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皮肤T细胞淋巴瘤患者的前瞻性分期评估。皮肤外播散高频率的证明。

Prospective staging evaluation of patients with cutaneous T-cell lymphomas. Demonstration of a high frequency of extracutaneous dissemination.

作者信息

Bunn P A, Huberman M S, Whang-Peng J, Schechter G P, Guccion J G, Matthews M J, Gazdar A F, Dunnick N R, Fischmann A B, Ihde D C, Cohen M H, Fossieck B, Minna J D

出版信息

Ann Intern Med. 1980 Aug;93(2):223-30. doi: 10.7326/0003-4819-93-2-223.

Abstract

A prospective pretreatment staging evaluation was done on 49 consecutive patients with mycosis fungoides or the Sézary syndrome to study patterns of disease spread and prognostic factors. Routine staging procedures included complete blood count, blood chemistries, chest roentgenogram, lymphangiogram, radionuclide scans, bone marrow aspiration and biopsy, liver biopsy, and lymph node biopsy. Special evaluations included cytogenetic analysis, electron microscopy, and T-cell cytology. Extracutaneous lymphoma was documented by light microscopy in 51% of patients and by the three special procedures in 88%. Extracutaneous lymphoma was most frequent in blood and lymph nodes; 18% of patients had visceral involvement. Patients with generalized erythroderma had a higher frequency of extracutaneous disease than did patients with cutaneous plaques and tumors by both light microscopy and special studies. Survival was directly related to the type of skin involvement and the presence or absence of extracutaneous disease. Systemic dissemination of cutaneous T-cell lymphoma is frequent, generally asymptomatic, and develops early via the circulation. These findings may explain why cutaneous therapies are associated with a high frequency of relapse.

摘要

对49例连续性蕈样肉芽肿或塞扎里综合征患者进行了前瞻性治疗前分期评估,以研究疾病传播模式和预后因素。常规分期程序包括全血细胞计数、血液化学检查、胸部X线检查、淋巴管造影、放射性核素扫描、骨髓穿刺和活检、肝活检以及淋巴结活检。特殊评估包括细胞遗传学分析、电子显微镜检查和T细胞细胞学检查。通过光学显微镜在51%的患者中记录到皮肤外淋巴瘤,通过三种特殊检查在88%的患者中记录到皮肤外淋巴瘤。皮肤外淋巴瘤在血液和淋巴结中最为常见;18%的患者有内脏受累。无论是通过光学显微镜还是特殊研究,泛发性红皮病患者的皮肤外疾病发生率均高于皮肤斑块和肿瘤患者。生存率与皮肤受累类型以及是否存在皮肤外疾病直接相关。皮肤T细胞淋巴瘤的全身播散很常见,通常无症状,并通过循环早期发生。这些发现可能解释了为什么皮肤治疗与高复发率相关。

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