Journal
BLOOD ADVANCES
Volume 5, Issue 14, Pages 2890-2900Publisher
ELSEVIER
DOI: 10.1182/bloodadvances.2021004334
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Funding
- National Institutes of Health, National Cancer Institute's National Clinical Trials Network (NCTN) Operations Center Grant [U10CA180886]
- NCTN Statistics & Data Center Grant [U10CA180899]
- Children's Oncology Group Biospecimen Bank Grant [U24CA196173]
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ETP-LLy is a subtype of T-LLy that shares similarities with ETP-ALL but also has distinct genetic features compared to non-ETP T-LLy, indicating it may be a separate entity.
Early T-cell precursor phenotype acute lymphoblastic leukemia (ETP-ALL) is a subtype of T-ALL with a unique immunophenotype and genetic abnormalities distinct from conventional T-ALL. A subset of T lymphoblastic lymphoma (T-LLy) also demonstrates the early T-cell precursor immunophenotype and may be a counterpart of ETP-ALL. Unlike ETP-ALL, the incidence, clinical features, and genomic features of ETP-LLy are unknown. We reviewed the immunophenotyping data of 218 T-LLy patients who enrolled in the Children's Oncology Group AALL0434 clinical trial and identified 9 cases (4%) exhibiting a definitive ETP immunophenotype. We performed single-nucleotide polymorphism array profiling on 9 ETP-LLy and 15 non-ETP T-LLy cases. Compared with non-ETP T-LLy, ETP-LLy showed less frequent deletion of 9p (CKDN2A/B), more frequent deletion of 12p (ETV6) and 1p (RPL22), and more frequent absence of biallelic T-cell receptor ? deletions. Recurrent abnormalities previously described in ETP-ALL such as deletions of 5q and 13q and gain of 6q were not observed in ETP-LLy cases. There were no failures of therapy among the ETP-LLy subtype with a 4-year event-free survival of 100%. Overall, ETP-LLy does not exhibit unifying genetic alterations but shows some distinct genomic features from non-ETP T-LLy suggesting that ETP-LLy may be a distinct entity from non-ETP T-LLy.
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