这取决于个体风险和患者的健康状况。这篇综述讨论了针对 SMZL 患者的个体化治疗。进展的危险因素包括结外和结内累及、外周血淋巴细胞增多、贫血和血小板减少。有两个或更多评分点的患者中位生存期<5 年。对于低危和无症状患者，观察和等待策略是合适的，而有症状患者的治疗范围从脾切除术到利妥昔单抗单药治疗或与化疗联合治疗。
