General Information of Disease (ID: DISP4D1R)

Disease Name Anaplastic large cell lymphoma
Synonyms
anaplastic large cell lymphoma; CD30 Positive anaplastic large cell lymphoma; Ki-1 lymphoma; Ki-1+ ALCL; primary systemic ALCL; Ki-1 positive anaplastic large cell lymphoma; ALCL; CD30 positive anaplastic large cell lymphoma; sACL; Ki-1+ anaplastic large cell lymphoma
Disease Class 2A90: Mature T-cell lymphoma
Definition
Anaplastic large cell lymphoma (ALCL) is a rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL.
Disease Hierarchy
DISCDT4E: Peripheral T-cell lymphoma
DISP4D1R: Anaplastic large cell lymphoma
ICD Code
ICD-11
ICD-11: 2A90.A
ICD-10
ICD-10: C84.6
Expand ICD-11
'2A90.A
Expand ICD-10
'C84.6
Disease Identifiers
MONDO ID
MONDO_0020325
MESH ID
D017728
UMLS CUI
C0206180
MedGen ID
61533
HPO ID
HP:0012193
SNOMED CT ID
53237008

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 1 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Brigatinib DM7W94S Approved Small molecular drug [1]
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This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
TRBC1 DM1ZK4W Phase 1/2 CAR T Cell Therapy [2]
ICAR30 T cells DM3YZCV Phase 1 CAR T Cell Therapy [3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 26 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
HSP90AA1 TT78R5H Limited Biomarker [4]
WAS TTE8T73 Limited Altered Expression [5]
JUN TTS7IR5 moderate Altered Expression [6]
STAT3 TTHJT3X moderate Altered Expression [7]
ANPEP TTPHMWB Strong Biomarker [8]
ATIC TT9NVXQ Strong Genetic Variation [9]
BACH1 TT2ME4S Strong Biomarker [10]
CCR3 TTD3XFU Strong Altered Expression [11]
CCR8 TTE836A Strong Altered Expression [12]
CD52 TTQT5S9 Strong Altered Expression [13]
CEBPB TTUI35N Strong Biomarker [14]
CYP51A1 TT67TDP Strong Biomarker [15]
DUSP5 TTZN92A Strong Genetic Variation [16]
E2F2 TT5FYX0 Strong Biomarker [10]
EML4 TT92GY4 Strong Biomarker [17]
GZMB TTKEPHX Strong Altered Expression [18]
JAK3 TTT7PJU Strong Altered Expression [19]
NCAM1 TTVXPHT Strong Biomarker [20]
PNLIP TTXMY0J Strong Biomarker [21]
PRSS1 TT2WR1T Strong Biomarker [22]
PTPN6 TT369M5 Strong Altered Expression [23]
RORC TTGV6LY Strong Biomarker [24]
RPE65 TTBOH16 Strong Genetic Variation [25]
SQLE TTE14XG Strong Altered Expression [26]
TWIST1 TTX1MY7 Strong Biomarker [27]
ID2 TTW8A5N Definitive Altered Expression [28]
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⏷ Show the Full List of 26 DTT(s)
This Disease Is Related to 60 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
BATF3 OTI61WXQ Limited Biomarker [29]
CCNT1 OTDYVGCP Limited Altered Expression [30]
GTF2H4 OTPD1DIU Limited Biomarker [31]
HOXC5 OTPQTLKZ Limited Altered Expression [32]
HOXC6 OTBCRAZV Limited Altered Expression [32]
TNFSF8 OTDYGDJ3 Limited Altered Expression [33]
BCL10 OT47MCLI Disputed Altered Expression [34]
FOSB OTW6C05J moderate Altered Expression [6]
JUNB OTG2JXV5 moderate Altered Expression [6]
JUND OTNKACJD moderate Altered Expression [6]
ADARB1 OTGKSZEV Strong Altered Expression [35]
ARNT OTMSIEZY Strong Biomarker [36]
BATF OT9VSD2D Strong Altered Expression [24]
BCL3 OT1M5B95 Strong Altered Expression [37]
BLNK OTSSPF6F Strong Altered Expression [38]
CARS1 OTOUZF6O Strong Altered Expression [9]
CARS2 OTGLZOFP Strong Genetic Variation [9]
CHP1 OTHTXN1A Strong Genetic Variation [39]
CIB1 OT4BVCRU Strong Altered Expression [40]
CKAP4 OTDUC9ME Strong Genetic Variation [25]
CLIP1 OTTGAEJE Strong Altered Expression [41]
CLTC OTBFASMA Strong Genetic Variation [42]
CLTCL1 OT6W5BNR Strong Genetic Variation [9]
COL15A1 OTTFKK18 Strong Altered Expression [41]
CROT OTWK68MM Strong Biomarker [43]
DOK2 OTIJ38UF Strong Biomarker [44]
DUSP22 OTEZ3U85 Strong Genetic Variation [25]
HERPUD1 OT9EROL6 Strong Genetic Variation [45]
HRNR OT560QR5 Strong Altered Expression [46]
IRF4 OT1DHQ1P Strong Biomarker [16]
MAGEH1 OTVE05K7 Strong Altered Expression [41]
MCC OTQVI1EM Strong Biomarker [47]
MSN OTZJ4J6G Strong Biomarker [48]
NAPSA OT6F8IAL Strong Biomarker [49]
PDLIM2 OTEURRPD Strong Altered Expression [50]
PDLIM7 OTAZVODU Strong Biomarker [51]
PRDM8 OTRA3265 Strong Genetic Variation [52]
PRF1 OTFVXD7H Strong Genetic Variation [53]
PRSS2 OTOMVUWL Strong Biomarker [22]
PRSS3 OTN3S5YB Strong Biomarker [22]
PWWP3A OTXQVL4U Strong Biomarker [13]
RBAK OTYJ6EAU Strong Biomarker [10]
RGL4 OTTAEWCF Strong Genetic Variation [54]
RHOV OTW4N3QN Strong Genetic Variation [39]
RNF213 OT4OVE9O Strong Genetic Variation [9]
RPL18A OTKBMVCX Strong Altered Expression [55]
RPTN OTOAA25U Strong Altered Expression [46]
SERPINB9 OTJ811IF Strong Altered Expression [56]
SKAP1 OTD4ZKAL Strong Biomarker [44]
SMG1 OTTS3SXE Strong Altered Expression [57]
TCHHL1 OTAMOJGN Strong Altered Expression [46]
TFG OT2KJENI Strong Genetic Variation [58]
TIA1 OTGPN3P8 Strong Altered Expression [59]
TMOD1 OTTRYF9Y Strong Biomarker [60]
TP53INP1 OT2363Z9 Strong Altered Expression [10]
TPM3 OT5RU5G6 Strong Altered Expression [61]
TRAP1 OTNG0L8J Strong Biomarker [62]
TRIM32 OTJOV0PG Strong Biomarker [10]
TSPYL2 OTGGW2EF Strong Biomarker [63]
GNLY OTZJKA8C Definitive Biomarker [64]
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⏷ Show the Full List of 60 DOT(s)

References

1 2017 FDA drug approvals.Nat Rev Drug Discov. 2018 Feb;17(2):81-85.
2 ClinicalTrials.gov (NCT03590574) Phase I/II Study Evaluating AUTO4 in Patients With TRBC1 Positive T Cell Lymphoma
3 ClinicalTrials.gov (NCT03383965) CD30 Targeted CAR-T in Treating CD30-Expressing Lymphomas
4 CD30 Induces Heat Shock Protein 90 and Signal Integration in Classic Hodgkin Lymphoma Cells.Am J Pathol. 2017 Jan;187(1):163-175. doi: 10.1016/j.ajpath.2016.09.007. Epub 2016 Nov 19.
5 Wiskott-Aldrich syndrome protein (WASP) is a tumor suppressor in T cell lymphoma.Nat Med. 2019 Jan;25(1):130-140. doi: 10.1038/s41591-018-0262-9. Epub 2018 Dec 3.
6 The Role of Activator Protein-1 (AP-1) Family Members in CD30-Positive Lymphomas.Cancers (Basel). 2018 Mar 28;10(4):93. doi: 10.3390/cancers10040093.
7 PD-L1 expression is associated with ALK positivity and STAT3 activation, but not outcome in patients with systemic anaplastic large cell lymphoma.Mod Pathol. 2020 Mar;33(3):324-333. doi: 10.1038/s41379-019-0336-3. Epub 2019 Aug 5.
8 CD13+ anaplastic large cell lymphoma with leukemic presentation and additional chromosomal abnormality.Diagn Cytopathol. 2010 Feb;38(2):141-6. doi: 10.1002/dc.21170.
9 Identification of novel fusion partners of ALK, the anaplastic lymphoma kinase, in anaplastic large-cell lymphoma and inflammatory myofibroblastic tumor.Genes Chromosomes Cancer. 2002 Aug;34(4):354-62. doi: 10.1002/gcc.10033.
10 Identification of putative pathogenic microRNA and its downstream targets in anaplastic lymphoma kinase-negative anaplastic large cell lymphoma.Hum Pathol. 2014 Oct;45(10):1995-2005. doi: 10.1016/j.humpath.2014.06.012. Epub 2014 Jun 30.
11 Expression of CCR3 and CCR4 Suggests a Poor Prognosis in Mycosis Fungoides and Szary Syndrome.Acta Derm Venereol. 2019 Jul 1;99(9):809-812. doi: 10.2340/00015555-3207.
12 Expression of the chemokine receptor gene, CCR8, is associated With DUSP22 rearrangements in anaplastic large cell lymphoma.Appl Immunohistochem Mol Morphol. 2015 Sep;23(8):580-9. doi: 10.1097/PAI.0000000000000118.
13 CD30-positive peripheral T-cell lymphomas share molecular and phenotypic features.Haematologica. 2013 Aug;98(8):1250-8. doi: 10.3324/haematol.2012.081935. Epub 2013 May 28.
14 Identification of C/EBP target genes in ALK+ anaplastic large cell lymphoma (ALCL) by gene expression profiling and chromatin immunoprecipitation.PLoS One. 2013 May 31;8(5):e64544. doi: 10.1371/journal.pone.0064544. Print 2013.
15 Crizotinib enhances anti-CD30-LDM induced antitumor efficacy in NPM-ALK positive anaplastic large cell lymphoma.Cancer Lett. 2019 Apr 28;448:84-93. doi: 10.1016/j.canlet.2019.02.002. Epub 2019 Feb 8.
16 DUSP22-IRF4 rearrangement in AIDS-associated ALK-negative anaplastic large cell lymphoma.BMJ Case Rep. 2019 Sep 30;12(9):e230641. doi: 10.1136/bcr-2019-230641.
17 Treatment Options for Paediatric Anaplastic Large Cell Lymphoma (ALCL): Current Standard and beyond.Cancers (Basel). 2018 Mar 30;10(4):99. doi: 10.3390/cancers10040099.
18 Expression of granzyme B sensitizes ALK+ ALCL tumour cells to apoptosis-inducing drugs.Mol Cancer. 2014 Aug 29;13:199. doi: 10.1186/1476-4598-13-199.
19 IL-21 contributes to JAK3/STAT3 activation and promotes cell growth in ALK-positive anaplastic large cell lymphoma.Am J Pathol. 2009 Aug;175(2):825-34. doi: 10.2353/ajpath.2009.080982. Epub 2009 Jul 16.
20 CD56-positive adult T-cell leukemia/lymphoma: a case report and a review of the literature.Med Mol Morphol. 2015 Mar;48(1):54-9. doi: 10.1007/s00795-014-0072-1. Epub 2014 Mar 28.
21 Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes.Hum Pathol. 1998 Apr;29(4):397-403. doi: 10.1016/s0046-8177(98)90122-8.
22 Sub-megabase resolution tiling (SMRT) array-based comparative genomic hybridization profiling reveals novel gains and losses of chromosomal regions in Hodgkin Lymphoma and Anaplastic Large Cell Lymphoma cell lines.Mol Cancer. 2008 Jan 7;7:2. doi: 10.1186/1476-4598-7-2.
23 SHP1 tyrosine phosphatase negatively regulates NPM-ALK tyrosine kinase signaling.Blood. 2006 May 15;107(10):4130-8. doi: 10.1182/blood-2005-06-2421. Epub 2006 Feb 9.
24 The AP-1-BATF and -BATF3 module is essential for growth, survival and TH17/ILC3 skewing of anaplastic large cell lymphoma.Leukemia. 2018 Sep;32(9):1994-2007. doi: 10.1038/s41375-018-0045-9. Epub 2018 Mar 28.
25 ALCL by any other name: the many facets of anaplastic large cell lymphoma.Pathology. 2020 Jan;52(1):100-110. doi: 10.1016/j.pathol.2019.09.007. Epub 2019 Nov 6.
26 Squalene accumulation in cholesterol auxotrophic lymphomas prevents oxidative cell death.Nature. 2019 Mar;567(7746):118-122. doi: 10.1038/s41586-019-0945-5. Epub 2019 Feb 13.
27 Aberrant expression of the transcriptional factor Twist1 promotes invasiveness in ALK-positive anaplastic large cell lymphoma. Cell Signal. 2012 Apr;24(4):852-8. doi: 10.1016/j.cellsig.2011.11.020. Epub 2011 Dec 8.
28 The helix-loop-helix protein Id2 is expressed differentially and induced by myc in T-cell lymphomas.Cancer. 2008 Feb 1;112(3):552-61. doi: 10.1002/cncr.23196.
29 Ectopic expression of transcription factor BATF3 induces B-cell lymphomas in a murine B-cell transplantation model.Oncotarget. 2018 Mar 23;9(22):15942-15951. doi: 10.18632/oncotarget.24639. eCollection 2018 Mar 23.
30 CDK9/CYCLIN T1 expression during normal lymphoid differentiation and malignant transformation.J Pathol. 2004 Aug;203(4):946-52. doi: 10.1002/path.1588.
31 Stimulation of CD30 in anaplastic large cell lymphoma leads to production of nuclear factor-kappaB p52, which is associated with hyperphosphorylated Bcl-3.Cancer Sci. 2005 Aug;96(8):487-97. doi: 10.1111/j.1349-7006.2005.00078.x.
32 HOXC4, HOXC5, and HOXC6 expression in primary cutaneous lymphoid lesions. High expression of HOXC5 in anaplastic large-cell lymphomas.Am J Pathol. 1997 Oct;151(4):1067-74.
33 The expression of CD30 in anaplastic large cell lymphoma is regulated by nucleophosmin-anaplastic lymphoma kinase-mediated JunB level in a cell type-specific manner.Cancer Res. 2006 Sep 15;66(18):9002-8. doi: 10.1158/0008-5472.CAN-05-4101.
34 BCL10 down-regulation in peripheral T-cell lymphomas.Hum Pathol. 2012 Dec;43(12):2266-73. doi: 10.1016/j.humpath.2012.03.024. Epub 2012 Jul 19.
35 CD26, together with cell surface adenosine deaminase, is selectively expressed on ALK-positive, but not on ALK-negative, anaplastic large cell lymphoma and Hodgkin's lymphoma.Leuk Lymphoma. 2006 Oct;47(10):2181-8. doi: 10.1080/10428190600773396.
36 The aryl hydrocarbon nuclear translocator alters CD30-mediated NF-kappaB-dependent transcription.Science. 2009 Jan 9;323(5911):251-5. doi: 10.1126/science.1162818.
37 Reappraisal of BCL3 as a molecular marker of anaplastic large cell lymphoma.Int J Hematol. 2005 Dec;82(5):397-405. doi: 10.1532/IJH97.05045.
38 The histologic, immunohistochemical, and genetic features of classical Hodgkin lymphoma and anaplastic large cell lymphoma with aberrant T-cell/B-cell antigen expression.Hum Pathol. 2019 Feb;84:309-320. doi: 10.1016/j.humpath.2018.10.003. Epub 2018 Oct 17.
39 Five-year outcomes for frontline brentuximab vedotin with CHP for CD30-expressing peripheral T-cell lymphomas.Blood. 2018 May 10;131(19):2120-2124. doi: 10.1182/blood-2017-12-821009. Epub 2018 Mar 5.
40 Expression of p27(Kip1) and c-Jun activation binding protein 1 are inversely correlated in systemic anaplastic large cell lymphoma.Clin Cancer Res. 2003 Mar;9(3):1121-8.
41 Restin in Hodgkin's disease and anaplastic large cell lymphoma.Leuk Lymphoma. 1993 Dec;12(1-2):21-6. doi: 10.3109/10428199309059567.
42 ALK activation by the CLTC-ALK fusion is a recurrent event in large B-cell lymphoma.Blood. 2003 Oct 1;102(7):2638-41. doi: 10.1182/blood-2003-04-1050. Epub 2003 May 15.
43 Involvement of Cot activity in the proliferation of ALCL lymphoma cells.Biochem Biophys Res Commun. 2011 Aug 12;411(4):655-60. doi: 10.1016/j.bbrc.2011.06.157. Epub 2011 Jun 29.
44 Intracellular TCR-signaling pathway: novel markers for lymphoma diagnosis and potential therapeutic targets.Am J Surg Pathol. 2014 Oct;38(10):1349-59. doi: 10.1097/PAS.0000000000000309.
45 Mitochondrial Hyperactivation and Enhanced ROS Production are Involved in Toxicity Induced by Oncogenic Kinases Over-Signaling.Cancers (Basel). 2018 Dec 12;10(12):509. doi: 10.3390/cancers10120509.
46 Expression of the novel intermediate filament-associated protein restin in Hodgkin's disease and anaplastic large-cell lymphoma.Blood. 1992 Dec 1;80(11):2891-6.
47 Conjugation of DM1 to anti-CD30 antibody has potential antitumor activity in CD30-positive hematological malignancies with lower systemic toxicity.MAbs. 2019 Aug/Sep;11(6):1149-1161. doi: 10.1080/19420862.2019.1618674. Epub 2019 Jun 4.
48 Heterogeneity of genomic breakpoints in MSN-ALK translocations in anaplastic large cell lymphoma.Hum Pathol. 2004 Aug;35(8):1038-41. doi: 10.1016/j.humpath.2004.05.006.
49 Aberrant expression of napsin A in a subset of malignant lymphomas.Histol Histopathol. 2016 Feb;31(2):213-21. doi: 10.14670/HH-11-669. Epub 2015 Sep 24.
50 Inactivation of the putative ubiquitin-E3 ligase PDLIM2 in classical Hodgkin and anaplastic large cell lymphoma.Leukemia. 2017 Mar;31(3):602-613. doi: 10.1038/leu.2016.238. Epub 2016 Aug 19.
51 Epstein-Barr virus in CD30 anaplastic large cell lymphoma involving the skin and lymphomatoid papulosis in South Korea.Int J Dermatol. 2006 Nov;45(11):1312-6. doi: 10.1111/j.1365-4632.2006.02951.x.
52 Common origin of sequential cutaneous CD30+ lymphoproliferations with nodal involvement evidenced by genome-wide clonal evolution.Histopathology. 2019 Mar;74(4):654-662. doi: 10.1111/his.13783. Epub 2019 Jan 31.
53 Monoallelic mutations of the perforin gene may represent a predisposing factor to childhood anaplastic large cell lymphoma.J Pediatr Hematol Oncol. 2014 Aug;36(6):e359-65. doi: 10.1097/MPH.0000000000000073.
54 Human rgr: transforming activity and alteration in T-cell malignancies.Oncogene. 2002 Aug 1;21(33):5108-16. doi: 10.1038/sj.onc.1205694.
55 Transcriptional analysis distinguishes breast implant-associated anaplastic large cell lymphoma from other peripheral T-cell lymphomas.Mod Pathol. 2019 Feb;32(2):216-230. doi: 10.1038/s41379-018-0130-7. Epub 2018 Sep 11.
56 Expression levels of apoptosis-related proteins predict clinical outcome in anaplastic large cell lymphoma.Blood. 2002 Jun 15;99(12):4540-6. doi: 10.1182/blood.v99.12.4540.
57 NPM-ALK-dependent expression of the transcription factor CCAAT/enhancer binding protein beta in ALK-positive anaplastic large cell lymphoma.Blood. 2006 Sep 15;108(6):2029-36. doi: 10.1182/blood-2005-10-014258. Epub 2006 May 18.
58 Diversity of genomic breakpoints in TFG-ALK translocations in anaplastic large cell lymphomas: identification of a new TFG-ALK(XL) chimeric gene with transforming activity.Am J Pathol. 2002 Apr;160(4):1487-94. doi: 10.1016/S0002-9440(10)62574-6.
59 Cytotoxic molecule expression is predictive of prognosis in Hodgkin's-like anaplastic large cell lymphoma.Histopathology. 2007 May;50(6):705-15. doi: 10.1111/j.1365-2559.2007.02674.x.
60 Identification of a 3-gene model as a powerful diagnostic tool for the recognition of ALK-negative anaplastic large-cell lymphoma.Blood. 2012 Aug 9;120(6):1274-81. doi: 10.1182/blood-2012-01-405555. Epub 2012 Jun 27.
61 Analysis of gene expression profile of TPM3-ALK positive anaplastic large cell lymphoma reveals overlapping and unique patterns with that of NPM-ALK positive anaplastic large cell lymphoma.Leuk Res. 2008 Mar;32(3):383-93. doi: 10.1016/j.leukres.2007.07.012. Epub 2007 Aug 27.
62 Studies of phosphoproteomic changes induced by nucleophosmin-anaplastic lymphoma kinase (ALK) highlight deregulation of tumor necrosis factor (TNF)/Fas/TNF-related apoptosis-induced ligand signaling pathway in ALK-positive anaplastic large cell lymphoma.Mol Cell Proteomics. 2010 Jul;9(7):1616-32. doi: 10.1074/mcp.M000153-MCP201. Epub 2010 Apr 14.
63 Interleukin-2 receptor-directed therapies for cutaneous lymphomas.Hematol Oncol Clin North Am. 2003 Dec;17(6):1449-58. doi: 10.1016/s0889-8588(03)00110-2.
64 The expression of granulysin in systemic anaplastic large cell lymphoma in childhood.Leuk Res. 2009 Jul;33(7):908-12. doi: 10.1016/j.leukres.2009.01.032. Epub 2009 Feb 24.