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{{Anaplastic large cell lymphoma}}
{{Anaplastic large cell lymphoma}}
{{CMG}}
{{CMG}}; {{AE}} {{AS}}
==Overview==
The  ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis.
==Prognosis==
==Prognosis==
Those with ALK positivity have a better [[prognosis]]. It is possible that ALK-negative anaplastic large cell lymphomas represent other T-cell lymphomas that are morphologic mimics of ALCL in a final common pathway of disease progression.  It is possible that existing systems of classification will be revised in the future to exclude such lymphomas from this specific diagnosis.
Those with ALK positivity have a better [[prognosis]]. It is possible that ALK-negative anaplastic large cell lymphomas represent other T-cell lymphomas that are morphologic mimics of ALCL in a final common pathway of disease progression.  
* Overall better prognosis than other "Aggressive Lymphomas"
* Overall better prognosis than other "Aggressive Lymphomas"
** ALK+ 5-year survival 70-80%
* ALK+ 5-year survival 70-80%
** ALK- 5-year survival 30-40%
* ALK- 5-year survival 30-40%


ALK positive anaplastic large cell lymphoma affects primarily young (between 10 and 29 years), male patients<ref name="pmid11090048">{{cite journal| author=Stein H, Foss HD, Dürkop H, Marafioti T, Delsol G, Pulford K et al.| title=CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features. | journal=Blood | year= 2000 | volume= 96 | issue= 12 | pages= 3681-95 | pmid=11090048 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11090048  }} </ref> and accounts for 3% of all [[NHL]], 40% of all large cell lymphomas<ref>{{cite web|url=http://www.bloodjournal.org/content/93/8/2697?sso-checked=true|title=ALK+ Lymphoma: Clinico-Pathological Findings and Outcome}}</ref> and 10%-20% of childhood lymphomas.<ref>{{cite web|url=http://www.bloodjournal.org/content/93/8/2697?sso-checked=true|title=ALK+ Lymphoma: Clinico-Pathological Findings and Outcome}}</ref>
* The '''I'''nternational '''P'''rognostic '''I'''index (IPI) is used to estimate the prognosis of patients.<ref>{{cite web|url=http://www.uptodate.com/contents/image?imageKey=HEME%2F70850&topicKey=HEME%2F4705&rank=1%7E150&source=see_link&search=Anaplastic+large+cell+lymphoma%2C+ALK+positive&utdPopup=true|title=International Prognostic Index for non-Hodgkin lymphoma}}</ref> The IPI takes into account 5 variables:
:* Patient's age (>60 years)
:* Elevated serum [[lactate dehydrogenase]] ([[LDH]])
:* Eastern Cooperative Oncology Group (ECOG) performance status
:* Ann Arbor clinical stage III or IV
:* Number of involved extra nodal sites > 1


According to a study on 1,320 cases of [[peripheral T-cell lymphomas]] and NK cell lymphomas between 1990 and 2002 in 22 different centers, ALK-Positive ALCL is the fifth most common type of peripheral T cell lymphoma (6.6% of total patients).<ref>{{cite web|url=http://jco.ascopubs.org/content/26/25/4124.full.pdf|title=International Peripheral T-Cell and Natural Killer/T-Cell Lymphoma Study: Pathology Findings and Clinical Outcomes}}</ref>  In the United states, ALK-Positive ALCL is the most frequent type of [[peripheral T-cell lymphoma]].
* If any of this criteria is met, one point is awarded for the IPI. The interpretation of the total score is as follows:
:* 0 to 1: Low risk
:* 2: Low-intermediate risk
:* 3: High-intermediate risk
:* 4 to 5: High risk


===Prognosis===
* According to the International Peripheral T cell Lymphoma Project, the estimated 5-years survival for each of the IPI stages are as follows:<ref>{{cite web|url=http://www.bloodjournal.org/content/111/12/5496.abstract?sso-checked=true|title=ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}}</ref>
The '''I'''nternational '''P'''rognostic '''I'''index (IPI) is used to estimate the prognosis of patients.<ref>{{cite web|url=http://www.uptodate.com/contents/image?imageKey=HEME%2F70850&topicKey=HEME%2F4705&rank=1%7E150&source=see_link&search=Anaplastic+large+cell+lymphoma%2C+ALK+positive&utdPopup=true|title=International Prognostic Index for non-Hodgkin lymphoma}}</ref> The IPI takes into account 5 variables:
:* Low risk (IPI 0-1): 90%
*Patient's age (>60 years)
:* Low-intermediate risk (IPI 2): 68%
*Elevated serum [[lactate dehydrogenase]] ([[LDH]])
:* High-intermediate risk (IPI 3): 23%
*Eastern Cooperative Oncology Group (ECOG) performance status
:* High risk (IPI 4-5): 33%
*Ann Arbor clinical stage III or IV
*Number of involved extra nodal sites > 1
 
If any of this criteria is met, one point is awarded for the IPI. The interpretation of the total score is as follows:
*0 to 1: Low risk
*2: Low-intermediate risk
*3: High-intermediate risk
*4 to 5: High risk
 
According to the International Peripheral T cell Lymphoma Project, the estimated 5-years survival for each of the IPI stages are as follows:<ref>{{cite web|url=http://www.bloodjournal.org/content/111/12/5496.abstract?sso-checked=true|title=ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}}</ref>
 
*Low risk (IPI 0-1): 90%
*Low-intermediate risk (IPI 2): 68%
*High-intermediate risk (IPI 3): 23%
*High risk (IPI 4-5): 33%
 
However, recently, the International peripheral T-cell lymphoma Project score (IPTCLP) has demonstrated to be the most accurate score to predict overall survival in patients. <ref>{{cite web|url=http://annonc.oxfordjournals.org/content/early/2010/07/14/annonc.mdq359.full.pdf+html|title=Comparison of four prognostic scores in peripheral T-cell lymphoma}}</ref>


* However, recently, the International peripheral T-cell lymphoma Project score (IPTCLP) has demonstrated to be the most accurate score to predict overall survival in patients. <ref>{{cite web|url=http://annonc.oxfordjournals.org/content/early/2010/07/14/annonc.mdq359.full.pdf+html|title=Comparison of four prognostic scores in peripheral T-cell lymphoma}}</ref>
This score includes:
This score includes:
*Age
*Age
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Revision as of 13:46, 19 October 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis.

Prognosis

Those with ALK positivity have a better prognosis. It is possible that ALK-negative anaplastic large cell lymphomas represent other T-cell lymphomas that are morphologic mimics of ALCL in a final common pathway of disease progression.

  • Overall better prognosis than other "Aggressive Lymphomas"
  • ALK+ 5-year survival 70-80%
  • ALK- 5-year survival 30-40%
  • The International Prognostic Iindex (IPI) is used to estimate the prognosis of patients.[1] The IPI takes into account 5 variables:
  • Patient's age (>60 years)
  • Elevated serum lactate dehydrogenase (LDH)
  • Eastern Cooperative Oncology Group (ECOG) performance status
  • Ann Arbor clinical stage III or IV
  • Number of involved extra nodal sites > 1
  • If any of this criteria is met, one point is awarded for the IPI. The interpretation of the total score is as follows:
  • 0 to 1: Low risk
  • 2: Low-intermediate risk
  • 3: High-intermediate risk
  • 4 to 5: High risk
  • According to the International Peripheral T cell Lymphoma Project, the estimated 5-years survival for each of the IPI stages are as follows:[2]
  • Low risk (IPI 0-1): 90%
  • Low-intermediate risk (IPI 2): 68%
  • High-intermediate risk (IPI 3): 23%
  • High risk (IPI 4-5): 33%
  • However, recently, the International peripheral T-cell lymphoma Project score (IPTCLP) has demonstrated to be the most accurate score to predict overall survival in patients. [3]

This score includes:

  • Age
  • Eastern Cooperative Oncology Group (ECOG) performance status
  • Platelet count

References

  1. ↑ "International Prognostic Index for non-Hodgkin lymphoma".
  2. ↑ "ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project".
  3. ↑ "Comparison of four prognostic scores in peripheral T-cell lymphoma".

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