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 levels, WBC Count):
| Points | Age (years) | ECOG | LDH (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:[5][6][7][8][9][10][11][12]
- 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:
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.
- ↑ Veronica Fernandez, Olga Salamero, Blanca Espinet, Francesc Sole, Cristina Royo, Alba Navarro, Francisca Camacho, Silvia Bea, Elena Hartmann, Virginia Amador, Luis Hernandez, Claudio Agostinelli, Rachel L. Sargent, Maria Rozman, Marta Aymerich, Dolors Colomer, Neus Villamor, Steven H. Swerdlow, Stefano A. Pileri, Francesc Bosch, Miguel A. Piris, Emili Montserrat, German Ott, Andreas Rosenwald, Armando Lopez-Guillermo, Pedro Jares, Sergi Serrano & Elias Campo (2010). "Genomic and gene expression profiling defines indolent forms of mantle cell lymphoma". Cancer research. 70 (4): 1408–1418. doi:10.1158/0008-5472.CAN-09-3419. PMID 20124476. Unknown parameter
|month=ignored (help) - ↑ Mohamad B. Sonbol, Matthew J. Maurer, Mary J. Stenson, Cristine Allmer, Betsy R. LaPlant, George J. Weiner, William R. Macon, James R. Cerhan, Thomas E. Witzig & Mamta Gupta (2014). "Elevated soluble IL-2Ralpha, IL-8, and MIP-1beta levels are associated with inferior outcome and are independent of MIPI score in patients with mantle cell lymphoma". American journal of hematology. 89 (12): E223–E227. doi:10.1002/ajh.23838. PMID 25164110. Unknown parameter
|month=ignored (help) - ↑ Julia Slotta-Huspenina, Ina Koch, Laurence de Leval, Gisela Keller, Margit Klier, Karin Bink, Marcus Kremer, Mark Raffeld, Falko Fend & Leticia Quintanilla-Martinez (2012). "The impact of cyclin D1 mRNA isoforms, morphology and p53 in mantle cell lymphoma: p53 alterations and blastoid morphology are strong predictors of a high proliferation index". Haematologica. 97 (9): 1422–1430. doi:10.3324/haematol.2011.055715. PMID 22315488. Unknown parameter
|month=ignored (help) - ↑ M. Meggendorfer, W. Kern, C. Haferlach, T. Haferlach & S. Schnittger (2013). "SOX11 overexpression is a specific marker for mantle cell lymphoma and correlates with t(11;14) translocation, CCND1 expression and an adverse prognosis". Leukemia. 27 (12): 2388–2391. doi:10.1038/leu.2013.141. PMID 23648671. Unknown parameter
|month=ignored (help) - ↑ Thomas E. Witzig, Matthew J. Maurer, Thomas M. Habermann, Brian K. Link, Ivana N. M. Micallef, Grzegorz S. Nowakowski, Stephen M. Ansell, Joseph P. Colgan, David J. Inwards, Luis F. Porrata, Svetomir N. Markovic, Patrick B. Johnston, Yi Lin, Carrie Thompson, Mamta Gupta, Jerry A. Katzmann & James R. Cerhan (2014). "Elevated monoclonal and polyclonal serum immunoglobulin free light chain as prognostic factors in B- and T-cell non-Hodgkin lymphoma". American journal of hematology. 89 (12): 1116–1120. doi:10.1002/ajh.23839. PMID 25228125. Unknown parameter
|month=ignored (help) - ↑ Changhoon Yoo, Dok Hyun Yoon, Shin Kim, Jooryung Huh, Chan-Sik Park, Chan-Jeong Park, Sang-Wook Lee & Cheolwon Suh (2016). "Serum beta-2 microglobulin as a prognostic biomarker in patients with mantle cell lymphoma". Hematological oncology. 34 (1): 22–27. doi:10.1002/hon.2188. PMID 25689467. Unknown parameter
|month=ignored (help) - ↑ Ji-Young Choe, Ji Yun Yun, Hee Young Na, Jooryung Huh, Su-Jin Shin, Hyun-Jung Kim, Jin Ho Paik, Young A. Kim, Soo Jeong Nam, Yoon Kyung Jeon, Gyeongsin Park & Ji Eun Kim (2016). "MYC overexpression correlates with MYC amplification or translocation, and is associated with poor prognosis in mantle cell lymphoma". Histopathology. 68 (3): 442–449. doi:10.1111/his.12760. PMID 26100211. Unknown parameter
|month=ignored (help) - ↑ Su-Jin Shin, Jin Roh, Hee Jeong Cha, Yoo Duk Choi, Jin-Man Kim, Soo Kee Min, Ji Eun Kim, Dae-Woon Eom, Hojung Lee, Hyun-Jung Kim, Dok Hyun Yoon, Cheolwon Suh & Jooryung Huh (2015). "TCL1 expression predicts overall survival in patients with mantle cell lymphoma". European journal of haematology. 95 (6): 583–594. doi:10.1111/ejh.12539. PMID 25688912. Unknown parameter
|month=ignored (help)