Anaplastic large cell lymphoma natural history, complications and prognosis: Difference between revisions
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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]]. | 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]]. | ||
===Prognosis=== | |||
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 | |||
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> | |||
This score includes: | |||
*Age | |||
*Eastern Cooperative Oncology Group (ECOG) performance status | |||
*Platelet count | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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.
- Overall better prognosis than other "Aggressive Lymphomas"
- ALK+ 5-year survival 70-80%
- ALK- 5-year survival 30-40%
ALK positive anaplastic large cell lymphoma affects primarily young (between 10 and 29 years), male patients[1] and accounts for 3% of all NHL, 40% of all large cell lymphomas[2] and 10%-20% of childhood lymphomas.[3]
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).[4] In the United states, ALK-Positive ALCL is the most frequent type of peripheral T-cell lymphoma.
Prognosis
The International Prognostic Iindex (IPI) is used to estimate the prognosis of patients.[5] 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:[6]
- 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. [7]
This score includes:
- Age
- Eastern Cooperative Oncology Group (ECOG) performance status
- Platelet count
References
- ↑ Stein H, Foss HD, Dürkop H, Marafioti T, Delsol G, Pulford K; et al. (2000). "CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features". Blood. 96 (12): 3681–95. PMID 11090048.
- ↑ "ALK+ Lymphoma: Clinico-Pathological Findings and Outcome".
- ↑ "ALK+ Lymphoma: Clinico-Pathological Findings and Outcome".
- ↑ "International Peripheral T-Cell and Natural Killer/T-Cell Lymphoma Study: Pathology Findings and Clinical Outcomes" (PDF).
- ↑ "International Prognostic Index for non-Hodgkin lymphoma".
- ↑ "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".
- ↑ "Comparison of four prognostic scores in peripheral T-cell lymphoma".