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{{ Mantle cell lymphoma }}
{{Mantle cell lymphoma }}


{{CMG}}
{{CMG}}; {{AE}} {{Akram}}


==Overview==
==Overview==
==Medical Therapy==


There are no proven standards of treatment for MCL, and not even consensus among specialists on how to treat it optimally. Many regimens are available and often get good response rates, but patients almost always get disease progression after chemotherapy. Each relapse is typically more difficult to treat, and relapse is generally faster. Fortunately, regimens are available that will treat relapse, and new approaches are under test. Because of the aforementioned factors, many MCL patients enroll in clinical trials to get the latest treatments.  
The mainstay of treatment for mantle cell lymphoma is [[chemotherapy]]. However, [[immunotherapy]], [[radioimmunotherapy]], [[targeted therapy]] using newer biologic agents and [[stem cell transplantation]] are also used along with [[chemotherapy]] to treat the [[disease]]. Mantle cell lymphoma shows a heterogeneous [[clinical]] behavior, with some patients having [[Indolent mantle cell lymphoma|indolent]] [[disease]] whereas a vast majority show aggressive presentation. Most of the patients eventually [[relapse]] and have [[disease]] progression after treatment. Hence, mantle cell lymphoma is still considered an incurable [[disease]] and there is no consensus among [[Oncologist|oncologists]] about its optimal treatment. It is therefore recommended that mantle cell lymphoma patients are seen by physicians having extensive experience in dealing with mantle cell lymphoma and they are also encouraged to participate in [[Clinical trial|clinical trials]] to get the latest treatments.


There are four classes of treatments currently in general use: chemotherapy, immune based therapy, radioimmunotherapy and new biologic agents. The phases of treatment are generally: frontline, following diagnosis, consolidation, after frontline response (to prolong remissions), and relapse. Relapse is usually experienced multiple times.
==Medical Therapy==
Mantle cell lymphoma shows a heterogeneous clinical behavior, with some patients having [[Indolent mantle cell lymphoma|indolent disease]] whereas a vast majority show aggressive presentation. Most of the patients eventually [[relapse]] and have [[disease]] progression after treatment. Hence, mantle cell lymphoma is still considered an incurable disease and there is no consensus among [[oncologists]] about its optimal treatment. It is therefore recommended that mantle cell lymphoma patients are seen by [[Physician|physicians]] having extensive experience in dealing with mantle cell lymphoma and they are also encouraged to participate in [[Clinical trial|clinical trials]] to get the latest treatments.  
*Different types of treatment currently being used to treat mantle cell lymphoma are as follows:
** [[Chemotherapy]]
** [[Immunotherapy]]
** [[Radioimmunotherapy]]
** [[Targeted therapy]] using newer biologic agents
** [[Stem cell transplantation]]


===Chemotherapy===
=== Stage I-II: ===
[[Chemotherapy]] is widely used as frontline treatment, and often is not repeated in relapse due to side effects. Alternate chemotherapy is sometimes used at first relapse. For frontline treatment, [[CHOP]] with [[rituximab]] (Rituxan, Mabthera) is the most common chemotherapy, and often given as outpatient by IV. A stronger chemotherapy with greater side effects (mostly hematologic) is [[HyperCVAD]], often given as in-patient, with rituximab and generally to fitter patients (some of which are over 65). HyperCVAD is becoming popular and showing promising results, especially with rituximab. It can be used on some elderly (over 65) patients, but seems only beneficial when the baseline Beta-2-MG blood test was normal. It is showing better complete remissions (CR) and progression free survival (PFS) than CHOP regimens. Another chemotherapy class is fludarabine monotherapy, sometimes combined with cyclophosphamide and mitoxatrone, usually with rituximab. Cladribine and clofarabine are two other drugs being investigated in MCL. Cytotoxic chemotherapies, including bendamustin, are being studied alone and with similar combinations. A relatively new regimen that uses old drugs is PEP-C, which includes relatively small, daily doses of[[prednisone]], [[etoposide]], [[procarbazine]], and [[cyclophosphamide]], taken orally, has proven effective for relapsed patients[http://www.asco.org/ASCO/Abstracts+&+Virtual+Meeting/Abstracts?&vmview=abst_detail_view&confID=23&abstractID=104642].
* [[Radiotherapy]] alone or combination chemoimmunotherapy with or without [[radiotherapy]] is recommended.<ref>https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf</ref>
* For patients with complete response(CR), clinical follow up is conducted every 3-6 months for 5 years, and then on a yearly basis.
* For patients treated with [[radiotherapy]] alone initially, [[disease]] progression and [[Relapse|relapses]] after a CR or partial response(PR) is treated with first-line induction therapy (recommended for stage II bulky and stage II-IV disease)
* For patients treated with chemoimmunotherapy with or without [[radiotherapy]], [[disease]] progression and [[Relapse|relapses]] after a CR or PR is treated with second-line therapy regimens (recommended for stage II bulky and stage II-IV disease)


Another approach involves using very high doses of chemotherapy, sometimes combined with [[total body irradiation]] (TBI), in an attempt to destroy all evidence of the disease.  The downside to this is the destruction of the patients' entire immune system as well, requiring rescue by transplantation of a new immune system, using either ones' own previously treated and stored stem cells (an autologous stem cell transplant), or those from a matched donor (an allogeneic stem cell transplant).
=== Stage II (bulky) and Stage III-IV: ===


===Immunotherapy===
==== First-line induction therapy: ====
[[Immunotherapy|Immune-based therapy]] is dominated now by the oft used and effective [[rituximab]] monoclonal antibody, sold under the trade name  Rituxan (or as Mabthera in Europe and Australia). Some say it is a landmark medicine. It can have good activity against MCL alone but especially in combination with chemotherapies to prolong response duration. Rituximab essentially tags the cancer cells for destruction by the body. There are newer variations on monoclonal antibodies combined with radioactive molecules known as [[Radioimmunotherapy]] (RIT). These include [[Zevalin]] and [[Bexxar]].
* Aggressive therapy:<ref>https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf</ref>
**Hyper-[[CVAD regimen|CVAD]] ([[cyclophosphamide]], [[vincristine]], [[doxorubicin]] (also known by its trade name, [[Adriamycin]]), and [[dexamethasone]]) + [[rituximab]]
**Dose-intensified [[CHOP]] ([[cyclophosphamide]], [[Doxorubicin|hydroxydaunorubicin]], [[Vincristine|oncovin]] ([[vincristine]]) and [[prednisone]]) alternating with [[rituximab]] + high-dose [[cytarabine]] (NORDIC regimen)
**[[Rituximab]] and [[methotrexate]] with augmented [[CHOP]]
**Sequential [[R-CHOP regimen|R-CHOP]] and [[R-ICE regimen|R-ICE]] ([[Ifosfamide]], [[carboplatin]], [[etoposide]])
**Alternating [[R-CHOP regimen|R-CHOP]] and [[DHAP regimen|R-DHAP]] ([[Dexamethasone]], High dose Ara-C [[Cytarabine]], [[Cisplatin|Platinol]])
* Less aggressive therapy:
**[[Bendamustine]] + [[rituximab]]
**[[Bortezomib]], [[rituximab]], [[cyclophosphamide]], [[doxorubicin]], and [[prednisone]] (VR-CAP)
**[[Cladribine]] + [[rituximab]]
**[[CHOP regimen|CHOP]] + [[rituximab]] ([[R-CHOP regimen|R-CHOP]])
**Modified Hyper-[[CVAD regimen|CVAD]] with [[rituximab]] maintainence in patients older than 65 years.
* For patients with CR to first-line therapies, HDT/ASCR (High dose therapy/Autologous Stem Cell Rescue) or [[clinical trial]] participation is recommended. Follow up is conducted every 3-6 months for 5 years, and then on a yearly basis.
* For patients with PR to first-line therapies, second-line therapies may be considered.


===Targeted Therapy===
==== First-line consolidation therapy: ====
New targeted agents include the proteasome inhibitor [[Velcade]] and mTor inhibitors such as [[Torisel|temsirolimus]].
* HDT/ASCR is recommended.<ref>https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf</ref>
* Patients who are not HDT/ASCR candidates, and are in remission after [[R-CHOP]] therapy, may get [[rituximab]] maintenance therapy every 8 weeks.


==Drug Regimen==
==== Second-line therapy: ====
*Induction therapy
* The following therapies may be used in [[Relapse|relapsed]]/[[refractory]] disease:<ref>https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf</ref>
:*Aggressive therapy
**[[Bendamustine]] +/- [[rituximab]]
::*Drug Regimen: Hyper CVAD ([[Cyclophosphamide]], [[Vincristine]], [[Doxorubicin]] {{and}} [[Dexamethasone]] alternating with high-dose [[Methotrexate]] {{and}} [[Cytarabine]])+ [[Rituximab]]
**[[Bortezomib]] +/- [[rituximab]]
::*Drug Regimen: NORDIC regimen ([[Rituximab]] {{plus}} [[Cyclophosphamide]], [[Vincristine]], [[Doxorubicin]], [[Prednisone]] alternating with [[Rituximab]] {{plus}} high dose [[Cytarabine]]
**[[Cladribine]] +/- [[rituximab]]
::*Drug Regimen: Alternating RCHOP/RDHAP ([[Rituximab]], [[Cyclophosphamide]], [[Doxorubicin]], [[Vincristine]], [[Prednisone]])/([[Rituximab]], [[Dexamethasone]], [[Cisplatin]], [[Cytarabine]])
**FC ([[fludarabine]], [[cyclophosphamide]]) +/- [[rituximab]]
::*Drug Regimen: Sequential RCHOP/RICE ([[Rituximab]], [[Cyclophosphamide]], [[Doxorubicin]], [[Vincristine]], [[Prednisone]])/([[Rituximab]], [[Ifosfamide]], [[Carboplatin]], [[Etoposide]])
**FCMR ([[fludarabine]], [[cyclophosphamide]], [[mitoxantrone]], [[rituximab]])
:*Less aggressive therapy
**FMR ([[fludarabine]], [[mitoxantrone]], [[rituximab]])
::*Drug Regimen: [[Bendamustine]] {{plus}} [[Rituximab]]
**[[Lenalidomide]] +/- [[rituximab]]
::*Drug Regimen: CHOP {{plus}} [[Rituximab]] followed by consolidation with rituximab maintenance (375 mg/m<sup>2</sup>every 8 weeks until progression)
**PCR ([[pentostatin]], [[cyclophosphamide]], [[rituximab]])
::*Drug Regimen: [[Cladribine]] {{plus}} [[Rituximab]]
**PEPC ([[prednisone]], [[etoposide]], [[procarbazine]], [[cyclophosphamide]]) +/- [[rituximab]]
::*Drug Regimen: Modified rituximab-Hyper CVAD with rituximab maintenanace in patients older than 65 years
**[[Ibrutinib]]
*First-line Consolidation
* [[Autologous bone marrow transplantation|Autologous stem cell transplant]] and [[Allogeneic stem cell transplantation|allogeneic stem cell transplant]] can be used as second-line maintenance therapy for patients in [[Remission (medicine)|remission]] with [[Relapse|relapsed]] or [[refractory]] disease.
::*Drug Regimen: High dose therapy with autologous stem cell rescue
*Second-line therapy
::*Drug Regimen: [[Bendamustine]] {{withorwithout}} [[Rituximab]]
::*Drug Regimen: [[Bortezomib]] {{withorwithout}} [[Rituximab]]
::*Drug Regimen: [[Cladribine]] {{withorwithout}} [[Rituximab]]
::*Drug Regimen: FC ([[Fludarabine]], [[Cyclophosphamide]]) {{withorwithout}} [[Rituximab]]
::*Drug Regimen: FCMR ([[Fludarabine]], [[Cyclophosphamide]], [[Mitoxantrone]], [[Rituximab]])
::*Drug Regimen: FMR ([[Fludarabine]], [[Mitoxantrone]], [[Rituximab]])
::*Drug Regimen: [[Ibrutinib]]
::*Drug Regimen: [[Lenalidomide]] {{withorwithout}} [[Rituximab]])
::*Drug Regimen: PCR ([[Pentostatin]], [[Cyclophosphamide]], [[Rituximab]])
::*Drug Regimen: PEPC ([[Prednisone]], [[Etoposide]], [[Procarbazine]], [[Cyclophosphamide]] {{withorwithout}} [[Rituximab]])
:*Second-line Consolidation
::*Drug Regimen: Allogenic stem cell transplant


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
{{Hematology}}


[[Category:Blood disorders]]
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[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Disease]]
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[[Category:Hematology]]
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Latest revision as of 22:37, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ali Akram, M.B.B.S.[2]

Overview

The mainstay of treatment for mantle cell lymphoma is chemotherapy. However, immunotherapy, radioimmunotherapy, targeted therapy using newer biologic agents and stem cell transplantation are also used along with chemotherapy to treat the disease. Mantle cell lymphoma shows a heterogeneous clinical behavior, with some patients having indolent disease whereas a vast majority show aggressive presentation. Most of the patients eventually relapse and have disease progression after treatment. Hence, mantle cell lymphoma is still considered an incurable disease and there is no consensus among oncologists about its optimal treatment. It is therefore recommended that mantle cell lymphoma patients are seen by physicians having extensive experience in dealing with mantle cell lymphoma and they are also encouraged to participate in clinical trials to get the latest treatments.

Medical Therapy

Mantle cell lymphoma shows a heterogeneous clinical behavior, with some patients having indolent disease whereas a vast majority show aggressive presentation. Most of the patients eventually relapse and have disease progression after treatment. Hence, mantle cell lymphoma is still considered an incurable disease and there is no consensus among oncologists about its optimal treatment. It is therefore recommended that mantle cell lymphoma patients are seen by physicians having extensive experience in dealing with mantle cell lymphoma and they are also encouraged to participate in clinical trials to get the latest treatments.

Stage I-II:

  • Radiotherapy alone or combination chemoimmunotherapy with or without radiotherapy is recommended.[1]
  • For patients with complete response(CR), clinical follow up is conducted every 3-6 months for 5 years, and then on a yearly basis.
  • For patients treated with radiotherapy alone initially, disease progression and relapses after a CR or partial response(PR) is treated with first-line induction therapy (recommended for stage II bulky and stage II-IV disease)
  • For patients treated with chemoimmunotherapy with or without radiotherapy, disease progression and relapses after a CR or PR is treated with second-line therapy regimens (recommended for stage II bulky and stage II-IV disease)

Stage II (bulky) and Stage III-IV:

First-line induction therapy:

First-line consolidation therapy:

  • HDT/ASCR is recommended.[3]
  • Patients who are not HDT/ASCR candidates, and are in remission after R-CHOP therapy, may get rituximab maintenance therapy every 8 weeks.

Second-line therapy:

References

  1. ↑ https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf
  2. ↑ https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf
  3. ↑ https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf
  4. ↑ https://www.nccn.org/professionals/physician_gls/pdf/b-cell.pdf
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