Mantle cell lymphoma (MCL) is a rare B-cell lymphoma defined by the translocation (11;14) (q13; q32) and resulting in overexpression of cyclin D1. MCL accounts for 3-10% of all non-Hodgkin’s lymphomas (NHL) , with an incidence of about 0.4 per 100,000 person/years. MCL is a heterogeneous disease and recognized as an aggressive lymphoma requiring treatment at diagnosis. The patients are mainly elderly men. 1
No risk factors have been clearly identified for MCL. Several chemical substances, such as solvents, insecticides, pesticides and fertilizers, as well as dusts and particles, hair dyes, smoking and diet have been suggested as possible aetiological factors, but there are no convincing data. The incidence rate has increased in the total population, particularly in the elderly. 1
Usually MCL occurs in adults with a median age ranging between 60 and 65 years, and a high male-to-female ratio. Generalized lymphadenopathy is the most frequent clinical finding, but isolated splenomegaly without lymphadenopathies has been occasionally observed. These patients present with a leukemic form, usually with splenomegaly. MCL can involve any region of the gastrointestinal tract, occasionally presenting as multiple intestinal polyposis. Less commonly, skin, lung, breast, soft tissue, salivary glands, and orbit are involved. 1
For patients ≤ 65 years old or in good general condition, suitable for autologous hematopoietic stem cell transplantation (ASCT), the standard chemotherapy regimen should be selected, and ASCT consolidation should be carried out after remission.
For patients aged >65 years old or with poor general conditions and not suitable for ASCT, the combination chemotherapy with less adverse reactions and better tolerance should be selected, and the combination with CD20 monoclonal antibody chemotherapy can improve the long-term survival rate of patients.
The median time to relapse after standard chemotherapy was about 1-2 years in patients with mantle cell lymphoma. There is no standard treatment recommendation for patients with relapse, which needs to be combined with previous chemotherapy regimens, treatment response, response duration and general conditions of patients.
Proteasome inhibitor was one of the first new drugs to be approved for relapse /refractory MCL, either alone or in combination with CD20 monoclonal antibody or chemotherapy. At present, BTK inhibitor is regarded as one of the most effective, with high total overall response rate and complete response rate. It is also possible to choose a regimen that is not cross-resistant to the previous treatment regimen, and new drugs combined with chemotherapy may be considered if conditions permit. If the patient is young, it may be considered to reduce the pretreatment dose of allogeneic hematopoietic stem cell transplantation. 1,2
1. Mantle Cell Lymphoma .Crti Rev Oncol Hematol .2012 April ;82(1):78-101.
2. 套細胞淋巴瘤診斷與治療中國專家共識（2016年版）. 中華血液學雜志,2016，37（9）：737-741 .
CP-87250 Approved date 2019-4-28