关键词: Cutaneous T-cell lymphoma Granuloma Granulomatous mycosis fungoides

Mesh : Humans Mycosis Fungoides / pathology Skin Neoplasms / pathology secondary Lung Neoplasms / pathology Prognosis Disease Progression

来  源:   DOI:10.1159/000537893

Abstract:
BACKGROUND: Granulomatous mycosis fungoides (GMF) is a rare form of cutaneous T-cell lymphoma characterized by a granulomatous inflammatory infiltrate.
OBJECTIVE: The impact of granulomatous inflammation on the prognosis of the disease remains controversial as there have been both favorable and unfavorable outcomes documented.
METHODS: We performed a systematic review of 116 GMF cases previously described in the literature.
RESULTS: In contrast to the classic Alibert-Bazin type of mycosis fungoides (MF), cutaneous lesions in GMF tend to involve distal extremities (lower legs, feet, hands) early in the disease course. In the literature, 30% of GMF patients developed organ metastasis, most frequently to the lung. The median time to stage progression was 25 months.
CONCLUSIONS: GMF is an aggressive form of MF. Therefore, screening for distant metastases should be considered at presentation and repeated during follow-up.
摘要:
背景:肉芽肿样真菌病(GMF)是一种罕见形式的皮肤T细胞淋巴瘤,其特征是肉芽肿性炎症浸润。
目的:肉芽肿性炎症对疾病预后的影响仍存在争议,因为有有利和不利的结局记录。
方法:我们对文献中先前描述的116例GMF病例进行了系统评价。
结果:与经典的Alibert-Bazin型真菌病(MF)相反,GMF的皮肤病变倾向于累及远端四肢(小腿,脚,手)在疾病进程的早期。在文学中,30%的GMF患者发生器官转移,最常见的是肺部。进展期的中位时间为25个月。
结论:GMF是真菌的侵袭性形式。因此,应在就诊时考虑筛查远处转移,并在随访期间重复筛查.
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