Skin manifestations of adult T-cell leukemia/lymphoma: clinical, cytological and immunological features.
Adult T-cell leukemia/lymphoma (ATLL) is a malignancy of mature T cells caused by human T-cell leukemia virus type I. The endemic areas include Japan, especially in Kyushu, the Caribbean, Papua New Guinea, South America and Africa. Approximately 50% of ATLL patients exhibit skin manifestations. Cytologically, ATLL tumor cells are characterized by CD4(+) CD25(+) regulatory T-cell phenotype, high expressions of CCR4, and programmed cell death (PD)-1 and PD-ligand 1. The skin eruptions are categorized into six types: patch, plaque, multipapular, nodulotumoral, erythrodermic and purpuric. The overall survival of the eruption-bearing patients was poorer than that of the non-eruption-bearing patients in acute, chronic and smoldering types, but the survival levels of both groups were comparable in lymphoma type. The prognosis was poor in the order of: erythrodermic, nodulotumoral, multipapular/purpuric, plaque, then patch. Multivariate analysis revealed that the eruption type is an independent prognostic factor for ATLL. Patients may have other skin manifestations, secondary and infective lesions.
Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Yoshiki
Y
Tokura
Yu
Y
Sawada
Takatoshi
T
Shimauchi
D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000276:immunology / Q000473:pathology*; D011379:Prognosis; D012867:Skin / Q000473:pathology*
CCR4; adult T-cell leukemia/lymphoma; prognosis; regulatory T cell; skin eruption