title: Long-term follow-up and management of small and medium-sized CD4+T cell lymphoma and CD8+lymphoid proliferations of acral sites: a multicenter experience.
abstract: BACKGROUND
  Primary cutaneous CD4+small-medium pleomorphic T cell lymphoma (SMPTCL) is a low-grade cutaneous T cell lymphoma. Its clinical and histopathologic features are comparable with those of CD8+lymphoid proliferations (LPs) of the ear and acral sites.
  
  
  OBJECTIVE
  We performed a retrospective analysis of patients with CD4+SMPTCL or CD8+LP to elucidate the clinical course, prognosis, and outcomes.
  
  
  METHODS
  Demographic, clinical, and treatment data were reviewed. Histopathologic data based on architectural, cytomorphologic, and immunohistochemical features were assessed. Immunohistochemical staining for T and B cell markers was evaluated.
  
  
  RESULTS
  A total of 25 patients including 22 with CD4+SMPTCL and three with CD8+LP were identified. All patients presented with a single lesion, predominantly on the head, neck, or upper trunk (84%). No patients showed extracutaneous disease at any evaluation. The most common histopathologic changes showed a dense nodular infiltrate of small cells with hyperchromatic nuclei without significant follicular or adnexal involvement. Patients were treated with excision (48%), local radiation (28%), or topical or intralesional steroids (24%). All patients achieved complete resolution of disease. Five patients demonstrated cutaneous relapse at new sites.
  
  
  CONCLUSIONS
  The CD4+SMPTCL/CD8+LP subgroup usually presents with solitary lesions and demonstrates an indolent clinical course. Typical presentation, classic histopathology, widespread expression of follicular T helper cell markers, and loss of a T cell antigen are diagnostic features of CD4+SMPTCL, whereas monomorphous CD8+infiltrate without follicular T helper cell markers is consistent with CD8+LP. Local skin-directed therapy is appropriate in these patients.
authors.affiliation: Dermatology Service, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Pooja
authors.initials: P
authors.lastname: Virmani
authors.affiliation: Dermatology Service, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Sarah
authors.initials: S
authors.lastname: Jawed
authors.affiliation: Dermatology Service, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Myskowski
authors.affiliation: Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Horwitz
authors.affiliation: Dermatology Service, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Anna
authors.initials: A
authors.lastname: Skripnik Lucas
authors.affiliation: Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Alison
authors.initials: A
authors.lastname: Moskowitz
authors.affiliation: Department of Pathology, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA.
authors.forename: Melissa
authors.initials: M
authors.lastname: Pulitzer
authors.affiliation: Departments of Hematology and Hematopoietic Cell Transplantation, City of Hope Comprehensive Cancer Center, Duarte, CA, USA.
authors.forename: Jasmine
authors.initials: J
authors.lastname: Zain
authors.affiliation: Departments of Hematology and Hematopoietic Cell Transplantation, City of Hope Comprehensive Cancer Center, Duarte, CA, USA.
authors.forename: Steven T
authors.initials: ST
authors.lastname: Rosen
authors.affiliation: Dermatology Service, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY, USA. cquerfeld@coh.org.|Department of Pathology, City of Hope Comprehensive Cancer Center, Duarte, CA, USA. cquerfeld@coh.org.
authors.forename: Christiane
authors.initials: C
authors.lastname: Querfeld
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015496:CD4-Positive T-Lymphocytes; D018414:CD8-Positive T-Lymphocytes; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000473:pathology* / Q000628:therapy; D006801:Humans; D035002:Lower Extremity; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology* / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000473:pathology* / Q000628:therapy; D013997:Time Factors; D060726:Torso; D047368:Tumor Burden; D034941:Upper Extremity; D055815:Young Adult
keywords: cutaneous T cell lymphoma; cutaneous lymphoma