Details of Disease

General Information of Disease (ID: DISGFZXW)

• Disease Name: T-lymphoblastic lymphoma
• Synonyms: precursor T-cell Lymphoblastic lymphoma; precursor T-Lymphoblastic lymphoma; precursor T-lymphoblastic lymphoma; precursor T Lymphoblastic lymphoma; T Lymphoblastic lymphoma; T-lymphoblastic lymphoma; T-Lymphoblastic lymphoma; Precur. T-lymphoblastic lymphoma
• Definition: The most frequent type of lymphoblastic lymphoma. It comprises approximately 85-90% of cases. It is more frequently seen in adolescent males. It frequently presents with a mass lesion in the mediastinum. Pleural effusions are common. (WHO, 2001)
• Disease Hierarchy:
  DISB9ZYC: Lymphoblastic lymphoma
  DISSXRTQ: T-cell lymphoma
  DISCZ298: Precursor T-lymphoblastic lymphoma/leukemia
  DISGFZXW: T-lymphoblastic lymphoma
• Disease Identifiers:
  MONDO ID: MONDO_0044917
  UMLS CUI: C1292758
  MedGen ID: 266232
  SNOMED CT ID: 128808004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Nelarabine	DMB6VEG	Approved	Small molecular drug	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 6 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CEP85L	OTSHJFOT	Limited	Biomarker	[2]
SEC31A	OT2U42AC	Limited	Biomarker	[3]
TPR	OTUBBA4W	Limited	Genetic Variation	[4]
NINJ1	OTLRZ1EU	Disputed	Biomarker	[5]
C12orf42	OTRQP2SW	Strong	Genetic Variation	[6]
TLX1	OTVN0MNW	Definitive	Altered Expression	[7]

References

• [1] Nelarabine FDA Label
• [2] Systematic screen for tyrosine kinase rearrangements identifies a novel C6orf204-PDGFRB fusion in a patient with recurrent T-ALL and an associated myeloproliferative neoplasm.Genes Chromosomes Cancer. 2012 Jan;51(1):54-65. doi: 10.1002/gcc.20930. Epub 2011 Sep 21.
• [3] JAK2 rearrangements, including the novel SEC31A-JAK2 fusion, are recurrent in classical Hodgkin lymphoma.Blood. 2011 Apr 14;117(15):4056-64. doi: 10.1182/blood-2010-06-291310. Epub 2011 Feb 15.
• [4] Molecular identification of a TPR-FGFR1 fusion transcript in an adult with myeloproliferative neoplasm, T-lymphoblastic lymphoma, and a t(1;8)(q25;p11.2).Cancer Genet. 2014 Jun;207(6):258-62. doi: 10.1016/j.cancergen.2014.05.011. Epub 2014 Jun 7.
• [5] Ninjurin 1 has two opposing functions in tumorigenesis in a p53-dependent manner.Proc Natl Acad Sci U S A. 2017 Oct 24;114(43):11500-11505. doi: 10.1073/pnas.1711814114. Epub 2017 Oct 9.
• [6] Molecular characterization of a novel chromosomal translocation t(12;14)(q23;q11.2) in T-lymphoblastic lymphoma between the T-cell receptor delta-deleting elements (TRDREC and TRAJ61) and the hypothetical gene C12orf42.Eur J Haematol. 2010 Nov;85(5):452-6. doi: 10.1111/j.1600-0609.2010.01508.x.
• [7] T cell receptor genotyping and HOXA/TLX1 expression define three T lymphoblastic lymphoma subsets which might affect clinical outcome.Clin Cancer Res. 2008 Feb 1;14(3):692-700. doi: 10.1158/1078-0432.CCR-07-1927.