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Mantle cell lymphoma (MCL) is an aggressive B-cell non-Hodgkin lymphoma, and the molecular hallmark of MCL is a chromosomal translocation: t(11;14)(q13;q32), which causes cyclin D1 (CCND1) is overexpressed
.
MCL accounts for 3.
07% of all lymphoma cases, and is one of the diseases with the worst clinical prognosis of lymphoma
.
In recent years, with the application of new drugs, there are more options for the treatment of MCL, and the prognosis of patients has been improved
.
The 2021 NCCN mantle cell lymphoma 5th edition guideline update further advances the standardized treatment of MCL
.
In this issue of Lymphoma Academy, Prof.
Song Yuqin from the Lymphoma Department of Peking University Cancer Hospital is specially invited to review the main contents of the 2021 V5 version of the NCCN Mantle Cell Lymphoma Guidelines for readers
.
Overview of Mantle Cell Lymphoma MCL is a rare non-Hodgkin lymphoma (NHL): MCL accounts for about 3% of adult NHL patients worldwide.
According to epidemiological statistics in China, MCL accounts for 3.
07% of all lymphoma cases.
That is, there are about 1820 new cases every year, and the incidence of MCL in China is lower than that in European and American countries
.
In the chapter of MCL overview, Professor Song Yuqin introduced MCL in detail from the epidemiology, pathogenesis, clinical manifestations and prognosis, diagnosis and differential diagnosis of MCL in combination with NCCN guidelines
.
The first-line strong induction therapy for MCL is aimed at prolonging patient survival as long as possible
.
The NCCN guidelines point out that for newly-treated MCL patients, ① for stage I or II continuous lesions: immunochemotherapy (non-intensive chemotherapy regimen) ± local radiotherapy can be considered; ② for stage II non-consecutive lesions: radiotherapy is not recommended, and immunotherapy is recommended.
chemotherapy regimen
.
③ Indolent MCL: With low tumor burden and the patient has no discomfort, observation and follow-up can be selected under the doctor's advice; when symptoms or other treatment indications are present during follow-up, a comprehensive examination should be performed to clarify the nature of the patient's disease.
Sexual MCL should be treated
.
④ Stage II large mass and stage III-IV: stratified treatment
.
Regarding the stratified treatment of MCL, for young patients with good physical condition who are suitable for autologous hematopoietic stem cell transplantation (ASCT), it is recommended to use intensive chemotherapy (the NCCN guidelines recommend alternating R-CHOP and cytarabine).
Consider ASCT as a first-line consolidation regimen, and then recommend rituximab for maintenance therapy; non-intensive chemotherapy regimens are recommended for patients who are not suitable for ASCT (NCCN guidelines recommend BR, VR-CAP, R-CHOP, and R2 regimens preferentially) or participate in clinical trials
.
First-line non-strong induction therapy for MCL For patients ≥65 years old or <65 years old who are not suitable for strong induction chemotherapy and transplantation, R-CHOP, BR, VR-CAP, and R2 are all recommended non-strong induction chemotherapy regimens.
The rate is high and the patient tolerates it well
.
Among the four regimens, most research results show that patients receiving BR regimen have longer PFS, and BR combined with BTK inhibitor is expected to further improve prognosis and benefit patients
.
After R-CHOP and R-HyperCVAD induction chemotherapy, rituximab maintenance therapy can achieve good prognosis
.
However, the trial data showed that there was no significant benefit of rituximab maintenance therapy after using the BR regimen.
There is no trial data to support VR-CAP and whether to use rituximab maintenance after the RBAC regimen
.
For patients who only achieved PR after first-line therapy, consider using second-line therapy for salvage therapy to improve the quality of remission; for patients who relapse after first-line therapy or who have disease progression during initial therapy, participating in clinical trials is the first choice
.
In the absence of appropriate clinical trials, second-line therapy may be considered
.
Second-line and follow-up treatment of MCL MCL patients have a high recurrence rate after first-line treatment, and the increase in the number of treatment lines is closely related to poor prognosis.
Therefore, effective second-line treatment can greatly delay the progression of MCL and improve the prognosis of patients, which has always been a clinical concern.
Research hotspots
.
For the treatment of R/R MCL, the preferred regimens recommended by the NCCN guidelines include BTK inhibitor alone or BTK inhibitor combined with rituximab and lenalidomide combined with rituximab
.
BR, bortezomib and other programs can be selected according to the specific situation of the patient
.
For patients with early recurrence, regimens containing small molecule inhibitors (eg, BTK inhibitors) or lenalidomide (±rituximab) are preferred; for patients with advanced recurrence, alternative non-cross-resistant chemotherapy regimens, such as benzophenone, are recommended.
Damustine (± rituximab)
.
The good efficacy and safety of BTK inhibitors have been confirmed in the PCYC-1104-CA study.
Monotherapy for early relapsed MCL has an overall response rate (ORR) as high as 67%, with a median PFS and OS of 13 months and 22.
5 months, respectively.
month
.
On the basis of BTK inhibitor, the combination of rituximab regimen in the treatment of early relapsed MCL can help to further improve the efficacy.
The ORR is as high as 88%, and the tolerability is good, which is recommended by the guidelines as a priority
.
New treatment regimens for MCL With the development of new drugs and the accelerating process of clinical exploration, many effective new targeted drugs or new treatment regimens have emerged for the treatment of MCL, such as PI3K inhibitors, mTOR inhibitors, BCL-2 inhibitors, Proteasome inhibitors, CDK4/6 inhibitors, HDAC inhibitors, CAR-T therapy, etc.
, have shown broad prospects in the field of MCL treatment
.
Professor Song Yuqin explained some of the new drugs recommended in the NCCN guidelines (BCL-2 inhibitors, CDK4/6 inhibitors and CAR-T therapy) and related clinical trial data
.
Summary • First-line treatment of MCL: In young, transplant-eligible patients, strong induction chemotherapy combined with ASCT and rituximab maintenance therapy is the main therapy; in young but transplant-ineligible or elderly patients, non-intensive induction chemotherapy combined with Rituximab maintenance therapy is the mainstay
.
• Second-line treatment of MCL: BTK inhibitors have shown good efficacy and safety, and have been recommended by the NCCN guidelines as a priority
.
• Novel treatments: Small molecule targeted drugs such as BCL-2 inhibitors, and new treatment options such as CAR-T therapy bring new hope to R/R MCL patients.
We look forward to the update of these data to bring better survival to patients benefit
.
Professor Song Yuqin Deputy Director, Chief Physician and Doctoral Supervisor of the Lymphoma Department of Peking University Cancer Hospital Director of the Chinese Society of Clinical Oncology (CSCO) Secretary General of the Anti-Lymphoma Alliance of the Chinese Society of Clinical Oncology (CSCO) Chairman of the Lymphoma and Hematology Committee of the Beijing Anti-Cancer Association Vice-chairman of the Lymphoma Professional Committee of the Chinese Society of Geriatric Oncology Vice-chairman of the Lymphoma Professional Committee of the Chinese Geriatrics Health Care Association Member of the Standing Committee and Secretary of the Oncology Committee of the Beijing Medical Association References: NCCN Guidelines B-Cell Lymphomas 2021 Version 5 All materials in this tweet ( Text and video) are supported by AstraZeneca and are only for reference by medical and health professionals.
Document approval number: CN-90108 Video approval number: CN-90102, CN-90103, CN-90104, CN-90105, CN-90106 stamp " Read the original", we make progress together
.
MCL accounts for 3.
07% of all lymphoma cases, and is one of the diseases with the worst clinical prognosis of lymphoma
.
In recent years, with the application of new drugs, there are more options for the treatment of MCL, and the prognosis of patients has been improved
.
The 2021 NCCN mantle cell lymphoma 5th edition guideline update further advances the standardized treatment of MCL
.
In this issue of Lymphoma Academy, Prof.
Song Yuqin from the Lymphoma Department of Peking University Cancer Hospital is specially invited to review the main contents of the 2021 V5 version of the NCCN Mantle Cell Lymphoma Guidelines for readers
.
Overview of Mantle Cell Lymphoma MCL is a rare non-Hodgkin lymphoma (NHL): MCL accounts for about 3% of adult NHL patients worldwide.
According to epidemiological statistics in China, MCL accounts for 3.
07% of all lymphoma cases.
That is, there are about 1820 new cases every year, and the incidence of MCL in China is lower than that in European and American countries
.
In the chapter of MCL overview, Professor Song Yuqin introduced MCL in detail from the epidemiology, pathogenesis, clinical manifestations and prognosis, diagnosis and differential diagnosis of MCL in combination with NCCN guidelines
.
The first-line strong induction therapy for MCL is aimed at prolonging patient survival as long as possible
.
The NCCN guidelines point out that for newly-treated MCL patients, ① for stage I or II continuous lesions: immunochemotherapy (non-intensive chemotherapy regimen) ± local radiotherapy can be considered; ② for stage II non-consecutive lesions: radiotherapy is not recommended, and immunotherapy is recommended.
chemotherapy regimen
.
③ Indolent MCL: With low tumor burden and the patient has no discomfort, observation and follow-up can be selected under the doctor's advice; when symptoms or other treatment indications are present during follow-up, a comprehensive examination should be performed to clarify the nature of the patient's disease.
Sexual MCL should be treated
.
④ Stage II large mass and stage III-IV: stratified treatment
.
Regarding the stratified treatment of MCL, for young patients with good physical condition who are suitable for autologous hematopoietic stem cell transplantation (ASCT), it is recommended to use intensive chemotherapy (the NCCN guidelines recommend alternating R-CHOP and cytarabine).
Consider ASCT as a first-line consolidation regimen, and then recommend rituximab for maintenance therapy; non-intensive chemotherapy regimens are recommended for patients who are not suitable for ASCT (NCCN guidelines recommend BR, VR-CAP, R-CHOP, and R2 regimens preferentially) or participate in clinical trials
.
First-line non-strong induction therapy for MCL For patients ≥65 years old or <65 years old who are not suitable for strong induction chemotherapy and transplantation, R-CHOP, BR, VR-CAP, and R2 are all recommended non-strong induction chemotherapy regimens.
The rate is high and the patient tolerates it well
.
Among the four regimens, most research results show that patients receiving BR regimen have longer PFS, and BR combined with BTK inhibitor is expected to further improve prognosis and benefit patients
.
After R-CHOP and R-HyperCVAD induction chemotherapy, rituximab maintenance therapy can achieve good prognosis
.
However, the trial data showed that there was no significant benefit of rituximab maintenance therapy after using the BR regimen.
There is no trial data to support VR-CAP and whether to use rituximab maintenance after the RBAC regimen
.
For patients who only achieved PR after first-line therapy, consider using second-line therapy for salvage therapy to improve the quality of remission; for patients who relapse after first-line therapy or who have disease progression during initial therapy, participating in clinical trials is the first choice
.
In the absence of appropriate clinical trials, second-line therapy may be considered
.
Second-line and follow-up treatment of MCL MCL patients have a high recurrence rate after first-line treatment, and the increase in the number of treatment lines is closely related to poor prognosis.
Therefore, effective second-line treatment can greatly delay the progression of MCL and improve the prognosis of patients, which has always been a clinical concern.
Research hotspots
.
For the treatment of R/R MCL, the preferred regimens recommended by the NCCN guidelines include BTK inhibitor alone or BTK inhibitor combined with rituximab and lenalidomide combined with rituximab
.
BR, bortezomib and other programs can be selected according to the specific situation of the patient
.
For patients with early recurrence, regimens containing small molecule inhibitors (eg, BTK inhibitors) or lenalidomide (±rituximab) are preferred; for patients with advanced recurrence, alternative non-cross-resistant chemotherapy regimens, such as benzophenone, are recommended.
Damustine (± rituximab)
.
The good efficacy and safety of BTK inhibitors have been confirmed in the PCYC-1104-CA study.
Monotherapy for early relapsed MCL has an overall response rate (ORR) as high as 67%, with a median PFS and OS of 13 months and 22.
5 months, respectively.
month
.
On the basis of BTK inhibitor, the combination of rituximab regimen in the treatment of early relapsed MCL can help to further improve the efficacy.
The ORR is as high as 88%, and the tolerability is good, which is recommended by the guidelines as a priority
.
New treatment regimens for MCL With the development of new drugs and the accelerating process of clinical exploration, many effective new targeted drugs or new treatment regimens have emerged for the treatment of MCL, such as PI3K inhibitors, mTOR inhibitors, BCL-2 inhibitors, Proteasome inhibitors, CDK4/6 inhibitors, HDAC inhibitors, CAR-T therapy, etc.
, have shown broad prospects in the field of MCL treatment
.
Professor Song Yuqin explained some of the new drugs recommended in the NCCN guidelines (BCL-2 inhibitors, CDK4/6 inhibitors and CAR-T therapy) and related clinical trial data
.
Summary • First-line treatment of MCL: In young, transplant-eligible patients, strong induction chemotherapy combined with ASCT and rituximab maintenance therapy is the main therapy; in young but transplant-ineligible or elderly patients, non-intensive induction chemotherapy combined with Rituximab maintenance therapy is the mainstay
.
• Second-line treatment of MCL: BTK inhibitors have shown good efficacy and safety, and have been recommended by the NCCN guidelines as a priority
.
• Novel treatments: Small molecule targeted drugs such as BCL-2 inhibitors, and new treatment options such as CAR-T therapy bring new hope to R/R MCL patients.
We look forward to the update of these data to bring better survival to patients benefit
.
Professor Song Yuqin Deputy Director, Chief Physician and Doctoral Supervisor of the Lymphoma Department of Peking University Cancer Hospital Director of the Chinese Society of Clinical Oncology (CSCO) Secretary General of the Anti-Lymphoma Alliance of the Chinese Society of Clinical Oncology (CSCO) Chairman of the Lymphoma and Hematology Committee of the Beijing Anti-Cancer Association Vice-chairman of the Lymphoma Professional Committee of the Chinese Society of Geriatric Oncology Vice-chairman of the Lymphoma Professional Committee of the Chinese Geriatrics Health Care Association Member of the Standing Committee and Secretary of the Oncology Committee of the Beijing Medical Association References: NCCN Guidelines B-Cell Lymphomas 2021 Version 5 All materials in this tweet ( Text and video) are supported by AstraZeneca and are only for reference by medical and health professionals.
Document approval number: CN-90108 Video approval number: CN-90102, CN-90103, CN-90104, CN-90105, CN-90106 stamp " Read the original", we make progress together
