Anaplastic large cell lymphoma, ALK negative: Difference between revisions
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===History and Symptoms=== | ===History and Symptoms=== | ||
Patients typically present [[B symptoms]] ([[fever]], [[weight loss]] and [[lymphadenopathy]]). ALK negative ALCL patients have a higher incidence of cutaneous, hepatic and gastrointestinal involvement that [[ALK positive ALCL]].<ref>{{cite web|url=http://www.bloodjournal.org/content/bloodjournal/111/12/5496.full.pdf|title=ALK negative anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK positive ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}} </ref> Other sites of involvement include the [[bronchus]]<ref name="pmid24427373">{{cite journal| author=Xu X| title=ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 1 | pages= 460-3 | pmid=24427373 | doi= | pmc=PMC3885507 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24427373 }} </ref>, [[central nervous system]],<ref name="pmid24649224">{{cite journal| author=Nomura M, Narita Y, Miyakita Y, Ohno M, Fukushima S, Maruyama T et al.| title=Clinical presentation of anaplastic large-cell lymphoma in the central nervous system. | journal=Mol Clin Oncol | year= 2013 | volume= 1 | issue= 4 | pages= 655-660 | pmid=24649224 | doi=10.3892/mco.2013.110 | pmc=PMC3915681 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24649224 }} </ref> [[pancreas]],<ref name="pmid16273656">{{cite journal| author=Savopoulos CG, Tsesmeli NE, Kaiafa GD, Zantidis AT, Bobos MT, Hatzitolios AI et al.| title=Primary pancreatic anaplastic large cell lymphoma, ALK negative: a case report. | journal=World J Gastroenterol | year= 2005 | volume= 11 | issue= 39 | pages= 6221-4 | pmid=16273656 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16273656 }} </ref> [[rectum]],<ref>{{citeweb|url=http://dx.doi.org/10.4236/ojpathology.2013.31007|title= | Patients typically present [[B symptoms]] ([[fever]], [[weight loss]] and [[lymphadenopathy]]). ALK negative ALCL patients have a higher incidence of cutaneous, hepatic and gastrointestinal involvement that [[ALK positive ALCL]].<ref>{{cite web|url=http://www.bloodjournal.org/content/bloodjournal/111/12/5496.full.pdf|title=ALK negative anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK positive ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}} </ref> Other sites of involvement include the [[bronchus]]<ref name="pmid24427373">{{cite journal| author=Xu X| title=ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 1 | pages= 460-3 | pmid=24427373 | doi= | pmc=PMC3885507 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24427373 }} </ref>, [[central nervous system]],<ref name="pmid24649224">{{cite journal| author=Nomura M, Narita Y, Miyakita Y, Ohno M, Fukushima S, Maruyama T et al.| title=Clinical presentation of anaplastic large-cell lymphoma in the central nervous system. | journal=Mol Clin Oncol | year= 2013 | volume= 1 | issue= 4 | pages= 655-660 | pmid=24649224 | doi=10.3892/mco.2013.110 | pmc=PMC3915681 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24649224 }} </ref> [[pancreas]],<ref name="pmid16273656">{{cite journal| author=Savopoulos CG, Tsesmeli NE, Kaiafa GD, Zantidis AT, Bobos MT, Hatzitolios AI et al.| title=Primary pancreatic anaplastic large cell lymphoma, ALK negative: a case report. | journal=World J Gastroenterol | year= 2005 | volume= 11 | issue= 39 | pages= 6221-4 | pmid=16273656 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16273656 }} </ref> [[rectum]],<ref>{{citeweb|url=http://dx.doi.org/10.4236/ojpathology.2013.31007|title= | ||
Anaplastic Large Cell Lymphoma, ALK-Negative Presenting in the Rectum: A Case Report and Review of the Literature}}</ref> [[breast]] peri-implant | Anaplastic Large Cell Lymphoma, ALK-Negative Presenting in the Rectum: A Case Report and Review of the Literature}}</ref> [[breast]] peri-implant seromas,<ref name="pmid25490535">{{cite journal| author=Brody GS, Deapen D, Taylor CR, Pinter-Brown L, House-Lightner SR, Andersen J et al.| title=Anaplastic Large Cell Lymphoma (ALCL) Occuring in Women with Breast Implants: Analysis of 173 Cases. | journal=Plast Reconstr Surg | year= 2014 | volume= | issue= | pages= | pmid=25490535 | doi=10.1097/PRS.0000000000001033 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25490535 }} </ref> [[skeletal muscle]],<ref name="pmid25292453">{{cite journal| author=Kubo Y, Aoi J, Johno T, Makino T, Sakai K, Masuguchi S et al.| title=A case of anaplastic large cell lymphoma of skeletal muscle. | journal=J Dermatol | year= 2014 | volume= 41 | issue= 11 | pages= 999-1002 | pmid=25292453 | doi=10.1111/1346-8138.12641 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25292453 }} </ref> and [[bone]].<ref name="pmid25346119">{{cite journal| author=Yu G, Huang X, Li M, Ding Y, Wang X, Lai Y et al.| title=[Clinicopathologic features and prognosis of primary bone anaplastic large cell lymphoma]. | journal=Zhonghua Bing Li Xue Za Zhi | year= 2014 | volume= 43 | issue= 8 | pages= 512-5 | pmid=25346119 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25346119 }} </ref> | ||
===Laboratory Findings=== | ===Laboratory Findings=== |
Revision as of 18:37, 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
History and Symptoms
Patients typically present B symptoms (fever, weight loss and lymphadenopathy). ALK negative ALCL patients have a higher incidence of cutaneous, hepatic and gastrointestinal involvement that ALK positive ALCL.[8] Other sites of involvement include the bronchus[9], central nervous system,[10] pancreas,[11] rectum,[12] breast peri-implant seromas,[13] skeletal muscle,[14] and bone.[15]
Laboratory Findings
According to the World Health Organization (WHO), the most important factor to diagnose a ALK negative ALCL is morphology and immunophenotype:[16]
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:[17]
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[18], reason why CHOP regimen must then be followed by an autologous stem cell transplant during remission.[17]
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".
- ↑ "ALK negative anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK positive ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project" (PDF).
- ↑ Xu X (2014). "ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review". Int J Clin Exp Pathol. 7 (1): 460–3. PMC 3885507. PMID 24427373.
- ↑ Nomura M, Narita Y, Miyakita Y, Ohno M, Fukushima S, Maruyama T; et al. (2013). "Clinical presentation of anaplastic large-cell lymphoma in the central nervous system". Mol Clin Oncol. 1 (4): 655–660. doi:10.3892/mco.2013.110. PMC 3915681. PMID 24649224.
- ↑ Savopoulos CG, Tsesmeli NE, Kaiafa GD, Zantidis AT, Bobos MT, Hatzitolios AI; et al. (2005). "Primary pancreatic anaplastic large cell lymphoma, ALK negative: a case report". World J Gastroenterol. 11 (39): 6221–4. PMID 16273656.
- ↑ Template:Citeweb
- ↑ Brody GS, Deapen D, Taylor CR, Pinter-Brown L, House-Lightner SR, Andersen J; et al. (2014). "Anaplastic Large Cell Lymphoma (ALCL) Occuring in Women with Breast Implants: Analysis of 173 Cases". Plast Reconstr Surg. doi:10.1097/PRS.0000000000001033. PMID 25490535.
- ↑ Kubo Y, Aoi J, Johno T, Makino T, Sakai K, Masuguchi S; et al. (2014). "A case of anaplastic large cell lymphoma of skeletal muscle". J Dermatol. 41 (11): 999–1002. doi:10.1111/1346-8138.12641. PMID 25292453.
- ↑ Yu G, Huang X, Li M, Ding Y, Wang X, Lai Y; et al. (2014). "[Clinicopathologic features and prognosis of primary bone anaplastic large cell lymphoma]". Zhonghua Bing Li Xue Za Zhi. 43 (8): 512–5. PMID 25346119.
- ↑ "Anaplastic large cell lymphoma: changes in the World Health Organization classification and perspectives for targeted therapy".
- ↑ 17.0 17.1 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.