title: Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with KIR3DL2 and NKp46 expression in a human immunodeficiency virus carrier.
abstract: Primary cutaneous aggressive epidermotropic T-cell lymphoma (PCAETCL) is a very rare lymphoma characterized by rapidly growing necrotic cutaneous lesions with an epidermotropic CD8+ T-cell neoplastic infiltrate observed histopathologically. It is associated with a very poor outcome, despite aggressive multi-agent chemotherapy. We report a 49-year-old human immunodeficiency virus (HIV)-infected patient who developed PCAETCL with associated marked vascular injury leading to diffuse purpuric and necrotic lesions complicated by recalcitrant hemophagocytic activation syndrome. The lymphoma strongly and diffusely expressed CD158k/KIR3DL2 at the protein and transcript level and NKp46 transcripts, in addition to CD8 and cytotoxic proteins. We observed a diffuse CD158k/KIR3DL2 protein expression in another case of PAETCL, not associated with immunodeficiency, which was used as a positive control. PCAETCL can develop in HIV-infected patients and may present in vasculitis-like fashion. The possible role of immunosuppression and/or HIV in oncogenesis can be postulated, as patients infected with HIV may develop anti-HIV cytotoxic CD8+ lymphoproliferations. The frequency of CD158k/KIR3DL2 and NKp46 expression in PCAECL remains to be studied in a series of cases, and may represent interesting targets for future treatments.
authors.affiliation: Département de Pathologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Raymond
authors.initials: R
authors.lastname: Karkouche
authors.affiliation: Département de Dermatologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Immunologie Biologique, Hôpital Henri Mondor - A. Chenevier, Créteil, France.
authors.forename: Sabine
authors.initials: S
authors.lastname: Le Gouvello
authors.affiliation: Département de Pathologie, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Charlotte
authors.affiliation: Département de Pathologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Marina
authors.initials: M
authors.lastname: Thomas
authors.affiliation: Département de Dermatologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Ouidad
authors.initials: O
authors.lastname: Zehou
authors.affiliation: Immunologie Biologique, Hôpital Henri Mondor - A. Chenevier, Créteil, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Frenkel
authors.affiliation: Immunologie Clinique, Hôpital Saint-Louis, Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Boutboul
authors.affiliation: Département de Dermatologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Chosidow
authors.affiliation: Département de Maladies Infectieuse, Hôpital Pitié-Salpêtrière, Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Caumes
authors.affiliation: Département de Pathologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaulard
authors.affiliation: Département de Pathologie, Hôpital Henri Mondor, Université paris Est, Créteil, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ortonne
mesh_terms: D001706:Biopsy; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology*; D002353:Carrier State / Q000473:pathology* / Q000821:virology; D006678:HIV / Q000302:isolation & purification*; D015658:HIV Infections / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D055627:Natural Cytotoxicity Triggering Receptor 1 / Q000096:biosynthesis*; D011379:Prognosis; D054347:Receptors, KIR3DL2 / Q000096:biosynthesis*; D012878:Skin Neoplasms / Q000473:pathology / Q000821:virology
chemical_list: C515084:KIR3DL2 protein, human; C112260:NCR1 protein, human; D055627:Natural Cytotoxicity Triggering Receptor 1; D054347:Receptors, KIR3DL2
keywords: HIV; KIR3DL2; NKp46; aggressive; cutaneous lymphoma