title: Clinical and biologic features of CD4(+)CD56(+) malignancies.
abstract: CD4(+)CD56(+) malignancies are rare hematologic neoplasms, which were recently shown to correspond to the so-called type 2 dendritic cell (DC2) or plasmacytoid dendritic cells. This study presents the biologic and clinical features of a series of 23 such cases, selected on the minimal immunophenotypic criteria defining the DC2 leukemic counterpart, that is, coexpression of CD4 and CD56 in the absence of B, T, and myeloid lineage markers. Clinical presentation typically corresponded to cutaneous nodules associated with lymphadenopathy or spleen enlargement or both. Cytopenia was frequent. Circulating malignant cells were often detected. Massive bone marrow infiltration was seen in 20 of 23 (87%) patients. Most tumor cells exhibited nuclei with a lacy chromatin, a blastic aspect, large cytoplasm-containing vacuoles or microvacuoles beside the plasma membrane, and cytoplasmic expansions resembling pseudopodia. Other immunophenotypic characteristics included both negative (CD16, CD57, CD116, and CD117) and positive (CD36, CD38, CD45 at low levels, CD45RA, CD68, CD123, and HLA DR) markers. The prognosis was rapidly fatal in the absence of chemotherapy. Complete remission was obtained in 18 of 23 (78%) patients after polychemotherapy. Most patients had a relapse in less than 2 years, mainly in the bone marrow, skin, or central nervous system. Considering these clinical and biologic features, the conclusion is made that CD4(+)CD56(+) malignancies constitute a genuine homogeneous entity. Furthermore, some therapeutic options were clearly identified. Finally, relationships between the pure cutaneous indolent form of the disease and acute leukemia as well as with the lymphoid/myeloid origin of the CD4(+)CD56(+) malignant cell are discussed.
authors.affiliation: Service d'Hématologie Biologique, Hôpital Avicenne, Université Paris 13 Bobigny, France. jean.feuillard@avc.ap-hop-paris.fr
authors.forename: Jean
authors.initials: J
authors.lastname: Feuillard
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Jacob
authors.forename: Françoise
authors.initials: F
authors.lastname: Valensi
authors.forename: Marc
authors.initials: M
authors.lastname: Maynadié
authors.forename: Rémy
authors.initials: R
authors.lastname: Gressin
authors.forename: Laurence
authors.initials: L
authors.lastname: Chaperot
authors.forename: Christine
authors.initials: C
authors.lastname: Arnoulet
authors.forename: Françoise
authors.initials: F
authors.lastname: Brignole-Baudouin
authors.forename: Bernard
authors.initials: B
authors.lastname: Drénou
authors.forename: Eliane
authors.initials: E
authors.lastname: Duchayne
authors.forename: Annie
authors.initials: A
authors.lastname: Falkenrodt
authors.forename: Richard
authors.initials: R
authors.lastname: Garand
authors.forename: Emanuelle
authors.initials: E
authors.lastname: Homolle
authors.forename: Bernard
authors.initials: B
authors.lastname: Husson
authors.forename: Emilienne
authors.initials: E
authors.lastname: Kuhlein
authors.forename: Geneviève
authors.initials: G
authors.lastname: Le Calvez
authors.forename: Danielle
authors.initials: D
authors.lastname: Sainty
authors.forename: Marie-France
authors.initials: MF
authors.lastname: Sotto
authors.forename: Franck
authors.initials: F
authors.lastname: Trimoreau
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Béné
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000951:Antigens, Neoplasm / Q000032:analysis / Q000276:immunology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015704:CD4 Antigens / Q000032:analysis / Q000276:immunology*; D019002:CD56 Antigen / Q000032:analysis / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D019337:Hematologic Neoplasms / Q000145:classification* / Q000276:immunology* / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000276:immunology; D009360:Neoplastic Cells, Circulating / Q000276:immunology; D012878:Skin Neoplasms / Q000145:classification / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000951:Antigens, Neoplasm; D015704:CD4 Antigens; D019002:CD56 Antigen