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==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.   
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
| author = [[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]]
| title = Intensive treatment strategies may not provide superior outcomes in mantle cell lymphoma: overall survival exceeding 7 years with standard therapies
| journal = [[Annals of oncology : official journal of the European Society for Medical Oncology]]
| volume = 19
| issue = 7
| pages = 1327–1330
| year = 2008
| month = July
| doi = 10.1093/annonc/mdn045
| pmid = 18349031
}}</ref>  


==Natural History==
==Natural History==

Revision as of 16:50, 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]

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) has recently
  • 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.
  • Mantle cell lymphoma cell types can aid in prognosis in a subjective way.
  • Diffuse is spread through the node.
  • Nodular are small groups of collected cells spread through the node. Diffuse and nodular are similar in behavior.
  • 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).
  • The Mantle Cell Lymphoma International Prognostic Index (MIPI) was derived from a data set of 455 advanced stage mantle cell lymphoma patients treated in series of clinical trials in Germany/Europe. Of the evaluable population, approximately 18% were treated with high-dose therapy and stem cell transplantation in first remission. In addition to the 4 independent prognostic factors included in the model, the cell proliferation index (Ki-67) was also shown to have additional prognostic relevance. When the Ki67 is available, a biologic MIPI can be calculated.[3] The MIPI is able to classify patients into three risk groups:
  • Low risk (median survival not reached after median 32 months follow-up and 5-year overall survival rate of 60%)
  • Intermediate risk (median survival 51 months)
  • High risk (median survival 29 months)
  • 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.[4]

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. Hoster E, Dreyling M, Klapper W, et al. (January 2008). "A new prognostic index (MIPI) for patients with advanced-stage mantle cell lymphoma". Blood. 111 (2): 558–65. doi:10.1182/blood-2007-06-095331. PMID 17962512.
  4. 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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