Mantle cell lymphoma natural history, complications and prognosis
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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):
| 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
- ↑ 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) - ↑ 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) - ↑ 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).