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Precursor T-Cell Acute Lymphoblastic Leukemia/ Lymphoblastic Lymphoma and Acute Biphenotypic Leukemias

Xin Han MD, Carlos E. Bueso-Ramos MD, PhD
DOI: http://dx.doi.org/10.1309/2QE3A6EKQ8UYDYRC 528-544 First published online: 1 April 2007


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 -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.

Key Words:
  • Precursor T-acute lymphoblastic leukemia/lymphoblastic lymphoma
  • Acute biphenotypic leukemia
  • Immunophenotype
  • Karyotype
  • Molecular findings