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在摩洛哥中心治疗的套细胞淋巴瘤的流行病学、临床和治疗特征:14 例病例回顾。

Epidemiological, clinical and therapeutic profiles of mantle cell lymphoma cared for in a Moroccan center: a review of 14 cases.

机构信息

Department of Clinical Hematology and Bone Marrow Transplantation, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakesh, Morocco.

Clinical Research Unit, Mohammed VI University Hospital, Marrakesh, Morocco.

出版信息

Pan Afr Med J. 2024 Mar 6;47:111. doi: 10.11604/pamj.2024.47.111.40405. eCollection 2024.

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 1 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%。我们鉴定了 14 例套细胞淋巴瘤患者,每年平均有 3.5 例新发病例。观察到男性居多,男女比例为 6。我们患者的平均年龄为 64.4±14.1 岁,平均诊断延迟为 6.57 个月。关于临床表现,淋巴结病是最常见的体征(78.6%),其次是 B 症状(57.1%)。在我们的系列中,弥漫性分期最为常见:IV 期(78.5%)和 III 期(7.1%)与 I 期(0%)和 II 期(7.1%)相比。观察到的非淋巴结局部定位为肝 5 例(31.1%)、肺 04 例(25%)、髓质 4 例(25%)、胸膜 2 例(12.5%)和前列腺 1 例(6.2%)。所有诊断病例均为套细胞淋巴瘤,其中 12 例(85.7%)为经典型,2 例(14.3%)为惰性型。根据国际预后指数(MIPI)MCL 预后评分,高危组在我们的系列中最为常见: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,3 例接受 R-CHOP,1 例接受 DHAOX,1 例接受 R-CVP。二线治疗:2 例患者接受 R-DHAP 治疗,1 例患者在接受 R-CHOP 治疗后,另 1 例患者在接受 R-CVP 治疗后。2 例患者在治疗结束后接受了自体造血干细胞移植。7 例患者死亡,3 例患者失访,4 例患者仍在随访中,导致病情进展。此外,该研究强调了套细胞淋巴瘤的特征和治疗模式,突出了其在男性中的优势、诊断延迟、频繁扩散以及高危分类,以化疗作为主要治疗方式,预后具有挑战性,有助于全面了解套细胞淋巴瘤的表现和管理。

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