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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; Primary Systemic Anaplastic Large Cell Lymphoma, ALK-Positive

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  
  • CROGVSystemic 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
Chihara D, Fanale MA
Hematol Oncol Clin North Am 2017 Apr;31(2):209-222. doi: 10.1016/j.hoc.2016.11.001. PMID: 28340874
Dagogo-Jack I, Shaw AT, Riely GJ
Clin Pharmacol Ther 2017 May;101(5):625-633. Epub 2017 Mar 17 doi: 10.1002/cpt.653. PMID: 28182271

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
Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project
Blood 2014 May 8;123(19):2915-23. Epub 2014 Mar 14 doi: 10.1182/blood-2013-11-536359. PMID: 24632715Free PMC Article
Ferreri AJ, Govi S, Pileri SA, Savage KJ
Crit Rev Oncol Hematol 2012 Aug;83(2):293-302. Epub 2012 Mar 21 doi: 10.1016/j.critrevonc.2012.02.005. PMID: 22440390
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, Peter N, Loeffler M, Rosenwald A, Pfreundschuh M
Blood 2010 Nov 4;116(18):3418-25. Epub 2010 Jul 21 doi: 10.1182/blood-2010-02-270785. PMID: 20660290
Vose J, Armitage J, Weisenburger D; International T-Cell Lymphoma Project
J Clin Oncol 2008 Sep 1;26(25):4124-30. Epub 2008 Jul 14 doi: 10.1200/JCO.2008.16.4558. PMID: 18626005

Diagnosis

Rigaud C, Knörr F, Brugières L, Woessmann W
Best Pract Res Clin Haematol 2023 Mar;36(1):101444. Epub 2023 Feb 2 doi: 10.1016/j.beha.2023.101444. PMID: 36907641
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
Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project
Blood 2014 May 8;123(19):2915-23. Epub 2014 Mar 14 doi: 10.1182/blood-2013-11-536359. PMID: 24632715Free PMC Article
Minoo P, Wang HY
Int J Clin Exp Pathol 2012;5(5):397-410. Epub 2012 May 23 PMID: 22808292Free PMC Article
Vose J, Armitage J, Weisenburger D; International T-Cell Lymphoma Project
J Clin Oncol 2008 Sep 1;26(25):4124-30. Epub 2008 Jul 14 doi: 10.1200/JCO.2008.16.4558. PMID: 18626005

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
Zhao S, Li J, Xia Q, Liu K, Dong Z
Oncogene 2023 Jun;42(24):1959-1969. Epub 2023 May 6 doi: 10.1038/s41388-023-02712-8. PMID: 37149665
Chihara D, Fanale MA
Hematol Oncol Clin North Am 2017 Apr;31(2):209-222. doi: 10.1016/j.hoc.2016.11.001. PMID: 28340874
Minoo P, Wang HY
Int J Clin Exp Pathol 2012;5(5):397-410. Epub 2012 May 23 PMID: 22808292Free PMC Article
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, Peter N, Loeffler M, Rosenwald A, Pfreundschuh M
Blood 2010 Nov 4;116(18):3418-25. Epub 2010 Jul 21 doi: 10.1182/blood-2010-02-270785. PMID: 20660290

Prognosis

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
Chihara D, Fanale MA
Hematol Oncol Clin North Am 2017 Apr;31(2):209-222. doi: 10.1016/j.hoc.2016.11.001. PMID: 28340874
Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project
Blood 2014 May 8;123(19):2915-23. Epub 2014 Mar 14 doi: 10.1182/blood-2013-11-536359. PMID: 24632715Free PMC Article
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, Peter N, Loeffler M, Rosenwald A, Pfreundschuh M
Blood 2010 Nov 4;116(18):3418-25. Epub 2010 Jul 21 doi: 10.1182/blood-2010-02-270785. PMID: 20660290
Vose J, Armitage J, Weisenburger D; International T-Cell Lymphoma Project
J Clin Oncol 2008 Sep 1;26(25):4124-30. Epub 2008 Jul 14 doi: 10.1200/JCO.2008.16.4558. PMID: 18626005

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
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Bonzheim I, Steinhilber J, Fend F, Lamant L, Quintanilla-Martinez L
Front Biosci (Schol Ed) 2015 Jun 1;7(2):248-59. doi: 10.2741/S438. PMID: 25961700
Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project
Blood 2014 May 8;123(19):2915-23. Epub 2014 Mar 14 doi: 10.1182/blood-2013-11-536359. PMID: 24632715Free PMC Article
Ferreri AJ, Govi S, Pileri SA, Savage KJ
Crit Rev Oncol Hematol 2012 Aug;83(2):293-302. Epub 2012 Mar 21 doi: 10.1016/j.critrevonc.2012.02.005. PMID: 22440390

Recent systematic reviews

Song F, Yu S, Jia M, Gao H, Sun PL
Int J Surg Pathol 2021 Sep;29(6):658-666. Epub 2021 Jan 11 doi: 10.1177/1066896920986967. PMID: 33427000

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