Anaplastic large cell lymphoma, ALK negative: Difference between revisions
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== Epidemiology and Demographics== | == Epidemiology and Demographics== | ||
The ALK(-) ALCL represents 2%-3% of all [[NHL]] and 12% of all [[T-cell]] [[Non-Hodgkin's lymphomas]].<ref name="ALK">{{cite web|url=http://www.sciencedirect.com/science/article/pii/S104084281200131X|title=Anaplastic large cell lymphoma, ALK-negative}}</ref> | The ALK(-) ALCL represents 2%-3% of all [[NHL]] and 12% of all [[T-cell]] [[Non-Hodgkin's lymphomas]].<ref name="ALK">{{cite web|url=http://www.sciencedirect.com/science/article/pii/S104084281200131X|title=Anaplastic large cell lymphoma, ALK-negative}}</ref> Affects primarily adults between 40-60 years old, with a modest male predominance, in comparison to women.<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref> | ||
==Risk Factors== | ==Risk Factors== | ||
Revision as of 16:38, 23 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] Affects primarily adults between 40-60 years old, with a modest male predominance, in comparison to women.[1]
Risk Factors
- Breast implants[5]
Natural History, Complications and Prognosis
Prognosis
The International Prognostic Iindex (IPI) is used to estimate the prognosis of patients.[7] 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
Diagnosis
Laboratory Findings
According to the World Health Organization (WHO), the most important factor to diagnose a ALK negative ALCL is morphology and immunophenotype:[8]
Morphologic criteria
- Absence of small-to-medium sized lymphocytes.
Immunophenotype criteria
- CD30 expression
- Nuclear negativity for the PAX5 transcription factor (usually expressed in Hodgkin’s lymphoma classic variant)
- Negativity for the EBV markers EBER and LMP1 (which may be expressed in Hodgkin’s lymphoma classic variant)
- Presence of clonal T-cell receptor rearrangements (usually absent in Hodgkin’s lymphoma classic variant).
Treatment
Although the peripheral T-cell lymphomas are a heterogenous group of pathologies, the treatment is the same:[9]
CHOP Regimen
- This regimen includes:
Some evidence suggest that although CHOP regimen is effective in treating the ALK(-) ALCL, a short 2-year event-free survival requires extra management[10], reason why CHOP regimen must then be followed by an autologous stem cell transplant during remission.[9]
References
- ↑ 1.0 1.1 1.2 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".
- ↑ "International Prognostic Index for non-Hodgkin lymphoma".
- ↑ "Anaplastic large cell lymphoma: changes in the World Health Organization classification and perspectives for targeted therapy".
- ↑ 9.0 9.1 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.
- ↑ Rattarittamrong E, Norasetthada L, Tantiworawit A, Chai-Adisaksopha C, Nawarawong W (2013). "CHOEP-21 chemotherapy for newly diagnosed nodal peripheral T-cell lymphomas (PTCLs) in Maharaj Nakorn Chiang Mai Hospital". J Med Assoc Thai. 96 (11): 1416–22. PMID 24428090.