Sausville E A, Eddy J L, Makuch R W, Fischmann A B, Schechter G P, Matthews M, Glatstein E, Ihde D C, Kaye F, Veach S R
National Cancer Institute, Bethesda, Maryland.
Ann Intern Med. 1988 Sep 1;109(5):372-82. doi: 10.7326/0003-4819-109-5-372.
To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sézary syndrome.
Retrospective review of a uniformly staged inception cohort.
Single-institution tertiary care center.
152 consecutive patients who had mycosis fungoides with or without the Sézary syndrome within 6 months of the initial definitive diagnosis.
A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients.
Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P less than 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sézary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement.
A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, greater than 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).
确定蕈样肉芽肿或塞扎里综合征初诊时的最佳分期评估方法。
对一个统一分期的起始队列进行回顾性研究。
单机构三级医疗中心。
152例在首次明确诊断后6个月内患有蕈样肉芽肿伴或不伴塞扎里综合征的连续患者。
进行详细的分期评估，包括体格检查、常规实验室检查、胸部X线片、淋巴管造影、外周血涂片、淋巴结活检、骨髓穿刺或活检，以及对部分患者进行肝活检。
蕈样肉芽肿患者初诊时的单因素不良预后特征包括（P<0.01）一个或多个皮肤肿瘤或全身性红皮病、淋巴结病、血涂片中有塞扎里细胞、淋巴结结构破坏、嗜酸性粒细胞增多和内脏受累。多因素分析中重要的独立预后因素是内脏疾病的存在和皮肤受累类型。
基于皮肤、淋巴结、血液和内脏部位组织病理学评估的分期系统比仅对皮肤疾病和淋巴结病进行临床评估能提供更全面的预后信息。患者在初次就诊时可分为三个预后组：低风险患者，仅患有斑块状皮肤疾病，无淋巴结、血液或内脏受累（中位生存期，大于12年）；中风险患者，患有皮肤肿瘤、红皮病或伴有淋巴结或血液受累的斑块状疾病，但无内脏疾病或淋巴结结构破坏（中位生存期，5年）；高风险患者，有内脏受累或淋巴结结构破坏（中位生存期，2.5年）。
