Details of Disease

General Information of Disease (ID: DISBYQTA)

• Disease Name: Central nervous system lymphoma
• Synonyms: primary lymphoma, CNS; primary central nervous system lymphoma; primary brain lymphoma; primary CNS lymphoma; microglioma; malignant lymphomas of the CNS; malignant lymphomas of CNS; lymphoma of central nervous system; central nervous system lymphoma; PCNSL; CNS lymphoma
• Disease Class: 2B33: Malignant haematopoietic neoplasm
• Definition: A non-Hodgkin or Hodgkin lymphoma that arises in the brain or spinal cord as a primary lesion. There is no evidence of lymphoma outside the central nervous system at the time of diagnosis.
• Disease Hierarchy:
  DISIT4DK: Immune system cancer
  DISNVYSW: Central nervous system cancer
  DIS0QRI7: Central nervous system hematopoietic neoplasm
  DISKAXO0: Neoplasm of mature B-cells
  DISN6V4S: Lymphoma
  DISGV1LI: Primary organ-specific lymphoma
  DISUUNPX: Epstein-Barr virus-associated malignant lymphoproliferative disorder
  DISBYQTA: Central nervous system lymphoma
• ICD Code:
  ICD-11: ICD-11: 2B33.5
  ICD-10: ICD-10: C69
  Expand ICD-11: 2D0Z
  Expand ICD-10: C69
• Disease Identifiers:
  MONDO ID: MONDO_0002571
  UMLS CUI: C0280803
  MedGen ID: 79187
  HPO ID: HP:0030069
  Orphanet ID: 46135
  SNOMED CT ID: 307649006

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 3 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Lisocabtagene maraleucel	DMP45ME	Approved	CAR T Cell Therapy	[1]
Nivolumab	DMAB9QE	Approved	Monoclonal antibody	[2]
Pemetrexed	DMMX2E6	Approved	Small molecular drug	[3]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 14 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
BCL6	TTC9YX5	Limited	Biomarker	[4]
BTK	TTGM6VW	Limited	Biomarker	[5]
CXCL13	TT0NIZ1	Limited	Altered Expression	[6]
TNFSF13B	TTWMIDN	Limited	Biomarker	[7]
HDC	TTV9GOF	Strong	Biomarker	[8]
HDGF	TTKGV26	Strong	Altered Expression	[9]
LAMC2	TTNS7H3	Strong	Biomarker	[10]
MGMT	TTJ8DV7	Strong	Biomarker	[11]
PASK	TTC0V1J	Strong	Biomarker	[12]
PDCD1LG2	TTW14O3	Strong	Biomarker	[13]
RPS6KA1	TTIXKA4	Strong	Biomarker	[12]
RPS6KA2	TT0ZW9O	Strong	Biomarker	[12]
RTN4R	TTVRZUO	Strong	Altered Expression	[14]
TNFRSF13B	TTL9OD4	Strong	Biomarker	[15]

This Disease Is Related to 21 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
ASL	OTI2NGQR	Strong	Genetic Variation	[16]
BTG2	OTZF6K1H	Strong	Biomarker	[17]
CANX	OTYP1F6J	Strong	Biomarker	[18]
CARD11	OTRCTLYC	Strong	Biomarker	[19]
CCDC8	OTO295IH	Strong	Biomarker	[18]
CD79A	OTOJC8DV	Strong	Biomarker	[20]
CEBPZ	OT11BATG	Strong	Biomarker	[21]
EXOSC3	OTNCF906	Strong	Biomarker	[18]
GNA13	OTVDL515	Strong	Genetic Variation	[22]
HRK	OTR4GWJ0	Strong	Biomarker	[23]
IBTK	OTB2GM4G	Strong	Biomarker	[24]
IVL	OT4VPNGY	Strong	Biomarker	[25]
MTX1	OTLSDNZO	Strong	Biomarker	[26]
PDLIM7	OTAZVODU	Strong	Altered Expression	[27]
PPP1R9A	OTJL2TOC	Strong	Biomarker	[28]
PTPRK	OTAP5AT3	Strong	Biomarker	[29]
PWWP3A	OTXQVL4U	Strong	Biomarker	[30]
SAMD14	OTLDQW51	Strong	Biomarker	[28]
TENM4	OT91K3FC	Strong	Genetic Variation	[31]
TOX	OTE8BL5Z	Strong	Posttranslational Modification	[32]
NFKBIE	OTLAYEL9	Definitive	Genetic Variation	[33]

References

• [1] ClinicalTrials.gov (NCT03484702) Trial to Determine the Efficacy and Safety of JCAR017 in Adult Subjects With Aggressive B-Cell Non-Hodgkin Lymphoma
• [2] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [3] Pemetrexed FDA Label
• [4] MYC, BCL2, and BCL6 rearrangements in primary central nervous system lymphoma of large B cell type.Ann Hematol. 2019 Jan;98(1):169-173. doi: 10.1007/s00277-018-3498-z. Epub 2018 Oct 11.
• [5] Introduction of novel agents in the treatment of primary CNS lymphoma.Neuro Oncol. 2019 Feb 19;21(3):306-313. doi: 10.1093/neuonc/noy193.
• [6] Diagnostic value of cerebrospinal fluid CXCL13 for acute Lyme neuroborreliosis. A systematic review and meta-analysis.Clin Microbiol Infect. 2018 Dec;24(12):1234-1240. doi: 10.1016/j.cmi.2018.04.007. Epub 2018 Apr 16.
• [7] APRIL and BAFF: novel biomarkers for central nervous system lymphoma.J Hematol Oncol. 2019 Oct 15;12(1):102. doi: 10.1186/s13045-019-0796-4.
• [8] Hematopoietic stem cell transplantation in primary central nervous system lymphoma: a review of the literature.Int J Hematol. 2019 Mar;109(3):260-277. doi: 10.1007/s12185-019-02594-1. Epub 2019 Jan 22.
• [9] The expression of hepatoma-derived growth factor in primary central nervous system lymphoma and its correlation with angiogenesis, proliferation and clinical outcome.Med Oncol. 2013;30(3):622. doi: 10.1007/s12032-013-0622-8. Epub 2013 Jun 15.
• [10] Diagnosis of central nervous system lymphoma via cerebrospinal fluid cytology: a case report.BMC Neurol. 2019 May 7;19(1):90. doi: 10.1186/s12883-019-1317-3.
• [11] miR-370 Sensitizes TMZ Response Dependent of MGMT Status in Primary Central Nervous System Lymphoma.Pathol Oncol Res. 2020 Apr;26(2):707-714. doi: 10.1007/s12253-019-00605-4. Epub 2019 Feb 2.
• [12] Discrepancy Between Low Levels of mTOR Activity and High Levels of P-S6 in Primary Central Nervous System Lymphoma May Be Explained by PAS Domain-Containing Serine/Threonine-Protein Kinase-Mediated Phosphorylation.J Neuropathol Exp Neurol. 2018 Apr 1;77(4):268-273. doi: 10.1093/jnen/nlx121.
• [13] Differential expression of individual transcript variants of PD-1 and PD-L2 genes on Th-1/Th-2 status is guaranteed for prognosis prediction in PCNSL.Sci Rep. 2019 Jul 10;9(1):10004. doi: 10.1038/s41598-019-46473-5.
• [14] R-CHOP preceded by blood-brain barrier permeabilization with engineered tumor necrosis factor- in primary CNS lymphoma.Blood. 2019 Jul 18;134(3):252-262. doi: 10.1182/blood.2019000633. Epub 2019 May 22.
• [15] CSF TACI and BAFF levels in patients with primary CNS lymphoma as novel diagnostic biomarkers.Ann Clin Transl Neurol. 2018 Oct 19;5(12):1611-1616. doi: 10.1002/acn3.668. eCollection 2018 Dec.
• [16] Differentiation between primary CNS lymphoma and glioblastoma: qualitative and quantitative analysis using arterial spin labeling MR imaging.Eur Radiol. 2018 Sep;28(9):3801-3810. doi: 10.1007/s00330-018-5359-5. Epub 2018 Apr 4.
• [17] Genomic characterization of primary central nervous system lymphoma.Acta Neuropathol. 2016 Jun;131(6):865-75. doi: 10.1007/s00401-016-1536-2. Epub 2016 Jan 12.
• [18] Primary central nervous system lymphoma and atypical glioblastoma: differentiation using the initial area under the curve derived from dynamic contrast-enhanced MR and the apparent diffusion coefficient.Eur Radiol. 2017 Apr;27(4):1344-1351. doi: 10.1007/s00330-016-4484-2. Epub 2016 Jul 19.
• [19] Mutations of CARD11 but not TNFAIP3 may activate the NF-kappaB pathway in primary CNS lymphoma.Acta Neuropathol. 2010 Oct;120(4):529-35. doi: 10.1007/s00401-010-0709-7. Epub 2010 Jun 11.
• [20] Intracranial Cell Lymphomas That Mimic Meningiomas: Case Report To Understand Complex Genetic, Radiologic, and Histopathologic Entities.World Neurosurg. 2019 May;125:339-342. doi: 10.1016/j.wneu.2019.01.298. Epub 2019 Feb 22.
• [21] Clinical Value of Vascular Permeability Estimates Using Dynamic Susceptibility Contrast MRI: Improved Diagnostic Performance in Distinguishing Hypervascular Primary CNS Lymphoma from Glioblastoma.AJNR Am J Neuroradiol. 2018 Aug;39(8):1415-1422. doi: 10.3174/ajnr.A5732. Epub 2018 Jul 19.
• [22] Analysis of Genomic Alteration in Primary Central Nervous System Lymphoma and the Expression of Some Related Genes.Neoplasia. 2018 Oct;20(10):1059-1069. doi: 10.1016/j.neo.2018.08.012. Epub 2018 Sep 15.
• [23] Defective expression of HRK is associated with promoter methylation in primary central nervous system lymphomas.Oncology. 2006;70(3):212-21. doi: 10.1159/000094322. Epub 2006 Jun 29.
• [24] Spotlight on Ibrutinib in PCNSL: Adding Another Feather to Its Cap.Cancer Discov. 2017 Sep;7(9):940-942. doi: 10.1158/2159-8290.CD-17-0714.
• [25] Different imaging patterns of PCNSL and IVL: a case report.BMC Neurol. 2019 Dec 3;19(1):311. doi: 10.1186/s12883-019-1548-3.
• [26] Risk of death, relapse or progression, and loss of life expectancy at different progression-free survival milestones in primary central nervous system lymphoma.Leuk Lymphoma. 2019 Oct;60(10):2516-2523. doi: 10.1080/10428194.2019.1594219. Epub 2019 Apr 3.
• [27] Interferon regulatory factor 4 is involved in Epstein-Barr virus-mediated transformation of human B lymphocytes.J Virol. 2008 Jul;82(13):6251-8. doi: 10.1128/JVI.00163-08. Epub 2008 Apr 16.
• [28] Hyper-N-glycosylated SAMD14 and neurabin-I as driver autoantigens of primary central nervous system lymphoma.Blood. 2018 Dec 27;132(26):2744-2753. doi: 10.1182/blood-2018-03-836932. Epub 2018 Sep 24.
• [29] Novel tumor suppressor loci on 6q22-23 in primary central nervous system lymphomas.Cancer Res. 2003 Feb 15;63(4):737-41.
• [30] Prognostic Significance of High Ki-67 Index and Histogenetic Subclassification in Primary Central Nervous System Lymphoma.Appl Immunohistochem Mol Morphol. 2018 Apr;26(4):254-262. doi: 10.1097/PAI.0000000000000424.
• [31] The mutational pattern of primary lymphoma of the central nervous system determined by whole-exome sequencing.Leukemia. 2015 Mar;29(3):677-85. doi: 10.1038/leu.2014.264. Epub 2014 Sep 5.
• [32] Genome-Wide Analysis Uncovers Novel Recurrent Alterations in Primary Central Nervous System Lymphomas.Clin Cancer Res. 2015 Sep 1;21(17):3986-94. doi: 10.1158/1078-0432.CCR-14-2116. Epub 2015 May 19.
• [33] Frequent NFKBIE deletions are associated with poor outcome in primary mediastinal B-cell lymphoma.Blood. 2016 Dec 8;128(23):2666-2670. doi: 10.1182/blood-2016-03-704528. Epub 2016 Sep 26.