摘要
目的分析蕈样肉芽肿患者的临床特征,提高对本病的认识,指导临床工作。方法回顾性分析1984～2006年我科住院治疗的51例蕈样肉芽肿及其亚型患者的临床资料,包括患者的确诊年龄、皮损特征、皮损组织病理表现、系统表现、误诊、治疗等情况。结果患者平均确诊年龄(44.24±2.05)岁。临床表现多数符合经典的红斑、斑块至肿瘤期的演变过程,亦有多种变异型和亚型的存在。68.6%的患者皮损全身泛发,70.6%的患者伴有瘙痒。组织病理显示68.6%出现嗜表皮现象,52.9%出现Pautriers微脓疡。T细胞受体基因重排的阳性率为81.3%,阳性率与疾病分期无关。64.7%的病例曾被误诊。早期患者经皮肤靶向治疗及生物学治疗可以完全缓解,晚期患者多数给予联合化疗。结论蕈样肉芽肿的临床病理表现多样,临床工作中应提高警惕,注意与其他良、恶性疾病进行鉴别。
Objective To update the clinical tients. Method We retrospectively analyzed the clinical mycosis fungoides in PUMC hospital from 1984 to 2006, characteristics of skin lesions, systemic manifestation, characteristics of mycosis fungoides in Chinese padata of 51 pahents （29 men and 22 women） with to determine the ages at diagnosis, clinicopathologic misdiagnosis and treatment of these patients. Results The mean age was （44.24 ± 2.05 ） years at the diagnosis. Most patients were characterized by the typical evolution of patches, plaques and tumors, with some variations and subtypes. Clinical manifestations included generalized lesions （ 68.6% ） and itchy （ 70.6% ）. Epidermotropism （ 68.6% ） and Pautrier＇s microabscesses （52. 9% ） were common histopathologic features. The positive rate of T-cell receptor gene rearrangement was 81.3%, and was independent of the histological features. Previous misdiagnosis rate was 64.7%. Skin-targeted therapies and biologic therapies were effective approaches to relieve the skin rash at early stage, and combined chemotherapy was typically applied in more advanced cases. Conclusion Mycosis fungoides has various clinical characteristics and careful differential diagnosis should be made in clinical practice.
出处
《中国医学科学院学报》
CAS
CSCD
北大核心
2007年第2期174-180,共7页
Acta Academiae Medicinae Sinicae
关键词
蕈样肉芽肿
临床病理
治疗
mycosis fungoides
clinical pathology
therapy
