ID

45175

Description

Principal Investigator: Charles Mullighan, MD, Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA MeSH: Leukemia, T-cell https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs000340 *CREST* The accurate identification of structural variations using whole-genome DNA sequencing data generated by next-generation sequencing technology is extremely difficult. To address this challenge, we have developed CREST, an algorithm that uses sequencing reads with partial alignments to the reference human genome (so-called soft-clipped reads) to directly map the breakpoints of somatic structural variations. We applied CREST to paired tumor/normal whole genome sequencing data from five cases of T-lineage acute lymphoblastic leukemia (T-ALL). A total of 110 somatic structural variants were identified, 80% of which were validated by genomic PCR and Sanger sequencing. The validated structural variants included 31 inter-chromosomal translocations, 19 intra-chromosomal translocations, one inversion, 22 deletions and 16 insertions. A comparison of the results generated with CREST to those obtained using the traditional paired-end discordant mapping methods demonstrate CREST to have a much higher sensitivity and specificity. In addition, application of CREST to publicly available whole-genome sequencing data from the human melanoma cancer cell line COLO-829 demonstrated the identification of 50 novel structural variations not detected using the standard methods, 20 of which were selected for validation with a 90% success rate. These data demonstrate that direct mapping of soft-clipped reads offers an improved method for detecting structural variants at the nucleotide level of resolution. *T-ALL* Early T-cell precursor acute lymphoblastic leukaemia (ETP ALL) is an aggressive malignancy of unknown genetic basis. We performed whole-genome sequencing of 12 ETP ALL cases and assessed the frequency of the identified somatic mutations in 94 T-cell acute lymphoblastic leukaemia cases. ETP ALL was characterized by activating mutations in genes regulating cytokine receptor and RAS signalling (67% of cases; *NRAS*, *KRAS*, *FLT3*, *IL7R*, *JAK3*, *JAK1*, *SH2B3* and *BRAF*), inactivating lesions disrupting haematopoietic development (58%; *GATA3*, *ETV6*, *RUNX1*, *IKZF1* and *EP300*) and histone-modifying genes (48%; *EZH2*, *EED*, *SUZ12*, iSETD2/i and iEP300/i). We also identified new targets of recurrent mutation including iDNM2/i, iECT2L/i and iRELN/i. The mutational spectrum is similar to myeloid tumours, and moreover, the global transcriptional profile of ETP ALL was similar to that of normal and myeloid leukaemia haematopoietic stem cells. These findings suggest that addition of myeloid-directed therapies might improve the poor outcome of ETP ALL.

Link

dbGaP study = phs000340

Keywords

  1. 8/22/22 8/22/22 - Simon Heim
  2. 10/12/22 10/12/22 - Adrian Schulz
Copyright Holder

Charles Mullighan, MD, Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA

Uploaded on

October 12, 2022

DOI

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License

Creative Commons BY 4.0

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dbGaP phs000340 Sequencing of ETP T-ALL

Eligibility Criteria

Inclusion and exclusion criteria
Description

Inclusion and exclusion criteria

Cases of early T cell progenitor acute lymphoblastic leukemia (ETP T-ALL). The cases in this cohort have had whole genome sequencing of tumor and normal DNA performed. The definition of ETP T-ALL can be found in Lancet Oncol. 2009 Feb;10(2):147-56. PMID: 19147408. Cases were selected that had appropriate consent for genetic studies, and suitable material for sequencing (high purity tumor populations and available normal DNA obtained at disease remission).
Description

Cases of early T cell progenitor acute lymphoblastic leukemia (ETP T-ALL). The cases in this cohort have had whole genome sequencing of tumor and normal DNA performed. Cases were selected that had appropriate consent for genetic studies, and suitable material for sequencing (high purity tumor populations and available normal DNA obtained at disease remission).

Data type

boolean

Alias
UMLS CUI [1,1]
C1706256
UMLS CUI [1,2]
C1301359
UMLS CUI [1,3]
C3640076
UMLS CUI [1,4]
C0027651
UMLS CUI [1,5]
C0012854
UMLS CUI [1,6]
C0021430
UMLS CUI [1,7]
C0370003

Similar models

Eligibility Criteria

Name
Type
Description | Question | Decode (Coded Value)
Data type
Alias
Item Group
Inclusion and exclusion criteria
Cases of early T cell progenitor acute lymphoblastic leukemia (ETP T-ALL). The cases in this cohort have had whole genome sequencing of tumor and normal DNA performed. Cases were selected that had appropriate consent for genetic studies, and suitable material for sequencing (high purity tumor populations and available normal DNA obtained at disease remission).
Item
Cases of early T cell progenitor acute lymphoblastic leukemia (ETP T-ALL). The cases in this cohort have had whole genome sequencing of tumor and normal DNA performed. The definition of ETP T-ALL can be found in Lancet Oncol. 2009 Feb;10(2):147-56. PMID: 19147408. Cases were selected that had appropriate consent for genetic studies, and suitable material for sequencing (high purity tumor populations and available normal DNA obtained at disease remission).
boolean
C1706256 (UMLS CUI [1,1])
C1301359 (UMLS CUI [1,2])
C3640076 (UMLS CUI [1,3])
C0027651 (UMLS CUI [1,4])
C0012854 (UMLS CUI [1,5])
C0021430 (UMLS CUI [1,6])
C0370003 (UMLS CUI [1,7])

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