title: CD4+ CD56+ cutaneous neoplasms: a distinct hematological entity? Groupe Français d'Etude des Lymphomes Cutanés (GFELC).
abstract: We report seven cases of particular cutaneous tumors selected from the register of the French Study Group on Cutaneous Lymphomas. The patients (three men, four women) were aged 37-86 years. They initially presented with cutaneous nodules or papules. Three cases presented with regional lymph nodes. Stagings were negative, except for one patient with bone marrow involvement. Histological features were relevant with pleomorphic medium T-cell lymphoma, but these cells exhibited a distinguishing phenotype. They were positive for CD4, CD56, and also CD45, CD43, and HLA-DR. All other T-cell and B-cell markers were negative. The myelomonocytic markers (CD13, CD14, CD15, CD33, CD117, myeloperoxidase, and lysozyme) were negative excepted CD68, which was clearly positive in four cases and weakly in two cases. Others natural killer cell markers (CD16, CD57, TiA1, granzyme B), TdT, and CD34 were negative. Polymerase chain reaction studies did not detect any B or T clonal rearrangement. The cytogenetic studies, performed in five cases, showed a del(5q) in two cases. All patients were treated successfully by polychemotherapy, but relapsed quickly in the skin, between 4 and 28 months. Five patients developed bone marrow involvement, with leukemia in three cases, and they died in 5-27 months. One patient died at 17 months with skin progression. The seventh patient is alive at 33 months, with cutaneous progression. The origin of these cells is unclear. Despite expression of CD4 or CD56, we failed to demonstrate a T-cell, natural killer cell origin. However, CD4 and CD56 are not specific for T or natural killer lineages. Although these two markers are also known to be expressed by monocytic cells, classic myeloid antigens were negative. These seven cases, together with other rare similar cases already reported, seem to represent a distinct entity likely developed from hematological precursor cells.
authors.affiliation: Department of Pathology, Dijon's University Hospital, Centre de Pathologie of Dijon, France.
authors.forename: T
authors.initials: T
authors.lastname: Petrella
authors.forename: S
authors.initials: S
authors.lastname: Dalac
authors.forename: M
authors.initials: M
authors.lastname: Maynadié
authors.forename: F
authors.initials: F
authors.lastname: Mugneret
authors.forename: E
authors.initials: E
authors.lastname: Thomine
authors.forename: P
authors.initials: P
authors.lastname: Courville
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: B
authors.initials: B
authors.lastname: Lenormand
authors.forename: L
authors.initials: L
authors.lastname: Arnould
authors.forename: J
authors.initials: J
authors.lastname: Wechsler
authors.forename: M
authors.initials: M
authors.lastname: Bagot
authors.forename: C
authors.initials: C
authors.lastname: Rieux
authors.forename: J
authors.initials: J
authors.lastname: Bosq
authors.forename: M F
authors.initials: MF
authors.lastname: Avril
authors.forename: A
authors.initials: A
authors.lastname: Bernheim
authors.forename: T
authors.initials: T
authors.lastname: Molina
authors.forename: A
authors.initials: A
authors.lastname: Devidas
authors.forename: M H
authors.initials: MH
authors.lastname: Delfau-Larue
authors.forename: P
authors.initials: P
authors.lastname: Gaulard
authors.forename: D
authors.initials: D
authors.lastname: Lambert
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D014408:Biomarkers, Tumor / Q000032:analysis; D015704:CD4 Antigens / Q000276:immunology*; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D019002:CD56 Antigen / Q000276:immunology*; D017931:DNA Primers / Q000737:chemistry; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D016130:Immunophenotyping; D007621:Karyotyping; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012878:Skin Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*