Blast cell deficiency of CD11a as a marker of acute megakaryoblastic leukemia and transient myeloproliferative disease in children with and without Down syndrome

Cytometry B Clin Cytom. 2013 Nov-Dec;84(6):370-8. doi: 10.1002/cyto.b.21082. Epub 2013 Feb 28.

Abstract

Background: The classification of acute myeloid leukemia (AML) FAB subtype M7 relies on immunophenotypic assessment. CD41 is expressed throughout all stages of maturation of megakaryocytes and has therefore been described as a specific blast cell marker in AML M7 as well as in transient myeloproliferative disease (TMD) of patients with Down syndrome (DS). However, technical difficulties underlie the need for new markers for these entities.

Methods: We evaluated the expression of human lymphocyte function-associated antigen 1 (CD11a) in a large cohort of pediatric AML and TMD patients (n = 91) of the Austrian AML-BFM 98 and 2004 studies.

Results: We found a consistent deficiency of CD11a as assessed by mean fluorescence intensity in all patients with non-DS AML M7 (n = 8) and M6 (n = 1), all cases of classical DS-AML (n = 12) as well as TMD (n = 15) that was statistically significant in comparison to non-DS AML M0-M5 patients (n = 55; P < 0.001, sensitivity 100%). Only three of 55 Non-DS M0-5 patients were CD11a deficient (specificity 95%). Monocytic leukemias (M4/5) and normal monocytes typically showed a high CD11a expression, FAB types M1/2 and normal neutrophils an intermediate expression level, while all M3 leukemias were rather low in CD11a expression.

Conclusions: We conclude, that deficiency of CD11a expression should be added to the diagnostic criteria of AML-M7, classical DS-AML and TMD.

Keywords: CD11a; Down syndrome; M7; acute myeloid leukemia; flow cytometry; transient myeloproliferative disease.

MeSH terms

  • Biomarkers / blood
  • Bone Marrow Cells / pathology
  • CD11a Antigen / blood
  • CD11a Antigen / genetics*
  • CD11a Antigen / metabolism
  • Child, Preschool
  • Down Syndrome / blood
  • Down Syndrome / complications
  • Down Syndrome / genetics*
  • Down Syndrome / pathology
  • Female
  • Flow Cytometry
  • Gene Expression Regulation
  • Humans
  • Infant
  • Infant, Newborn
  • Leukemia, Megakaryoblastic, Acute / blood
  • Leukemia, Megakaryoblastic, Acute / complications
  • Leukemia, Megakaryoblastic, Acute / genetics*
  • Leukemia, Megakaryoblastic, Acute / pathology
  • Male
  • Monocytes / metabolism
  • Monocytes / pathology
  • Myeloproliferative Disorders / blood
  • Myeloproliferative Disorders / genetics*
  • Myeloproliferative Disorders / pathology

Substances

  • Biomarkers
  • CD11a Antigen

Supplementary concepts

  • Transient Myeloproliferative Disorder of Down Syndrome