4例大细胞转化的蕈样肉芽肿病情分析及文献复习

Four Cases of Granuloma Fungoides with Large Cell Transformation and Literature Review

目的总结大细胞转化的蕈样肉芽肿(MF)发病特点及危险因素,加强对MF发生大细胞转化的认识。方法回顾性分析4例大细胞转化的MF患者的病情进展和诊疗过程,并结合相关文献复习。结果4例患者早期临床表现各异,1例患者初发病即诊断为MF,余3例均出现早期误诊;2例男性患者初发病时处于青年期,病程久,确定发生大细胞转化的病程远高于2例女性患者;随访半年有2例患者皮损有复发加重,无其他系统性病变。结论MF临床表现多样,呈惰性发展,性别、年龄、乳酸盐脱氢酶(LDH)对疾病进展和发生大细胞转化有一定影响,临床中需密切关注带这些危险因素的患者,定期随访,早发现,早调整治疗方案。

Objective In order to summarize the pathogenic characteristics and risk factors of mycosis fungoides(MF)with large cell transformation,and to strengthen the understanding of the large cell transformation of MF.Methods The disease progression and diagnosis and treatment process of four patients with mycosis fungoides transformed by large cells were analyzed retrospectively,and the related literatures were reviewed.Results The early clinical manifestations of four patients were different.One patient was diagnosed as MF at the first onset,and the other three patients were misdiagnosed at the early stage.Two male patients were in their youth at the time of initial onset and had a long course of disease.It was confirmed that the course of disease with large cell transformation was much higher than that of two female patients.During the follow-up period of six months,two patients suffered from recurrence and aggravation of skin lesions without other systemic diseases.Conclusion MF is characterized by various clinical manifestations and inert development.Sex,age and lactate dehydrogenase(LDH)have certain effects on disease progression and large cell transformation.Patients with these risk factors should be paid close attention to in clinical practice,and regular follow-up,early detection and early adjustment of treatment plan are required.