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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 chain (IL-3R ), HLA-DR, and CD45RA. Tumor cells produce
interferon- 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-T 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.

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