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.[3]
- Presence of blastoid or pleomorphic morphologic characteristics have also shown to have poor outcomes.[4]
- Other markers which have shown to adversely affect prognosis are as follows:
- High expression of eukaryotic initiation factor 4E
- Myc overexpression
- Increased expression of SOX11
- Low TCL1 expression
- TP53 deletion
- Lack of hypermutated heavy chain immunoglobulin variable regions
- Increased absolute levels of the following in the serum:
- monocytes
- beta-2 microglobulin
- monoclonal and polyclonal immunoglobulin free light chain
- IL-2Rα
- IL-8
- MIP-1β.
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) - ↑ Eva Hoster, Andreas Rosenwald, Francoise Berger, Heinz-Wolfram Bernd, Sylvia Hartmann, Christoph Loddenkemper, Thomas F. E. Barth, Nicole Brousse, Stefano Pileri, Grzegorz Rymkiewicz, Roman Kodet, Stephan Stilgenbauer, Roswitha Forstpointner, Catherine Thieblemont, Michael Hallek, Bertrand Coiffier, Ursula Vehling-Kaiser, Reda Bouabdallah, Lothar Kanz, Michael Pfreundschuh, Christian Schmidt, Vincent Ribrag, Wolfgang Hiddemann, Michael Unterhalt, Johanna C. Kluin-Nelemans, Olivier Hermine, Martin H. Dreyling & Wolfram Klapper (2016). "Prognostic Value of Ki-67 Index, Cytology, and Growth Pattern in Mantle-Cell Lymphoma: Results From Randomized Trials of the European Mantle Cell Lymphoma Network". Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 34 (12): 1386–1394. doi:10.1200/JCO.2015.63.8387. PMID 26926679. Unknown parameter
|month=ignored (help) - ↑ German Ott, Andreas Rosenwald & Elias Campo (2013). "Understanding MYC-driven aggressive B-cell lymphomas: pathogenesis and classification". Hematology. American Society of Hematology. Education Program. 2013: 575–583. doi:10.1182/asheducation-2013.1.575. PMID 24319234.