Poor Response to Standard Chemotherapy in Early T-precursor (ETP)-ALL: A Subtype of T-ALL Associated with Unfavourable Outcome: A Brief Report

Abstract

Early T-precursor (ETP)-ALL, a type of T-ALL, is a new pathobiologic entity with distinct immunophenotype (CD1a -, CD8 -, CD5 weak/absent with stem-

Early precursor T-cell acute lymphoblastic leukemia: current paradigms and evolving concepts

“…In a small series from India, outcomes of six patients were described, and only one responded to intensive chemotherapy. 30 In another report, four patients received FLAG-IDA [fludarabine, cytarabine (Ara-C), granulocyte-colony stimulating factor (G-CSF) and idarubicin] early on after induction, with three achieving CR without MRD. 31 One patient had a reduction in leukemic burden and subsequently received high dose cytarabine with sorafenib.…”

T- Acute lymphoblastic leukemia: Does our understanding of molecular pathogenesis will impact future treatment?

“…Patients with ETP-ALL have a particularly poor response to chemotherapy, very high risk of remission failure and subsequent relapse indicating the need for alternative approaches to treatment. On the basis of this, ETP-ALL is regarded as a high-risk subgroup and allogeneic hematopoietic SCT is recommended in first complete remission (CR) (Iqbal et al, 2014).…”

Comprehensive Overview of Gene Rearrangements in Childhood T-Cell Acute Lymphoblastic Leukaemia

“…In recent years, a subgroup of early T-cell precursor (ETP)-ALL has been identified among patients, which is characterized by worse prognosis and chemoresistance in comparison to non-ETP-ALL [ 1 ]. ETP is derived from thymocytes, which are capable of differentiating into T cells and marrow cells, which proves their hematopoietic nature [ 19 , 20 ]. In paediatric T-ALL/acute lymphoblastic lymphoma (LBL), the ETP-ALL subgroup represents 11–13% cases, while in adults it constitutes 7.4%, but some studies indicate that ETP-ALL occurs in 15 and 10–30%, respectively [ 2 , 20 , 21 , 22 , 23 ].…”

“…ETP is derived from thymocytes, which are capable of differentiating into T cells and marrow cells, which proves their hematopoietic nature [ 19 , 20 ]. In paediatric T-ALL/acute lymphoblastic lymphoma (LBL), the ETP-ALL subgroup represents 11–13% cases, while in adults it constitutes 7.4%, but some studies indicate that ETP-ALL occurs in 15 and 10–30%, respectively [ 2 , 20 , 21 , 22 , 23 ]. Patients with ETP-ALL are significantly older at diagnosis, have a higher percentage of blasts in the marrow or peripheral blood, and circulating white blood cell (WBC) counts less than 50 × 109/L in comparison to patients with non-ETP-ALL [ 24 ].…”