Anaplastic large cell lymphoma, ALK negative: Difference between revisions
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== Treatment == | == Treatment == | ||
Although the [[peripheral T-cell lymphomas]] are a heterogenous group of pathologies, the treatment is the same<ref name="pmid24615779">{{cite journal| author=Moskowitz AJ, Lunning MA, Horwitz SM| title=How I treat the peripheral T-cell lymphomas. | journal=Blood | year= 2014 | volume= 123 | issue= 17 | pages= 2636-44 | pmid=24615779 | doi=10.1182/blood-2013-12-516245 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24615779 }} </ref>: | |||
====CHOP Regimen=== | |||
*This regimen includes: | |||
**[[Cyclophosphamide]] | |||
**[[Doxorubicin]] | |||
**[[Vincristine]] | |||
**[[Prednisone]] | |||
This regimen in then followed by a autologous stem cell transplant during remission.<ref name="pmid24615779">{{cite journal| author=Moskowitz AJ, Lunning MA, Horwitz SM| title=How I treat the peripheral T-cell lymphomas. | journal=Blood | year= 2014 | volume= 123 | issue= 17 | pages= 2636-44 | pmid=24615779 | doi=10.1182/blood-2013-12-516245 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24615779 }} </ref> | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 21:55, 20 February 2015
Template:DiseaseDisorder infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alberto Plate [2]
Synonyms and keywords: ALCL-ALK(-); ALK-negative ALCL; ALK negative ALCL; ALK negative anaplastic large cell lymphoma
Overview
The ALK negative anaplastic large cell lymphoma is a peripheral T-cell lymphoma Non-Hodgkin's lymphoma. ALK negative ALCL T-cells express CD30, but not the ALK (Anaplastic Lymphoma Kinase) chimeric protein,[1] reason why the clinical outcome is more variable than the ALK(+)-ALCL.[2] Instead, this T-cells have a chromosomal rearrangement, affecting DUSP22 and TP63 gene. ALK(-) patients with DUSP22 mutation have shown to have a higher five-year overall survival rate in comparison to ALK(+)-ALCL.[3]
Historical Perspective
The WHO added the ALK(-) ALCL as a provisional entity since 2008 in the peripheral T-cell lymphoma classification.[4]
Classification
Morphologic Classification
Pathophysiology
Causes
Differential Diagnosis
- Peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS)[5][6]
- Classical Hodgkin's lymphoma[5]
- Primary cutaneous anaplastic large cell lymphoma[1]
- Anaplastic large cell lymphoma, ALK positive[6]
Epidemiology and Demographics
The ALK(-) ALCL represents 2%-3% of all NHL and 12% of all T-cell Non-Hodgkin's lymphomas.[4]
Risk Factors
- Breast implants[5]
Natural History, Complications and Prognosis
Prognosis
Diagnosis
History and Symptoms
Laboratory Findings
Treatment
Although the peripheral T-cell lymphomas are a heterogenous group of pathologies, the treatment is the same[7]:
=CHOP Regimen
- This regimen includes:
This regimen in then followed by a autologous stem cell transplant during remission.[7]
References
- ↑ 1.0 1.1 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ Xing X, Feldman AL (2015). "Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous". Adv Anat Pathol. 22 (1): 29–49. doi:10.1097/PAP.0000000000000047. PMID 25461779.
- ↑ Parrilla Castellar ER, Jaffe ES, Said JW, Swerdlow SH, Ketterling RP, Knudson RA; et al. (2014). "ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes". Blood. 124 (9): 1473–80. doi:10.1182/blood-2014-04-571091. PMC 4148769. PMID 24894770.
- ↑ 4.0 4.1 "Anaplastic large cell lymphoma, ALK-negative".
- ↑ 5.0 5.1 5.2 Ferreri AJ, Govi S, Pileri SA, Savage KJ (2013). "Anaplastic large cell lymphoma, ALK-negative". Crit Rev Oncol Hematol. 85 (2): 206–15. doi:10.1016/j.critrevonc.2012.06.004. PMID 22789917.
- ↑ 6.0 6.1 "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".
- ↑ 7.0 7.1 Moskowitz AJ, Lunning MA, Horwitz SM (2014). "How I treat the peripheral T-cell lymphomas". Blood. 123 (17): 2636–44. doi:10.1182/blood-2013-12-516245. PMID 24615779.