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Pre-B-Cell Acute Lymphoblastic Leukemia in Childhood.

W M Crist1, A J Carroll2, C H Pui1

  • 1a Department of Hematology/Oncology, St. Jude Children's, Research Hospital Division of Hematology/Oncology, University of Tennessee, Memphis, College of Medicine, Memphis, Tenn and the Pediatric Oncology Group, St Louis, Mo.

Leukemia & Lymphoma
|July 27, 2016
PubMed
Summary

Childhood acute lymphoblastic leukemia (ALL) with the pre-B-cell phenotype, especially those with the t(1;19) translocation, presents challenges. However, aggressive treatment may overcome poor prognoses, and molecular diagnostics are advancing rapidly.

Keywords:
Pre-B ALLimmunophenotypeprognostic factors in ALL

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Area of Science:

  • Pediatric Oncology
  • Hematology
  • Molecular Biology

Background:

  • The pre-B-cell (clgM) phenotype accounts for approximately 20% of childhood acute lymphoblastic leukemia (ALL) cases.
  • This phenotype is frequently associated with chromosomal translocations, particularly t(1;19)(q23;p13).
  • Historically, this ALL subtype has been linked to poor treatment outcomes and high-risk features.

Purpose of the Study:

  • To investigate the prognostic implications of the pre-B-cell ALL phenotype, specifically the t(1;19) translocation.
  • To explore the impact of aggressive treatment strategies on outcomes for patients with t(1;19) ALL.
  • To highlight recent molecular insights into the pathogenesis of pre-B-cell ALL and the potential of molecular diagnostics.

Main Methods:

  • Phenotypic analysis of childhood ALL cases.
  • Cytogenetic analysis to identify chromosomal translocations, including t(1;19).
  • Review of treatment outcomes and prognostic factors.
  • Molecular analyses including recombinant DNA technology (polymerase chain reaction).

Main Results:

  • The t(1;19) translocation is a primary driver of the poor prognosis previously associated with the pre-B-cell ALL phenotype.
  • Preliminary data suggest that intensive treatment regimens may mitigate the adverse prognostic impact of the t(1;19) translocation.
  • Molecular studies are providing novel insights into the pathogenesis of t(1;19) ALL.

Conclusions:

  • The t(1;19) translocation is the key adverse prognostic factor in pre-B-cell ALL.
  • Aggressive therapeutic approaches show promise in improving outcomes for patients with t(1;19) ALL.
  • Advances in molecular diagnostics, such as PCR, are expected to enhance diagnosis and minimal residual disease detection for this ALL subtype.