Precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma and acute biphenotypic leukemias

Am J Clin Pathol. 2007 Apr;127(4):528-44. doi: 10.1309/2QE3A6EKQ8UYDYRC.

Abstract

Session 4 of the 2005 Society of Hematopathology/European Association for Haematopathology Workshop focused on case presentations of precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma (pre-T ALL/LBL) and acute biphenotypic leukemia. Pre-T ALL represents approximately 15% of childhood and 25% of adult ALL cases. Pre-T LBL comprises 85% to 90% of LBL and frequently manifests as a mediastinal mass. Gene expression studies have shown distinct subtypes of LYL1+, HOX11+, TAL1+, and MLL+ pre-T ALL/LBL. HOX11 overexpression may correlate with a good prognosis in adult pre-T ALL. ABL gene amplification and NOTCH1 gene mutations in subsets of pre-T ALL/LBL suggest patients may benefit from therapy with tyrosine kinase and gamma-secretase inhibitors, respectively. Acute biphenotypic leukemias are characterized by a single population of blasts that express myeloid, T- or B-lineage antigens in various combinations and account for fewer than 4% of all acute leukemias. The blasts have a high incidence of chromosome abnormalities. An accurate diagnosis of pre-T ALL/LBL and acute biphenotypic leukemia requires a multiparametric approach, including examination of morphologic features, immunophenotype, clinical characteristics, and cytogenetic and molecular findings.

Publication types

  • Congress

MeSH terms

  • Biomarkers, Tumor / analysis*
  • Chromosome Aberrations
  • Diagnosis, Differential
  • Gene Expression Profiling
  • Humans
  • Immunohistochemistry
  • Leukemia, Lymphoid / classification
  • Leukemia, Lymphoid / diagnosis*
  • Leukemia, Lymphoid / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / classification
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics

Substances

  • Biomarkers, Tumor