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Blood, 15 May 2001, Vol. 97, No. 10, pp. 3210-3217

NEOPLASIA

Identification of a leukemic counterpart of the plasmacytoid dendritic cells

Laurence Chaperot, Nathalie Bendriss, Olivier Manches, Rémy Gressin, Marc Maynadie, Franck Trimoreau, Hubert Orfeuvre, Bernadette Corront, Jean Feuillard, Jean-Jacques Sotto, Jean-Claude Bensa, Francine Brière, Joël Plumas, and Marie-Christine Jacob

From the Department of Research and Development, Research Group on Lymphoma, EFS Rhône-Alpes Grenoble, La Tronche, France; Schering Plough Laboratory for Immunological Research, Dardilly, France; Groupe d'Etudes Immunologique des Leucémies, Immunology Laboratory, Medicine Faculty, Vandoeuvre Les Nancy, France; Clinical Hematological Department, Research Group on Lymphoma, Michallon Hospital, Grenoble, France; Hematological Department, Dijon Hospital, Dijon, France; Cellular Hematological Department, Dupuytren Hospital, Limoges, France; Oncology Department, Bourg en Bresse Hospital, Bourg en Bresse, France; Hematological Department, Annecy Hospital, Annecy, France; and Hematological Department, Avicenne Hospital, Bobigny, France.

This work aims to demonstrate that CD4+CD56+ malignancies arise from transformed cells of the lymphoid-related plasmacytoid dendritic cell (pDC) subset. The analysis of malignant cells from 7 patients shows that in all cases, like pDCs, leukemic cells are negative for lineage markers CD3, CD19, CD13, CD33, and CD11c but express high levels of interleukin-3 receptor alpha  chain (IL-3Ralpha ), HLA-DR, and CD45RA. Tumor cells produce interferon-alpha in response to influenza virus, while upon maturation with IL-3 they become a powerful inducer of naive CD4+ T-cell proliferation and promote their T-helper 2 polarization. As pDCs, leukemic cells also express pre-Talpha and lambda-like 14.1 transcripts, arguing in favor of a lymphoid origin. In addition, malignant cells express significant levels of CD56 and granzyme B. Overall, those observations suggest that CD4+CD56+ leukemic cells could represent the malignant counterpart of pDCs, both of which are closely related to B, T, and NK cells.

© 2001 by The American Society of Hematology.
 

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