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

Authors

  • Heidrun Boztug,

    Corresponding author
    1. Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria
    • Correspondence to: Heidrun Boztug, Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Kinderspitalgasse 9, 1090 Vienna, Austria or Michael Dworzak, Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Kinderspitalgasse 9, 1090 Vienna, Austria. E-mail: heidrun.boztug@stanna.at or michael.dworzak@stanna.at.

    Search for more papers by this author
  • Angela Schumich,

    1. Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria
    Search for more papers by this author
  • Ulrike Pötschger,

    1. Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria
    Search for more papers by this author
  • Nora Mühlegger,

    1. Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria
    Search for more papers by this author
  • Alexandra Kolenova,

    1. Department of Pediatric Haematology and Oncology, University Childrens' Hospital and Medical Faculty of Comenius University, Bratislava, Slovakia
    Search for more papers by this author
  • Katarina Reinhardt,

    1. Department of Pediatric Haematology and Oncology, Medical School Hanover, Hanover, Germany
    Search for more papers by this author
  • Michael Dworzak

    Corresponding author
    1. Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria
    • Correspondence to: Heidrun Boztug, Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Kinderspitalgasse 9, 1090 Vienna, Austria or Michael Dworzak, Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Kinderspitalgasse 9, 1090 Vienna, Austria. E-mail: heidrun.boztug@stanna.at or michael.dworzak@stanna.at.

    Search for more papers by this author

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. © 2013 International Clinical Cytometry Society

Ancillary