A Case of Mycosis Fungoides with Gastric and Central Nervous System Involvement.
A Case of Mycosis Fungoides with Gastric and Central Nervous System Involvement.
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累及胃和中枢神经系统的蕈样肉芽肿一例。
DOI：
10.1111/1346-8138.13820
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发表时间：
2017
期刊：
影响因子：
3.1
通讯作者：
Sato S
中科院分区：
文献类型：
--
作者：
Senda N;Miyagaki T;Oka T;Ito Y;Katayama K;Kamiyama T;Sugaya M;Sato S
亲爱的编辑：真菌样肉芽肿(MF)通常具有较长的临床病程，内脏受累很少见。对于皮肤外受累的MF，可采用单一或联合化疗或分子靶向治疗，但其疗效有限。在此，我们报告一例罕见的胃和中枢神经系统(CNS)受累的MF病例。1例67岁男性于2014年9月因全身情况突然恶化转至本院就诊。2006年，根据临床表现和皮肤活检，他被诊断为MF。皮肤活检标本显示非典型淋巴细胞渗入浅表真皮，并偏向表皮(图1a)。他接受了窄谱UVB光疗、静脉注射干扰素-r、局部和整体皮肤电子束照射、口服乙酸乙酯、口服止血剂、口服依托泊苷及其组合。入院时，红斑和溃烂的肿瘤(图1B)分布在大部分身体部位。实验室检查发现白细胞减少(2.4x109/L)，贫血(9.0g/dl)，可溶性IL-2受体水平升高(2257U/ml)。颈部、胸部、腹部和骨盆的计算机断层扫描和正电子发射断层扫描显示没有皮外受累的证据，包括中枢神经系统受累。上消化道内窥镜检查显示多个溃疡隆起的病变并伴有胃粘膜渗出，类似于皮肤病变(图1C)。虽然我们不能进行胃粘膜活检，因为华法林治疗之前的深静脉血栓，我们的临床诊断为MF的胃部受累。他接受吉西他滨治疗(在28天的计划中的第1、8和15天，每次1000 mg/m2的BSA，共6个周期)。一个周期后，皮肤病变显著改善(图1D)，胃部病变在内窥镜下消失(图1E)。最终，他获得了完全的回应。半年后，意识水平有所下降。磁共振成像显示
Dear Editor, Mycosis fungoides (MF) usually has a prolonged clinical course, and visceral involvement is rare. Single or combination chemotherapy, or molecular targeted therapy is used for MF with extracutaneous involvement, but their efficacy is limited. Here, we report a rare case of MF with gastric and central nervous system (CNS) involvement. A 67-year-old man was transferred to our hospital due to sudden deterioration of general condition on September 2014. He was diagnosed as MF based on clinical presentation and skin biopsy in 2006. Skin biopsy specimens showed infiltration of atypical lymphocytes in the superficial dermis with epidermotropism (Fig. 1a). He had been treated with narrowband UVB phototherapy, intravenous injection of interferon-r, local and total skin electron beam irradiation, oral etretinate, oral vorinostat, oral etoposide, and their combinations. On admission, erythematous plaques and ulcerated tumors (Fig. 1b) were distributed over most of the body. The laboratory tests revealed leukocytopenia (2.4 x 109/l), anemia (9.0 g/dl), and elevated soluble IL-2 receptor level (2,257 U/ml). A computed tomography scan of the neck, chest, abdomen, and pelvis and a positron emission tomography scan showed no evidence of extracutaneous involvement, including CNS involvement. Upper gastrointestinal endoscopy showed multiple ulcerated, elevated lesions with oozing of gastric mucosa, which were similar to skin lesions (Fig. 1c). Although we could not perform gastric mucosa biopsy because of warfarin therapy for previous deep venous thrombosis, we clinically diagnosed him as MF with gastric involvement. He received gemcitabine therapy (1000 mg/m2 BSA on days 1, 8, and 15 of a 28-day schedule for a total of six cycles). After one cycle, skin lesions dramatically improved (Fig. 1d) and the gastric lesions disappeared endoscopically (Fig. 1e). Finally, he achieved complete response. After half a year, a decreased level of consciousness developed. A magnetic resonance imaging showed