View source for Mantle cell lymphoma medical therapy

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{{Mantle cell lymphoma }}

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

==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 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.

==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]] 

=== Stage I-II: ===
* [[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)

=== Stage II (bulky) and Stage III-IV: ===

==== First-line induction therapy: ====
* 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.

==== First-line consolidation therapy: ====
* 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.

==== Second-line 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>
**[[Bendamustine]] +/- [[rituximab]]
**[[Bortezomib]] +/- [[rituximab]]
**[[Cladribine]] +/- [[rituximab]]
**FC ([[fludarabine]], [[cyclophosphamide]]) +/- [[rituximab]]
**FCMR ([[fludarabine]], [[cyclophosphamide]], [[mitoxantrone]], [[rituximab]])
**FMR ([[fludarabine]], [[mitoxantrone]], [[rituximab]])
**[[Lenalidomide]] +/- [[rituximab]]
**PCR ([[pentostatin]], [[cyclophosphamide]], [[rituximab]])
**PEPC ([[prednisone]], [[etoposide]], [[procarbazine]], [[cyclophosphamide]]) +/- [[rituximab]]
**[[Ibrutinib]]
* [[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.

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

[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]