title: CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Français de Cytogénétique Hématologique.
abstract: CD4(+), CD56(+) DC2 malignancies constitute a novel disease entity, which has recently been shown to arise from a transformed lymphoid-related plasmacytoid dendritic cell (DC2). Diagnosis is primarily based on a particular immunophenotype with tumor cells expressing CD4 and CD56 antigens in the absence of common lymphoid or myeloid lineage markers. Little is currently known about the cytogenetic features of this disease entity. In this setting, the Groupe Français de Cytogénétique Hématologique (GFCH) initiated a cytogenetic study of 18 adults and 3 children with CD4(+), CD56(+) DC2 acute leukemia using conventional and fluorescence in situ hybridization/24-color karyotyping. Clonal, mostly complex chromosome aberrations were found in 14 patients (66%). Six major recurrent chromosomal targets were defined. These were 5q, 12p, 13q, 6q, 15q, and 9, which were involved in 72% (5q), 64% (12p and 13q), 50% (6q), 43% (15q), and 28% (monosomy 9) of cases, respectively. Cytogenetic features can be summarized as follows: (1) gross genomic imbalances (mostly losses) predominate, (2) no single anomaly can be considered as specific, whereas their combination/accumulation is, and (3) both lymphoid and myeloid lineage-associated rearrangements are observed in unusual combinations in the same cell. This is suggestive of complex multistep tumorigenic mechanisms and is supportive of the hypothesis that CD4(+), CD56(+) DC2 acute leukemia may arise from an undifferentiated nonmyeloid nonlymphoid progenitor cell. In conclusion, the present study documents for the first time the existence of a characteristic cytogenetic profile for this novel disease entity.
authors: Laboratoire d'Hématologie Cellulaire et Moléculaire, Centre Hospitalier Universitaire (CHU) Michallon, Grenoble, France. dleroux@chu-grenoble.fr
authors: Dominique
authors: D
authors: Leroux
authors: Francine
authors: F
authors: Mugneret
authors: Mary
authors: M
authors: Callanan
authors: Isabelle
authors: I
authors: Radford-Weiss
authors: Nicole
authors: N
authors: Dastugue
authors: Jean
authors: J
authors: Feuillard
authors: Franseza
authors: F
authors: Le Mée
authors: Ghislaine
authors: G
authors: Plessis
authors: Pascaline
authors: P
authors: Talmant
authors: Nathalie
authors: N
authors: Gachard
authors: Françoise
authors: F
authors: Uettwiller
authors: Marie-Pierre
authors: MP
authors: Pages
authors: Marie-Joëlle
authors: MJ
authors: Mozziconacci
authors: Virginie
authors: V
authors: Eclache
authors: Catherine
authors: C
authors: Sibille
authors: Hervé
authors: H
authors: Avet-Loiseau
authors: Marina
authors: M
authors: Lafage-Pochitaloff
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000368:Aged; D000369:Aged, 80 and over; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D002648:Child; D002869:Chromosome Aberrations; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007621:Karyotyping; D007938:Leukemia / Q000235:genetics* / Q000276:immunology; D008297:Male; D008875:Middle Aged
chemical_list: D015704:CD4 Antigens; D019002:CD56 Antigen