Clinical and molecular characterization of early T-cell precursor acute lymphoblastic leukemia

Wang, Xiaoxue; Wu, Danyang; Zhang, Lijun

doi:10.1097/md.0000000000013856

Cited by 8 publications

(10 citation statements)

References 39 publications

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“…Wang et al reported two cases ETP-ALL, presenting with fever and abnormal hemogram showing blasts, one case presented with lymphadenopathy. 18 LBL cases in a study done by Jain et al presented lymphadenopathy as most common symptom followed by skin involvement, supraclavicular and tonsillar mass. 14 Lymphadenopathy is most prominent presentation in our study mainly seen in non ETP-ALL cases and was not present in ETP-ALL cases.…”

Section: Casementioning

confidence: 96%

Prevalence of early T-cell precursor acute lymphoblastic leukemia among immunophenotypically categorised acute T-cell lymphoblastic leukaemia cases

et al. 2021

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Acute lymphoblastic leukaemia is the most common hematopoietic malignancy in childhood, comprising of B-cell lineage (85%) and T-cell lineage (15%). Recent studies have identified a subtype of T-cell acute lymphoblastic leukaemia (T-ALL) termed “early T-cell precursors (ETP)” recognised as a new provisional entity in 2016 update to the World Health Organization (WHO) classification of acute leukaemia, early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is characterized by a unique immunophenotype and genetic profile and its origin has been found to be from migration of cells from thymus to bone marrow. Hence, our study aims at reporting the prevalence of ETP-ALL among immunophenotypically categorised acute T-cell lymphoblastic leukaemia cases. Present work is a retrospective observation of acute T-cell lymphoblastic leukemias and reporting ETP-ALL cases seen during the period of over two years (from August 2018 to August 2020) received for flowcytometry in the department of Pathology, PGIMS, Rohtak, Haryana. Peripheral blood showed features of acute leukemia and immunophenotyping was performed. Fourteen cases were received for flowcytometry showing features of acute leukemia and immunophenotyping was performed revealing two ETP-ALL cases with positivity for cytCD3, CD7 (T-cell markers), HLA-DR, CD13 (myeloid marker-aberrant expression), sCD34, CD117 (stem cell markers), CD19 (B-cell marker) and dim expression of CD45. This study is a supportive data for immunophenotypic identification of ETP-ALL cases in centres where genetic study and other ancillary techniques are not available. It needs to be differentiated from non ETP-ALLs as this entity has been reported to show treatment failure with the treatment modalities for non ETP-ALLs.

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Section: Casementioning

confidence: 96%

Prevalence of early T-cell precursor acute lymphoblastic leukemia among immunophenotypically categorised acute T-cell lymphoblastic leukaemia cases

et al. 2021

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“… 5 , 11 ETP-ALL is sensitive to cytarabine therapy and high dose cytarabine may be a good regimen for ETP-ALL. 61 , 62 FLAG-IDA regime may be a potential and beneficial schedule for ETP-ALL. 10 , 60 Therefore, after the initial induction failed, we turned to FLAG-IDA combined with sorafenib, a treatment that achieved satisfactory outcomes.…”

Section: Discussionmentioning

confidence: 99%

A New Treatment Strategy for Early T‐Cell Precursor Acute Lymphoblastic Leukemia: A Case Report and Literature Review

Mao¹,

Xue²,

Wang³

et al. 2021

OTT

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Early T‐cell precursor acute lymphoblastic leukemia (ETP-ALL) is an aggressive and extremely fatal subtype of T-cell acute lymphoblastic leukemia (T-ALL), characterized by the similar transcriptional and immunophenotypic profiles to those of early T-cell precursors and positive expressions of myeloid antigens. Besides, the gene expression profile in ETP-ALL is similar to that in myeloid malignancies. The clinical characteristics, treatments and prognoses of ETP-ALL are significantly heterogeneous. In the present study, we reported a 43-year-old female patient who lacked terminal deoxynucleotidyl transferase (TDT) expression in immunophenotype and displayed mutations of fms-like tyrosine kinase-internal tandem duplication (FLT3-ITD), paired-box domain 5 (PAX5) and SH2B adaptor protein 3 (SH2B3) (PAX5 and SH2B3, the genes critical to B cell identity and function), which represent myeloid and precursor B-lineage associated gene mutations, respectively. It was a rare T-ALL or T-lineage case. Because of multiple poor prognostic factors in this case, conventional induction regimens, like hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone), were invalid. The patient showed inadequate response, suggesting that this treatment was not employed on the basis of the immunophenotype. FLAG-IDA regimen (fludarabine, cytarabine [Ara-C], granulocyte-colony stimulating factor [G-CSF] and idarubicin), which is usually applied to eliminate leukemia cells, was administered combining with sorafenib as an effective induction chemotherapy. The case achieved long-term survival following the allogeneic hematopoietic stem cell transplantation (allo-HSCT). We recommend that adult ETP-ALL patients can be treated with a myeloid-oriented chemotherapy (as frontline induction treatment) along with gene-targeting inhibitors, followed by allo-HSCT.

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“…ETP-ALL/LBL has been reported with PB and BM involvement, differing from our patient’s presentation. These patients may have higher rates of central nervous system involvement, stage IV disease, remission failure, and relapse, as well as lower event-free survival and OS compared with T-ALL/LBL patients 1–7,9–13,16,17. When compared with established T-ALL/LBL prognostic markers like prednisone response and MRD, ETP status may be a stronger predictor of poorer outcome 1.…”

Section: Discussionmentioning

confidence: 99%

A Novel Case of Concurrent T-cell and Early T-cell Precursor Lymphoblastic Lymphoma in an Adolescent Female

Treinen

Abu-Arja

Kahwash

et al. 2021

Journal of Pediatric Hematology/Oncology

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In the context of an evolving understanding of early T-cell precursor (ETP) lymphoma and leukemia, we present a case of concurrent T-cell lymphoblastic lymphoma and ETP lymphoma in an adolescent female. To our knowledge, this represents the first reported case of both lymphoblastic lymphoma and ETP lymphoma as distinct and conjoined components of the same neoplasm. As an exception to current literature, our patient had a strictly lymphomatous ETP component with no leukemic manifestation. Her ETP component remained viable following induction, supporting ETP resistance to chemotherapy. The patient remains in remission 4 years postallogeneic matched sibling donor bone marrow transplant.

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Clinical and molecular characterization of early T-cell precursor acute lymphoblastic leukemia

Cited by 8 publications

References 39 publications

Prevalence of early T-cell precursor acute lymphoblastic leukemia among immunophenotypically categorised acute T-cell lymphoblastic leukaemia cases

Prevalence of early T-cell precursor acute lymphoblastic leukemia among immunophenotypically categorised acute T-cell lymphoblastic leukaemia cases

A New Treatment Strategy for Early T‐Cell Precursor Acute Lymphoblastic Leukemia: A Case Report and Literature Review

A Novel Case of Concurrent T-cell and Early T-cell Precursor Lymphoblastic Lymphoma in an Adolescent Female

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