Heterogeneity of presenting features and their relation to treatment outcome in 120 children with T-cell acute lymphoblastic leukemia (original) (raw)

Outcome of Children with Standard-Risk T-Lineage Acute Lymphoblastic Leukemia-Comparison among Different Treatment Strategies

Pediatric blood & cancer, 2015

Children with T-lineage acute lymphoblastic leukemia ALL (T-ALL) historically have had inferior outcomes compared with the children with precursor-B ALL (B-ALL). After 1995, the Children's Cancer Group (CCG) treated patients with B- and T-ALL according to the National Cancer Institute (NCI) risk criteria, basing risk stratification on age and white blood cell (WBC) count regardless of immunophenotype. The Pediatric Oncology Group (POG) treated all the patients with T-ALL on separate, generally more intensive protocols than those used to treat the patients with B-ALL. We compared the outcomes of children with T-ALL and NCI standard-risk (SR) criteria treated on CCG and POG trials between 1996 and 2005. CCG SR-ALL 1952 and 1991 enrolled 80 and 86 patients with T-ALL, respectively, utilizing a reduced intensity Berlin-Frankfurt-Münster backbone. Treatment was intensified for slow early responders and only patients with overt central nervous system leukemia received cranial irradiat...

Significance of commonly used prognostic factors differs for children with T cell acute lymphocytic leukemia (ALL), as compared to those with B-precursor ALL. A Pediatric Oncology Group (POG) study

Leukemia, 1999

T cell acute lymphocytic leukemia (T-ALL) and B-precursor ALL differ significantly in the clinical characteristics of the patients at presentation and in laboratory-defined characteristics of the leukemic cells. We assessed for pediatric patients with TALL the relative importance of prognostic factors previously demonstrated to predict outcome in B-precursor ALL. Presenting clinical and laboratory features were correlated with outcome for 441 children 12 months to 21 years of age with previously untreated TALL , registered on the Pediatric Oncology Group (POG) T3 protocol between 1986 and 1992. These TALL prognostic factor analyses were then compared to similar analyses for 1993 patients with B-precursor ALL enrolled during the same time period on the POG ALinC 14 protocol. Quantitative interaction between phenotype and each prognostic factor was studied to determine the relative importance of the prognostic factor for each of the two major immunophenotypes. We also analyzed the importance of maturational stage as a TALL prognostic factor, using a modified Ludwig definition of maturational stage. We conclude that several of the clinical and laboratory prognostic factors, which are used reliably for Bprecursor ALL, are much less predictive in TALL (ie age, WBC, consensus risk group, hyperdiploidy, presence of translocations and CALLA expression). There was no significant difference between the phenotypes in the prognostic importance of race or gender. Our data demonstrate a significant difference in outcome among the three maturational stages of Tcell ALL, with the intermediate group faring best. Using traditional risk group criteria to stratify patients with TALL for therapy may not be appropriate.

Clinical Profile and Induction Outcome of Children with T-cell Acute Lymphoblastic Leukemia and T-cell Lymphoblastic Lymphoma

Pakistan Armed Forces Medical Journal

Objective: To study the clinical profile and induction outcome in children diagnosed with T-cell acute lymphoblastic leukaemia and lymphoblastic lymphoma. Study Design: Cross-sectional study. Place and Duration of Study: Paediatric Oncology Unit, Combined Military Hospital, Rawalpindi Pakistan, from Jan 2012 to Dec 2020. Methodology: One hundered and twenty-one diagnosed patients of T-ALL and LBL were evaluated with bone marrow biopsy and Contrast-enhanced CT scan of the active region after induction chemotherapy with Dexamethasone, Vincristine, Asparaginase and Daunorubicin along with intrathecal Methotrexate. This was carried out on days-8 and 29 to determine the early remission status and end of induction response, respectively. Results: Out of 121 patients, 99(81.8 %) had T–ALL, while 22(18.18%) had LBL. Day-8 assessment showed that 94(77.7%) patients were rapid early responders, 24(19.4%) were slow early responders, while 3(2.4%) patients expired before the day eight assessment...

Role of Minimal Residual Disease in the Clinical Course of T cell Acute Lymphoblastic Leukemia in Pediatric Patients

Journal of Leukemia

Background: Minimal Residual Disease (MRD) is the most important prognostic parameter in pediatric precursor B cell-Acute Lymphoblastic Leukemia (B-ALL). However, the feasibility of flow cytometry in the detection of Minimal Residual Disease (MRD) in T cell-Acute Lymphoblastic Leukemia (T-ALL) is not well defined Objectives: We aimed to investigate the prognostic impact of Flow Cytometry-MRD in TALL measured at different time points post-induction. Patients and Methods: In the current study, the impact of MRD was investigated in 58 newly diagnosed pediatric ALL at day 15, 28 and 42 post induction treatment in relation to other clinical and hematological parameters as well as disease-free survival. Results: At day 15, day 28 and day 42 patients with MRD level ≥ 0.1% had inferior Disease-Free Survival (DFS) compared to patients with lower MRD levels which was significant for day 15, day 28 and 42 (p=0.007, p=0.0148 and p=0.0004 respectively). No association was detected between MRD status and different clinical and laboratory parameters. Conclusion: Post-induction MRD detection is sensitively reflecting the disease progression in pediatric (T-ALL). This is most evident at day 42 followed by day 15.

Prognosis of children with mixed phenotype acute leukemia treated on the basis of consistent immunophenotypic criteria

Haematologica, 2010

Background Mixed phenotype acute leukemia (MPAL) represents a diagnostic and therapeutic dilemma. The European Group for the Immunological Classification of Leukemias (EGIL) scoring system unambiguously defines MPAL expressing aberrant lineage markers. Discussions surrounding it have focused on scoring details, and information is limited regarding its biological, clinical and prognostic significance. The recent World Health Organization classification is simpler and could replace the EGIL scoring system after transformation into unambiguous guidelines. Design and Methods Simple immunophenotypic criteria were used to classify all cases of childhood acute leukemia in order to provide therapy directed against acute lymphoblastic leukemia or acute myeloid leukemia. Prognosis, genotype and immunoglobulin/T-cell receptor gene rearrangement status were analyzed. Results The incidences of MPAL were 28/582 and 4/107 for children treated with acute lymphoblastic leukemia and acute myeloid leukemia regimens, respectively. In immunophenotypic principal component analysis, MPAL treated as T-cell acute lymphoblastic leukemia clustered between cases of non-mixed T-cell acute lymphoblastic leukemia and acute myeloid leukemia, while other MPAL cases were included in the respective non-mixed B-cell progenitor acute lymphoblastic leukemia or acute myeloid leukemia clusters. Analogously, immunoglobulin/T-cell receptor gene rearrangements followed the expected pattern in patients treated as having acute myeloid leukemia (non-rearranged, 4/4) or as having B-cell progenitor acute lymphoblastic leukemia (rearranged, 20/20), but were missing in 3/5 analyzed cases of MPAL treated as having T-cell acute lymphobastic leukemia. In patients who received acute lymphoblastic leukemia treatment, the 5-year event-free survival of the MPAL cases was worse than that of the nonmixed cases (53±10% and 76±2% at 5 years, respectively, P=0.0075), with a more pronounced difference among B lineage cases. The small numbers of MPAL cases treated as T-cell acute lymphoblastic leukemia or as acute myeloid leukemia hampered separate statistics. We compared prognosis of all subsets with the prognosis of previously published cohorts. Conclusions Simple immunophenotypic criteria are useful for therapy decisions in MPAL. In B lineage leukemia, MPAL confers poorer prognosis. However, our data do not justify a preferential use of current acute myeloid leukemia-based therapy in MPAL.

Common Presenting Signs and Symptoms in Children with Acute Lymphoblastic Leukemia

Basic & Clinical Cancer Research, 2020

Background: Acute lymphoblastic leukemia (ALL) is one of the blood cancers responsible for 80% of children's leukemia and is also the most common malignancy in patients aged under 14 years (frequency of 23% among all types of cancers). Regarding the importance of identifying clinical symptoms to diagnose the disease in the early stages, this study is conducted to investigate the symptoms at diagnosis in ALL children. Methods: In this retrospective cohort study, 350 patients aged under 14, referring to four hospitals of Shiraz University of Medical Sciences as reference hospitals in Southern Iran, participated between 2013 and 2019. Their information was collected using patients' records, and the data were analyzed using SPSS version16. Results: Based on the findings of this study, the first clinical manifestations of the disease happened suddenly and acute, occurring within a few days to a maximum of 6 weeks before diagnosis. Fever (70%) and hepatomegaly (60%) were the most common signs and symptoms in patients. However, a significant percentage of ALL patients referred with non-specific symptoms. Conclusion:The results of this study indicate the importance of recognizing common and unusual signs and symptoms based on a complete and thorough history taking and accurate physical examination as well as rare symptoms that may be ignored or misdiagnosed by physicians. The knowledge of common signs and symptoms results in early diagnosis of the disease in early stages.