Service d'Hématologie Adultes, AP-HP, Hôpital Necker, 149 rue de Sèvres, 75743, Paris Cedex 15, France.
Drugs Aging. 2013 Dec;30(12):979-86. doi: 10.1007/s40266-013-0129-1.
Mantle cell lymphoma is a distinct subtype of B-cell non-Hodgkin lymphoma, accounting for 3-10 % of all non-Hodgkin lymphoma cases. The median age at diagnosis is nearly 70 years. The prognosis of patients is based on the Mantle Cell Lymphoma International Prognostic Index, which is calculated on the basis of four independent prognostic factors (age, performance status, serum lactate dehydrogenase and leukocyte count). Treatment of elderly patients with de novo untreated mantle cell lymphoma is based on rituximab combined with chemotherapy. The most commonly used regimen is the classical CHOP21 (cyclophosphamide, doxorubicin, vincristine and prednisone) regimen. Bendamustine is also an option, especially for patients with cardiac comorbidities. In elderly patients who are relatively young and fit, an approach based on treatment usually used for younger patients, with cytarabine-based induction followed by autologous stem cell transplantation, should be discussed. Treatment of relapsing patients is based on the use of newer effective drugs, including bortezomib, lenalidomide and thalidomide, and mammalian target of rapamycin (mTOR) inhibitors, such as temsirolimus. These drugs are often combined with rituximab and can be prescribed in combination with chemotherapy. Promising new drugs are Bruton tyrosine kinase inhibitors and other inhibitors of the phosphoinositide 3-kinase (PI3K)-mTOR-protein kinase B (AKT) pathway. Despite these new advances, mantle cell lymphoma remains an incurable disease, and further basic and clinical research is warranted.
套细胞淋巴瘤是一种独特的 B 细胞非霍奇金淋巴瘤亚型，占所有非霍奇金淋巴瘤病例的 3-10%。诊断时的中位年龄接近 70 岁。患者的预后基于套细胞淋巴瘤国际预后指数，该指数基于四个独立的预后因素（年龄、体能状态、血清乳酸脱氢酶和白细胞计数）进行计算。初治的老年套细胞淋巴瘤患者的治疗基于利妥昔单抗联合化疗。最常用的方案是经典的 CHOP21（环磷酰胺、多柔比星、长春新碱和泼尼松）方案。苯达莫司汀也是一种选择，特别是对于有心脏合并症的患者。对于相对年轻和健康的老年患者，可以讨论基于通常用于年轻患者的治疗方法，包括基于阿糖胞苷的诱导治疗后进行自体干细胞移植。复发性患者的治疗基于使用新的有效药物，包括硼替佐米、来那度胺和沙利度胺，以及哺乳动物雷帕霉素靶蛋白（mTOR）抑制剂，如替西罗莫司。这些药物通常与利妥昔单抗联合使用，并可与化疗联合使用。有前途的新药是布鲁顿酪氨酸激酶抑制剂和其他磷酸肌醇 3-激酶（PI3K）-mTOR-蛋白激酶 B（AKT）通路抑制剂。尽管有这些新进展，但套细胞淋巴瘤仍然是一种不可治愈的疾病，需要进一步进行基础和临床研究。
