title: Blast cell deficiency of CD11a as a marker of acute megakaryoblastic leukemia and transient myeloproliferative disease in children with and without Down syndrome.
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.
authors: Department of Pediatrics, St. Anna Kinderspital and Children's Cancer Research Institute, Medical University of Vienna, Vienna, Austria.
authors: Heidrun
authors: H
authors: Boztug
authors: Angela
authors: A
authors: Schumich
authors: Ulrike
authors: U
authors: Pötschger
authors: Nora
authors: N
authors: Mühlegger
authors: Alexandra
authors: A
authors: Kolenova
authors: Katarina
authors: K
authors: Reinhardt
authors: Michael
authors: M
authors: Dworzak
mesh_terms: D015415:Biomarkers / Q000097:blood; D001854:Bone Marrow Cells / Q000473:pathology; D040881:CD11a Antigen / Q000097:blood / Q000235:genetics* / Q000378:metabolism; D002675:Child, Preschool; D004314:Down Syndrome / Q000097:blood / Q000150:complications / Q000235:genetics* / Q000473:pathology; D005260:Female; D005434:Flow Cytometry; D005786:Gene Expression Regulation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007947:Leukemia, Megakaryoblastic, Acute / Q000097:blood / Q000150:complications / Q000235:genetics* / Q000473:pathology; D008297:Male; D009000:Monocytes / Q000378:metabolism / Q000473:pathology; D009196:Myeloproliferative Disorders / Q000097:blood / Q000235:genetics* / Q000473:pathology
chemical_list: D015415:Biomarkers; D040881:CD11a Antigen
keywords: CD11a; Down syndrome; M7; acute myeloid leukemia; flow cytometry; transient myeloproliferative disease