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


{{CMG}}; {{AE}} {{Akram}} {{AS}}
{{CMG}}; {{AE}} {{Akram}} {{AS}}


==Overview==
==Overview==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Prognosis is generally poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
 
OR
 
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
 
OR
 
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==


===Natural History===
===Natural History===
*The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
*Mantle cell lymphoma is generally considered to be an aggressive disease with patients having early relapses and poor long-term survival rates.  
*The symptoms of (disease name) typically develop ___ years after exposure to ___.  
*However, rare forms of the disease which show an indolent behavior, have better outcomes.  
*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].


===Complications===
===Complications===

Revision as of 17:41, 21 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

Prognosis is generally poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

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.

Complications

  • Common complications of [disease name] include:
    • [Complication 1]
    • [Complication 2]
    • [Complication 3]

Prognosis

  • Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
  • Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
  • The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
  • [Subtype of disease/malignancy] is associated with the most favorable prognosis.
  • The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.


Overview

Prognosis is generally poor, and the 5-year survival rate of patients with mantle cell lymphoma is approximately 50-70%. Depending on the cell type of the tumor, the prognosis may vary.

Prognosis

  • The 5-year survival rate of patients with mantle cell lymphoma is approximately 50%[1] (advanced stage mantle cell lymphoma) to 70%[2] (for limited-stage mantle cell lymphoma).
  • 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. Most recent values in: Herrmann A, Hoster E, Zwingers T; et al. (February 2009). "Improvement of overall survival in advanced stage mantle cell lymphoma". J. Clin. Oncol. 27 (4): 511–8. doi:10.1200/JCO.2008.16.8435. PMID 19075279.
  2. Leitch HA, Gascoyne RD, Chhanabhai M, Voss NJ, Klasa R, Connors JM (October 2003). "Limited-stage mantle-cell lymphoma". Ann. Oncol. 14 (10): 1555–61. doi:10.1093/annonc/mdg414. PMID 14504058.
  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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