Details

Autor(en) / Beteiligte

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

Titel

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

Ist Teil von

• Journal of the American Academy of Dermatology, 2015-02, Vol.72 (2), p.293-301.e2

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2015

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.