Cutaneous manifestations of human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma: A single-center, retrospective study

Michael A. Marchetti, Melissa P. Pulitzer, Patricia L. Myskowski, Stephen W. Dusza, Matthew A. Lunning, Steven M. Horwitz, Alison J. Moskowitz, Christiane Querfeld

Research output: Contribution to journalArticle

20 Scopus citations

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%). Limitations: This was a retrospective, single-center, tertiary referral center study with small sample size. Conclusion: Skin-first patients with ATLL in the United States are diagnostically challenging. Familiarity with clinicopathologic features may aid in diagnosis.

Original languageEnglish (US)
Pages (from-to)293-301.e2
JournalJournal of the American Academy of Dermatology
Volume72
Issue number2
DOIs
StatePublished - 2015

Keywords

  • Adult T-cell leukemia/lymphoma
  • CD25 expression
  • CD30 expression
  • Human T-cell lymphotropic virus type-1
  • Immunophenotyping
  • Large cell morphology
  • Pautrier-like microabscesses
  • Skin manifestations

ASJC Scopus subject areas

  • Dermatology

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