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ALK-positive large B-cell lymphoma

MedGen UID:
232429
•Concept ID:
C1333294
•
Neoplastic Process
Synonyms: ALK positive large B-cell lymphoma; ALK+ large B-cell lymphoma; ALK+ LBCL; ALK-DLBCL; ALK-Positive Large B-Cell Lymphoma; Anaplastic lymphoma kinase positive large B-cell lymphoma; Diffuse Large B-Cell Lymphoma with Expression of Full-Length ALK; diffuse large B-cell lymphoma with expression of full-length ALK; Diffuse Large B-Cell Lymphoma with Expression of Full-Length Anaplastic Lymphoma Kinase; diffuse large B-cell lymphoma with expression of full-length anaplastic lymphoma kinase
SNOMED CT: Anaplastic lymphoma kinase positive large B-cell lymphoma (450910000); ALK positive large B-cell lymphoma (450910000); Anaplastic lymphoma kinase positive large B-cell lymphoma (715950008); ALK-positive large B-cell lymphoma (715950008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018225
Orphanet: ORPHA364043

Definition

A very rare variant of diffuse large B-cell lymphoma mainly affecting middle-aged immunocompetent men with features of a consistent primary involvement of lymph nodes (mainly in the cervical and mediastinum lymph nodes) and with infrequent extra nodal involvement of the bone marrow and other extra-nodal sites (head and neck region, liver, spleen, and gastrointestinal tract). It has an aggressive disease course, and is associated with a poor prognosis. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVALK-positive large B-cell lymphoma

Professional guidelines

PubMed

Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK + LBCL): a systematic review of clinicopathological features and management.
Castillo JJ, Beltran BE, Malpica L, Marques-Piubelli ML, Miranda RN
Leuk Lymphoma 2021 Dec;62(12):2845-2853. Epub 2021 Jun 21 doi: 10.1080/10428194.2021.1941929. PMID: 34151703

Recent clinical studies

Etiology

An Expanded Spectrum of High-Grade B-Cell Non-Hodgkin Lymphomas Involving the Cervicovaginal Region.
Pather S, Philip V, Lakha AB, Wiggill TM, Suleman M, Patel M
Int J Gynecol Pathol 2015 Nov;34(6):564-9. doi: 10.1097/PGP.0000000000000220. PMID: 26352550
Anaplastic lymphoma kinase-positive large B-cell lymphoma: description of a case with an unexpected clinical outcome.
Zanelli M, Valli R, Capodanno I, Ragazzi M, Ascani S
Int J Surg Pathol 2015 Feb;23(1):78-83. Epub 2014 May 26 doi: 10.1177/1066896914536223. PMID: 24868005
Hyperactivated STAT3 in ALK-positive diffuse large B-cell lymphoma with clathrin-ALK fusion.
Momose S, Tamaru J, Kishi H, Mikata I, Mori M, Toyozumi Y, Itoyama S
Hum Pathol 2009 Jan;40(1):75-82. Epub 2008 Aug 27 doi: 10.1016/j.humpath.2008.06.009. PMID: 18755494

Diagnosis

ALK+ large B cell lymphoma presenting as multiple bowel-obstructing, cytokeratin-positive tumours.
Petronilho S, Gournay V, Tauziede-Espariat A, Pina H, Pecriaux A, Drieux F, Poullot E, Briere J, Lechapt E, Gaulard P
Histopathology 2022 Jun;80(7):1128-1130. Epub 2022 Mar 17 doi: 10.1111/his.14635. PMID: 35238057
Lymphoproliferative Neoplasms With Plasmablastic Morphology.
Zhou J, Nassiri M
Arch Pathol Lab Med 2022 Apr 1;146(4):407-414. doi: 10.5858/arpa.2021-0117-RA. PMID: 34559873
Diffuse large B-cell lymphoma variants: an update.
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
ALK-positive Large B-cell Lymphoma: A Clinicopathologic Study of 26 Cases With Review of Additional 108 Cases in the Literature.
Pan Z, Hu S, Li M, Zhou Y, Kim YS, Reddy V, Sanmann JN, Smith LM, Chen M, Gao Z, Wang HY, Yuan J
Am J Surg Pathol 2017 Jan;41(1):25-38. doi: 10.1097/PAS.0000000000000753. PMID: 27740969
Large B-cell lymphomas with plasmablastic differentiation: a biological and therapeutic challenge.
Montes-Moreno S, Montalbán C, Piris MA
Leuk Lymphoma 2012 Feb;53(2):185-94. Epub 2011 Sep 19 doi: 10.3109/10428194.2011.608447. PMID: 21812534

Therapy

Acquired resistance to crizotinib in novel CDK14-ALK and CLTC-ALK fusions of ALK-positive large B-cell lymphoma identified by next-generation sequencing.
Xia Y, Zhang L, He W, Pan H, Fang J, Cui G
Cancer Biol Ther 2023 Dec 31;24(1):2271212. Epub 2023 Oct 31 doi: 10.1080/15384047.2023.2271212. PMID: 37906510Free PMC Article
Next-generation ALK inhibitors are highly active in ALK-positive large B-cell lymphoma.
Soumerai JD, Rosenthal A, Harkins S, Duffy J, Mecca C, Wang Y, Grewal RK, El-Jawahri AR, Liu H, Menard C, Dogan A, Yang L, Rimsza LM, Bantilan K, Martin H, Lei M, Mohr S, Kurilovich A, Kudryashova O, Postovalova E, Nardi V, Abramson JS, Chiarle R, Zelenetz AD, Louissaint A Jr
Blood 2022 Oct 20;140(16):1822-1826. doi: 10.1182/blood.2022015443. PMID: 35802834Free PMC Article
Primary Cutaneous Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma.
Kempf W, Torricelli R, Zettl A, Zimmermann AK, Berisha A, Ghielmini M
Am J Dermatopathol 2019 Aug;41(8):602-605. doi: 10.1097/DAD.0000000000001348. PMID: 31335416
Anaplastic lymphoma kinase-positive large B-cell lymphoma: description of a case with an unexpected clinical outcome.
Zanelli M, Valli R, Capodanno I, Ragazzi M, Ascani S
Int J Surg Pathol 2015 Feb;23(1):78-83. Epub 2014 May 26 doi: 10.1177/1066896914536223. PMID: 24868005
Identification of a novel fusion, SQSTM1-ALK, in ALK-positive large B-cell lymphoma.
Takeuchi K, Soda M, Togashi Y, Ota Y, Sekiguchi Y, Hatano S, Asaka R, Noguchi M, Mano H
Haematologica 2011 Mar;96(3):464-7. Epub 2010 Dec 6 doi: 10.3324/haematol.2010.033514. PMID: 21134980Free PMC Article

Prognosis

Acquired resistance to crizotinib in novel CDK14-ALK and CLTC-ALK fusions of ALK-positive large B-cell lymphoma identified by next-generation sequencing.
Xia Y, Zhang L, He W, Pan H, Fang J, Cui G
Cancer Biol Ther 2023 Dec 31;24(1):2271212. Epub 2023 Oct 31 doi: 10.1080/15384047.2023.2271212. PMID: 37906510Free PMC Article
ALK-positive Large B-Cell Lymphoma With Multiple Epithelial Antigen Expression and PABPC1::ALK Fusion : A Novel Molecular Alteration.
Fu JJ, Seth A, Ali N, Chandar A, Bains A
Am J Surg Pathol 2023 Aug 1;47(8):950-954. Epub 2023 Jun 19 doi: 10.1097/PAS.0000000000002069. PMID: 37326123
Survival of patients with CD20-negative variants of large B-cell lymphoma: an analysis of the National Cancer Data Base.
Qunaj L, Castillo JJ, Olszewski AJ
Leuk Lymphoma 2018 Jun;59(6):1375-1383. Epub 2017 Oct 11 doi: 10.1080/10428194.2017.1387912. PMID: 29019447
ALK-positive large B-cell lymphoma: identification of EML4-ALK and a review of the literature focusing on the ALK immunohistochemical staining pattern.
Sakamoto K, Nakasone H, Togashi Y, Sakata S, Tsuyama N, Baba S, Dobashi A, Asaka R, Tsai CC, Chuang SS, Izutsu K, Kanda Y, Takeuchi K
Int J Hematol 2016 Apr;103(4):399-408. Epub 2016 Jan 18 doi: 10.1007/s12185-016-1934-1. PMID: 26781614
Anaplastic lymphoma kinase-positive large B-cell lymphoma: description of a case with an unexpected clinical outcome.
Zanelli M, Valli R, Capodanno I, Ragazzi M, Ascani S
Int J Surg Pathol 2015 Feb;23(1):78-83. Epub 2014 May 26 doi: 10.1177/1066896914536223. PMID: 24868005

Clinical prediction guides

Anaplastic lymphoma kinase-positive large B-cell lymphoma: a case report with emphasis on the cytological features of the pleural effusion.
Ishida M, Yoshida K, Kagotani A, Iwai M, Yoshii M, Okuno H, Horinouchi A, Nakanishi R, Harada A, Yoshida T, Okuno T, Hodohara K, Okabe H
Int J Clin Exp Pathol 2013;6(11):2631-5. Epub 2013 Oct 15 PMID: 24228132Free PMC Article
ALK-positive large B-cell lymphomas express a terminal B-cell differentiation program and activated STAT3 but lack MYC rearrangements.
Valera A, Colomo L, Martínez A, de Jong D, Balagué O, Matheu G, Martínez M, Taddesse-Heath L, Jaffe ES, Bacchi CE, Campo E
Mod Pathol 2013 Oct;26(10):1329-37. Epub 2013 Apr 19 doi: 10.1038/modpathol.2013.73. PMID: 23599149Free PMC Article

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