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


{{CMG}}; {{AE}}{{Akram}} {{AS}}
{{CMG}}; {{AE}}  
 
{{Akram}} {{AS}}
 
==Overview==
==Overview==
The prognosis of mantle cell lymphoma has historically been very poor. However, recently improvements have been made and the median survival has increased from 3-4 years to 5-7 years.<ref>{{Cite journal
The prognosis of mantle cell lymphoma has historically been very poor. However, recently improvements have been made and the median survival has increased from 3-4 years to 5-7 years.<ref>{{Cite journal
Line 34: Line 37:
== Prognosis ==
== Prognosis ==
* The evolution of disease in mantle cell lymphoma is highly heterogeneous.  Therefore, it is very important to stratify the patients according to their biological risk to better direct the therapeutic approaches.
* The evolution of disease in mantle cell lymphoma is highly heterogeneous.  Therefore, it is very important to stratify the patients according to their biological risk to better direct the therapeutic approaches.
* The Mantle Cell Lymphoma International Prognostic Index (MIPI) is now widely used as a prognostic model in MCL patients. It uses the following four parameters to assess the prognosis (Age, ECOG performance, LDH/LSN levels, WBC Count):<ref>{{Cite journal
* The Mantle Cell Lymphoma International Prognostic Index (MIPI) is now widely used as a prognostic model in MCL patients. It uses the following four parameters to assess the prognosis (Age, ECOG performance, LDH/LSN levels, WBC Count):  
| author = [[Christian H. Geisler]], [[Arne Kolstad]], [[Anna Laurell]], [[Riikka Raty]], [[Mats Jerkeman]], [[Mikael Eriksson]], [[Marie Nordstrom]], [[Eva Kimby]], [[Anne Marie Boesen]], [[Herman Nilsson-Ehle]], [[Outi Kuittinen]], [[Grete F. Lauritzsen]], [[Elisabeth Ralfkiaer]], [[Mats Ehinger]], [[Christer Sundstrom]], [[Jan Delabie]], [[Marja-Liisa Karjalainen-Lindsberg]], [[Peter Brown]] & [[Erkki Elonen]]
| title = The Mantle Cell Lymphoma International Prognostic Index (MIPI) is superior to the International Prognostic Index (IPI) in predicting survival following intensive first-line immunochemotherapy and autologous stem cell transplantation (ASCT)
| journal = [[Blood]]
| volume = 115
| issue = 8
| pages = 1530–1533
| year = 2010
| month = February
| doi = 10.1182/blood-2009-08-236570
| pmid = 20032504
}}</ref>
   
{| class="wikitable"
{| class="wikitable"
|+
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|29 months
|29 months
|}
|}
* Prognosis of mantle cell lymphoma is problematic and indexes do not work as well due to patients presenting with advanced stage disease. Staging is used but is not very informative, since the malignant B-cells can travel freely though the [[lymphatic system]] and therefore most patients are at stage II or IV at diagnosis. Prognosis is not strongly affected by staging in mantle cell lymphoma and the concept of metastasis does not really apply.
* The tumor proliferation marker, Ki-67, is also an important prognostic marker for mantle cell lymphoma. Values of >30% are associated with a poor progression free survival.
* Mantle cell lymphoma cell types can aid in prognosis in a subjective way.
* Presence of blastoid or pleomorphic morphologic characteristics have also shown to have poor outcomes.
:* Diffuse is spread through the node.
 
:* Nodular are small groups of collected cells spread through the node. Diffuse and nodular are similar in behavior.  
* Mantle cell lymphoma is one of the few [[NHL]]s that can cross the boundary into the brain, yet it can be treated in that event.  
:* Blastic is a larger cell type. Blastic is faster growing and it is harder to get long remissions. Some thought is that given a long time, some non-blastic mantle cell lymphoma transforms to blastic. Although survival of most blastic patients is shorter, some data shows that 25% of blastic mantle cell lymphoma patients survive to 5 years. That is longer than diffuse type and almost as long as nodular (almost 7 yrs
* There are a number of prognostic indicators that have been studied. There is not universal agreement on their importance or usefulness in prognosis.  
* [[Ki-67_(Biology)|Ki-67]] is an indicator of how fast cells mature and is expressed in a range from about 10% to 90%.  
* [[Ki-67_(Biology)|Ki-67]] is an indicator of how fast cells mature and is expressed in a range from about 10% to 90%.  
:* The lower the percentage, the lower the speed of maturity, and the more indolent the disease.<ref name="pmidPMID: 16597597">{{cite journal| author=Katzenberger T, Petzoldt C, Höller S, Mäder U, Kalla J, Adam P et al.| title=The Ki67 proliferation index is a quantitative indicator of clinical risk in mantle cell lymphoma. | journal=Blood | year= 2006 | volume= 107 | issue= 8 | pages= 3407 | pmid=PMID: 16597597 | doi=10.1182/blood-2005-10-4079 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16597597  }} </ref>
:* The lower the percentage, the lower the speed of maturity, and the more indolent the disease.<ref name="pmidPMID: 16597597">{{cite journal| author=Katzenberger T, Petzoldt C, Höller S, Mäder U, Kalla J, Adam P et al.| title=The Ki67 proliferation index is a quantitative indicator of clinical risk in mantle cell lymphoma. | journal=Blood | year= 2006 | volume= 107 | issue= 8 | pages= 3407 | pmid=PMID: 16597597 | doi=10.1182/blood-2005-10-4079 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16597597  }} </ref>

Revision as of 19:24, 29 November 2018

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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] Sowminya Arikapudi, M.B,B.S. [3]

Overview

The prognosis of mantle cell lymphoma has historically been very poor. However, recently improvements have been made and the median survival has increased from 3-4 years to 5-7 years.[1] It is very important to stratify the patients according to their biological risk to better direct the therapeutic approaches.

Natural History

  • Mantle cell lymphoma is generally considered to be an aggressive disease with patients having early relapses and poor long-term survival rates.
  • However, rare forms of the disease which show an indolent behavior, have better outcomes. [2]

Prognosis

  • The evolution of disease in mantle cell lymphoma is highly heterogeneous. Therefore, it is very important to stratify the patients according to their biological risk to better direct the therapeutic approaches.
  • The Mantle Cell Lymphoma International Prognostic Index (MIPI) is now widely used as a prognostic model in MCL patients. It uses the following four parameters to assess the prognosis (Age, ECOG performance, LDH/LSN levels, WBC Count):
Mantle Cell Lymphoma International Prognostic Index
Points Age (years) ECOG LDH/LSN (upper limit) WBC Count (x 109/L)
0 <50 0/1 <0.67 <6700
1 50-59 - 0.67-0.99 6700-9999
2 60-69 1/2 1-1.49 10000-14999
3 >70 - >1.50 >15000
Risk Points Survival at five years
Low 0-3 60% alive at 5 years
Medium 4-5 51 months
High >5 29 months
  • The tumor proliferation marker, Ki-67, is also an important prognostic marker for mantle cell lymphoma. Values of >30% are associated with a poor progression free survival.
  • Presence of blastoid or pleomorphic morphologic characteristics have also shown to have poor outcomes.
  • Mantle cell lymphoma is one of the few NHLs that can cross the boundary into the brain, yet it can be treated in that event.
  • There are a number of prognostic indicators that have been studied. There is not universal agreement on their importance or usefulness in prognosis.
  • Ki-67 is an indicator of how fast cells mature and is expressed in a range from about 10% to 90%.
  • The lower the percentage, the lower the speed of maturity, and the more indolent the disease.[3]

References

  1. P. Martin, A. Chadburn, P. Christos, R. Furman, J. Ruan, M. A. Joyce, E. Fusco, P. Glynn, R. Elstrom, R. Niesvizky, E. J. Feldman, T. B. Shore, M. W. Schuster, S. Ely, D. M. Knowles, S. Chen-Kiang, M. Coleman & J. P. Leonard (2008). "Intensive treatment strategies may not provide superior outcomes in mantle cell lymphoma: overall survival exceeding 7 years with standard therapies". Annals of oncology : official journal of the European Society for Medical Oncology. 19 (7): 1327–1330. doi:10.1093/annonc/mdn045. PMID 18349031. Unknown parameter |month= ignored (help)
  2. Pedro Jares & Elias Campo (2008). "Advances in the understanding of mantle cell lymphoma". British journal of haematology. 142 (2): 149–165. doi:10.1111/j.1365-2141.2008.07124.x. PMID 18410453. Unknown parameter |month= ignored (help)
  3. Katzenberger T, Petzoldt C, Höller S, Mäder U, Kalla J, Adam P; et al. (2006). "The Ki67 proliferation index is a quantitative indicator of clinical risk in mantle cell lymphoma". Blood. 107 (8): 3407. doi:10.1182/blood-2005-10-4079. PMID 16597597 PMID: 16597597 Check |pmid= value (help).

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