title: Cutaneous manifestations of human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma: a single-center, retrospective study.
abstract: BACKGROUND
  Limited data exist regarding cutaneous involvement of adult T-cell leukemia/lymphoma (ATLL), particularly in the United States.
  
  
  OBJECTIVE
  We sought to characterize clinical and histopathologic features of ATLL in patients with skin involvement.
  
  
  METHODS
  We retrospectively identified patients with ATLL from a single institution given a diagnosis during a 15-year period (1998-2013). Patients were categorized by the Shimoyama classification and stratified into skin-first, skin-second, and skin-uninvolved courses.
  
  
  RESULTS
  The study population included 17 skin-first, 8 skin-second, and 29 skin-uninvolved cases. Skin-first patients (6 acute, 1 lymphoma, 4 chronic, 6 smoldering) were overwhelmingly of Caribbean origin (94%). They had longer median symptom duration (11.9 vs 1.9 months, P < .001) and overall survival (26.7 vs 10.0 months, P < .001) compared with skin-second/skin-uninvolved patients. Cutaneous lesion morphology at diagnosis included nodulotumoral (35%), multipapular (24%), plaques (24%), patches (12%), and erythroderma (6%). After initial skin biopsy, 14 of 17 received a non-ATLL diagnosis, most commonly mycosis fungoides (47%). Notable histopathologic findings from 43 biopsy specimens included greater than or equal to 20:1 CD4:CD8 ratio (79%), angiocentrism (78%), CD25(+) (71%), large cell morphology (70%), CD30(+) (68%), epidermal infiltration of atypical lymphocytes (67%) forming large Pautrier-like microabscesses (55%), and folliculotropism (65%).
  
  
  CONCLUSIONS
  This was a retrospective, single-center, tertiary referral center study with small sample size.
  
  
  CONCLUSIONS
  Skin-first patients with ATLL in the United States are diagnostically challenging. Familiarity with clinicopathologic features may aid in diagnosis.
authors.affiliation: Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Marchetti
authors.affiliation: Dermatopathology Service, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Melissa P
authors.initials: MP
authors.lastname: Pulitzer
authors.affiliation: Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Weill Cornell Medical College, New York, New York.
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Myskowski
authors.affiliation: Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Stephen W
authors.initials: SW
authors.lastname: Dusza
authors.affiliation: Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Matthew A
authors.initials: MA
authors.lastname: Lunning
authors.affiliation: Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Weill Cornell Medical College, New York, New York.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Horwitz
authors.affiliation: Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Weill Cornell Medical College, New York, New York.
authors.forename: Alison J
authors.initials: AJ
authors.lastname: Moskowitz
authors.affiliation: Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Weill Cornell Medical College, New York, New York. Electronic address: querfelc@mskcc.org.
authors.forename: Christiane
authors.initials: C
authors.lastname: Querfeld
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D003872:Dermatitis / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D015490:HTLV-I Infections / Q000401:mortality / Q000473:pathology*; D015368:Human T-lymphotropic virus 1; D006801:Humans; D016130:Immunophenotyping; D053645:Interleukin-2 Receptor alpha Subunit / Q000032:analysis; D017730:Ki-1 Antigen / Q000032:analysis; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000175:diagnosis; D012189:Retrospective Studies; D012751:Sezary Syndrome / Q000175:diagnosis / Q000473:pathology; D012878:Skin Neoplasms / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D015996:Survival Rate
chemical_list: C508017:IL2RA protein, human; D053645:Interleukin-2 Receptor alpha Subunit; D017730:Ki-1 Antigen
keywords: CD25 expression; CD30 expression; Pautrier-like microabscesses; adult T-cell leukemia/lymphoma; human T-cell lymphotropic virus type-1; immunophenotyping; large cell morphology; skin manifestations