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Blood, 1 February 2005, Vol. 105, No. 3, pp. 1256-1264. Prepublished online as a Blood First Edition Paper on September 23, 2004; DOI 10.1182/blood-2004-06-2416.
Submitted June 28, 2004
Etablissement Francais du Sang Bourgogne Franche-Comte, Besancon, France; Unite Mixte EFS/Universite EA2284/Inserm U645, IFR133, Besancon, France * Corresponding author; email: philippe.saas{at}efs.sante.fr.
A new entity of acute leukemia co-expressing CD4+CD56+ markers without any other lineage-specific markers has been recently identified as arising from lymphoid-related plasmacytoid dendritic cells (pDC). Cells from a patient with such CD4+CD56+ lineage negative leukemia was unexpectedly found in our laboratory to also express the myeloid marker CD33. To confirm the diagnosis of pDC leukemia despite the CD33 expression, we demonstrated that the leukemic cells indeed exhibited pDC phenotypic and functional properties. In 7 out of 8 other patients with CD4+CD56+ pDC malignancies, we were able to confirm that the tumor cells expressed CD33 although with variable expression levels. CD33 expression was shown by flow cytometry, RT-PCR and immunoblot analysis. Furthermore, CD33 monoclonal antibody (mAb) stimulation of purified CD4+CD56+ leukemic cells led to cytokine secretion thus confirming the presence of a functional CD33 on these leukemic cells. Moreover, we found that circulating pDC in normal subjects also weakly express CD33. Overall, our results demonstrate that the expression of CD33 on CD4+CD56+ lineage negative cells should not exclude the diagnosis of pDC leukemia and underline that pDC specific markers should be used at diagnosis for CD4+CD56+ malignancies.
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