Journal of the American Academy of Dermatology · 2015
Sézary syndrome without erythroderma.
СтатьяНазвание и аннотация: Перевод в очереди
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Henn Aurélia, Michel Laurence, Fite Charlotte, Deschamps Lydia, Ortonne Nicolas, Ingen-Housz-Oro Saskia, Marinho Eduardo, Beylot-Barry Marie, Bagot Martine, Laroche Liliane, Crickx Béatrice, Maubec Eve
Аннотация
BACKGROUND: Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement.
OBJECTIVE: We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma.
METHODS: Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied.
RESULTS: Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred.
LIMITATIONS: Retrospective design and small sample size are limitations.
CONCLUSION: Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.
OBJECTIVE: We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma.
METHODS: Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied.
RESULTS: Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred.
LIMITATIONS: Retrospective design and small sample size are limitations.
CONCLUSION: Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.
Предметные рубрики MeSH
Administration, TopicalAdrenal Cortex HormonesAge FactorsAgedAged, 80 and overAntineoplastic Combined Chemotherapy ProtocolsBiopsy, NeedleCombined Modality TherapyDermatitis, ExfoliativeFemaleFollow-Up StudiesFranceHumansImmunohistochemistryMaleMethotrexateMiddle AgedNeoplasm InvasivenessNeoplasm StagingPhotopheresisRare DiseasesRetrospective StudiesRisk AssessmentSampling StudiesSex FactorsSezary SyndromeSkin Neoplasms