Smith Benjamin D, Wilson Lynn D
Yale University School of Medicine, New Haven, Connecticut, USA.
Oncology (Williston Park). 2003 Sep;17(9):1281-8.
Mycosis fungoides is a low-grade lymphoproliferative disorder caused by CD4+ lymphocytes. It is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumors, or erythroderma. Diagnosis of early mycosis fungoides can be difficult due to the nonspecific nature of cutaneous and histologic findings. However, recent advances in the application of histologic criteria, coupled with molecular biology tools such as immunophenotyping and polymerase chain reaction, have improved diagnostic accuracy. Independent prognostic factors include the extent and nature of skin involvement, the presence of extracutaneous disease, blood involvement, age > or = 60 years, and lactate dehydrogenase elevation. Accordingly, patients with limited patches and/or plaques (stage IA or IIA) experience long-term survival comparable to that of matched controls. The median survival is 11 years for patients with extensive patch/plaque (stage IB or IIA), 3.2 years for those with cutaneous tumors (stage IIB), 4.6 years for those with erythroderma (stage III), 1.2 years for those with pathologic nodal involvement (stage IVA), and 0.9 years for those with visceral disease (stage IVB). Over time, mycosis fungoides may progress to Sézary syndrome or transform to large-cell histology.
蕈样肉芽肿是一种由CD4 +淋巴细胞引起的低度淋巴细胞增殖性疾病。它是最常见的皮肤T细胞淋巴瘤类型。通常，肿瘤性T细胞定位于皮肤并产生斑块、斑片、肿瘤或红皮病。由于皮肤和组织学表现的非特异性，早期蕈样肉芽肿的诊断可能具有挑战性。然而，组织学标准应用的最新进展，加上免疫表型分析和聚合酶链反应等分子生物学工具，提高了诊断准确性。独立的预后因素包括皮肤受累的程度和性质、皮肤外疾病的存在、血液受累、年龄≥60岁以及乳酸脱氢酶升高。因此，局限性斑块和/或斑片（IA期或IIA期）患者的长期生存率与匹配的对照组相当。广泛斑块/斑片患者（IB期或IIB期）的中位生存期为11年，皮肤肿瘤患者（IIB期）为3.2年，红皮病患者（III期）为4.6年，病理淋巴结受累患者（IVA期）为1.2年，内脏疾病患者（IVB期）为0.9年。随着时间的推移，蕈样肉芽肿可能进展为Sezary综合征或转化为大细胞组织学类型。
