PDF(Pubmed)
Abstract：
Mantle cell lymphoma (MCL) accounts for 3-10% of non-Hodgkin\'s lymphomas (NHL). We identified 14 patients with mantle cell lymphoma, with an average number of 3.5 new cases/year. A male predominance was observed with a sex ratio equal to 6. The average age of our patients was 64.4±14.1 years, with an average diagnostic delay of 6.57 months. Regarding the clinical presentation, adenopathy was the most reported physical sign (78.6%) followed by B symptoms (57.1%). Disseminated stages were the most frequent in our series: stages IV (78.5%) and III (7.1%) versus stages I (0%) and II (7.1%). The extra-ganglionic localizations observed were hepatic 5 cases (31.1%), pulmonary 04 cases (25%), medullary 4 cases (25%), pleural 2 cases (12.5%) and prostate 1 case (6.2%). All diagnosed cases are mantle cell lymphomas, of which 12 cases (85.7%) are classical and 2 cases (14.3%) indolent. The high-risk group is, according to international prognostic index (MIPI) MCL prognostic score, the most represented in our series: 0-3 = 6 cases (42.9%), 6-11 = 8 cases (57.1%). The therapeutic protocol chosen 1st line: 9 patients treated with R-DHAP, three with R-CHOP, one with DHAOX and one with R-CVP. Second line: two patients treated with R-DHAP, one after R-CHOP and the other after R-CVP. Two patients received autologous hematopoietic stem cell transplant at the end of the treatment. The evolution was marked by the death of 7 patients, 3 lost to follow-up and 4 still followed. Additionally, the study highlights characteristics and treatment patterns of mantle cell lymphoma, emphasizing its predominance in males, delayed diagnosis, frequent dissemination, and high-risk classification, with chemotherapy as the primary treatment modality and a challenging prognosis contributing to a comprehensive understanding of mantle cell lymphoma presentation and management.
摘要：
套细胞淋巴瘤（MCL）占非霍奇金淋巴瘤（NHL）的3-10％。我们确定了14例套细胞淋巴瘤患者，平均每年新增3.5例。观察到男性占主导地位，性别比等于6。我们患者的平均年龄为64.4±14.1岁，平均诊断延迟6.57个月。关于临床表现，腺病是报告最多的体征（78.6％），其次是B症状（57.1％）。传播阶段是我们系列中最常见的阶段：阶段IV（78.5％）和阶段III（7.1％）与阶段I（0％）和阶段II（7.1％）。观察到的神经节外定位为肝5例（31.1％），肺04例（25%），髓质4例（25%），胸膜2例(12.5%),前列腺1例(6.2%)。所有确诊病例都是套细胞淋巴瘤,其中经典12例(85.7%)，惰性2例(14.3%)。高危人群是,根据国际预后指数（MIPI）MCL预后评分，在我们的系列中代表最多的是：0-3=6例(42.9%)，6-11=8例（57.1%）。选择一线治疗方案：9例患者接受R-DHAP治疗，三个用R-CHOP，一个用DHAOX，一个用R-CVP。第二行：两名接受R-DHAP治疗的患者，一个在R-CHOP之后，另一个在R-CVP之后。两名患者在治疗结束时接受了自体造血干细胞移植。演变以7名患者的死亡为标志，3失去了随访，4仍然跟随。此外,这项研究强调了套细胞淋巴瘤的特点和治疗模式，强调它在男性中的优势，延迟诊断,频繁传播,和高风险分类，化疗作为主要治疗方式和具有挑战性的预后有助于全面了解套细胞淋巴瘤的表现和管理。
