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Primary Systemic Anaplastic Large Cell Lymphoma, ALK-Positive

MedGen UID:
233347
Concept ID:
C1335492
Neoplastic Process
Synonyms: Primary Systemic ALK-Positive ALCL; Primary Systemic ALK-Positive Anaplastic Large Cell Lymphoma; Primary Systemic ALKoma

Definition

A T-cell peripheral lymphoma affecting multiple anatomic sites. It is composed of usually large, pleomorphic, CD30 positive T-lymphocytes with abundant cytoplasm. It is characterized by the presence of a translocation involving the ALK gene and expression of ALK fusion protein. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Systemic Anaplastic Large Cell Lymphoma, ALK-Positive

Professional guidelines

PubMed

Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Boccalatte FE, Voena C, Riganti C, Bosia A, D'Amico L, Riera L, Cheng M, Ruggeri B, Jensen ON, Goss VL, Lee K, Nardone J, Rush J, Polakiewicz RD, Comb MJ, Chiarle R, Inghirami G
Blood 2009 Mar 19;113(12):2776-90. Epub 2008 Oct 9 doi: 10.1182/blood-2008-06-161018. PMID: 18845790Free PMC Article
ten Berge RL, Oudejans JJ, Ossenkoppele GJ, Meijer CJ
J Pathol 2003 May;200(1):4-15. doi: 10.1002/path.1331. PMID: 12692835

Recent clinical studies

Etiology

Ahrendsen JT, Ta R, Li J, Weinberg OK, Ferry JA, Hasserjian RP, Meredith DM, Varma H, Sadigh S, Michaels PD
Am J Clin Pathol 2022 Aug 4;158(2):300-310. doi: 10.1093/ajcp/aqac046. PMID: 35460414
Pang Y, Chihara D
Semin Hematol 2021 Apr;58(2):123-129. Epub 2021 Mar 3 doi: 10.1053/j.seminhematol.2021.02.003. PMID: 33906722
Amador C, Feldman AL
Am J Clin Pathol 2021 Mar 15;155(4):479-497. doi: 10.1093/ajcp/aqab012. PMID: 33686426
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Xing X, Feldman AL
Adv Anat Pathol 2015 Jan;22(1):29-49. doi: 10.1097/PAP.0000000000000047. PMID: 25461779

Diagnosis

Ahrendsen JT, Ta R, Li J, Weinberg OK, Ferry JA, Hasserjian RP, Meredith DM, Varma H, Sadigh S, Michaels PD
Am J Clin Pathol 2022 Aug 4;158(2):300-310. doi: 10.1093/ajcp/aqac046. PMID: 35460414
Brivio E, Baruchel A, Beishuizen A, Bourquin JP, Brown PA, Cooper T, Gore L, Kolb EA, Locatelli F, Maude SL, Mussai FJ, Vormoor-Bürger B, Vormoor J, von Stackelberg A, Zwaan CM
Eur J Cancer 2022 Mar;164:1-17. Epub 2022 Feb 1 doi: 10.1016/j.ejca.2021.12.029. PMID: 35121370
Amador C, Feldman AL
Am J Clin Pathol 2021 Mar 15;155(4):479-497. doi: 10.1093/ajcp/aqab012. PMID: 33686426
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
ten Berge RL, Oudejans JJ, Ossenkoppele GJ, Meijer CJ
J Pathol 2003 May;200(1):4-15. doi: 10.1002/path.1331. PMID: 12692835

Therapy

Brugières L, Cozic N, Houot R, Rigaud C, Sibon D, Arfi-Rouche J, Bories P, Cottereau AS, Delmer A, Ducassou S, Garnier N, Lamant L, Leruste A, Millot F, Moalla S, Morschhauser F, Nolla M, Pagnier A, Reguerre Y, Renaud L, Schmitt A, Simonin M, Verschuur A, Hoog Labouret N, Mahier Ait Oukhatar C, Vassal G
Eur J Cancer 2023 Sep;191:112984. Epub 2023 Jul 17 doi: 10.1016/j.ejca.2023.112984. PMID: 37549532
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Cao Q, Liu F, Li S, Liu N, Li L, Li C, Peng T
Diagn Pathol 2016 Sep 9;11(1):83. doi: 10.1186/s13000-016-0539-6. PMID: 27612448Free PMC Article
Savage KJ
Hematology Am Soc Hematol Educ Program 2008:280-8. doi: 10.1182/asheducation-2008.1.280. PMID: 19074097
Fiorani C, Vinci G, Sacchi S, Bonaccorsi G, Artusi T
Clin Lymphoma 2001 Jun;2(1):29-37; discussion 38-9. doi: 10.3816/clm.2001.n.009. PMID: 11707867

Prognosis

Ahrendsen JT, Ta R, Li J, Weinberg OK, Ferry JA, Hasserjian RP, Meredith DM, Varma H, Sadigh S, Michaels PD
Am J Clin Pathol 2022 Aug 4;158(2):300-310. doi: 10.1093/ajcp/aqac046. PMID: 35460414
Brivio E, Baruchel A, Beishuizen A, Bourquin JP, Brown PA, Cooper T, Gore L, Kolb EA, Locatelli F, Maude SL, Mussai FJ, Vormoor-Bürger B, Vormoor J, von Stackelberg A, Zwaan CM
Eur J Cancer 2022 Mar;164:1-17. Epub 2022 Feb 1 doi: 10.1016/j.ejca.2021.12.029. PMID: 35121370
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Noh BJ, Han CS, Park JS, Lee J, Kim YW, Park YK
Malays J Pathol 2018 Aug;40(2):161-167. PMID: 30173234
Xing X, Feldman AL
Adv Anat Pathol 2015 Jan;22(1):29-49. doi: 10.1097/PAP.0000000000000047. PMID: 25461779

Clinical prediction guides

Ahrendsen JT, Ta R, Li J, Weinberg OK, Ferry JA, Hasserjian RP, Meredith DM, Varma H, Sadigh S, Michaels PD
Am J Clin Pathol 2022 Aug 4;158(2):300-310. doi: 10.1093/ajcp/aqac046. PMID: 35460414
Gru AA, Bhagat G, Subtil A, Raghavan SS, Pulitzer M, Chung C, Sangueza M, Plaza JA
Am J Surg Pathol 2021 Jun 1;45(6):796-802. doi: 10.1097/PAS.0000000000001623. PMID: 33234878Free PMC Article
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Menon MP, Nicolae A, Meeker H, Raffeld M, Xi L, Jegalian AG, Miller DC, Pittaluga S, Jaffe ES
Am J Surg Pathol 2015 Dec;39(12):1719-1729. doi: 10.1097/PAS.0000000000000503. PMID: 26379152Free PMC Article
Costello R, Sanchez C, Le Treut T, Rihet P, Imbert J, Sébahoun G
Br J Haematol 2010 Jul;150(1):21-7. Epub 2009 Nov 12 doi: 10.1111/j.1365-2141.2009.07977.x. PMID: 19912218

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