口腔和喉部皮肤T细胞淋巴瘤(CTCL)很少见,通常与预后不良有关。这里,我们讨论了2例严重预处理的患者发生口服CTCL的病例,并提供了文献综述.第一例是一名46岁的非洲裔美国男性,患有快速进展的疾病,在被诊断为CD4+CD8+CTCL6个月后,他的硬腭出现病变。他的皮肤疾病广泛存在,其体表面积的80%以上都有肿瘤。不幸的是,患者在CTCL诊断后2½年和口腔CTCL病变发展后7个月死亡.第二例是一名38岁的非洲裔美国男性,患有IIb期CD3+CD4+CD30+霉菌病(MF),诊断后6个月在硬腭出现肿瘤。他接受了口腔病变的姑息性放疗和肺部的多种全身治疗,喉,食道,和胃受累。胃病变的活检显示CD30T细胞淋巴瘤具有与他的皮肤相同的克隆峰,但具有大细胞转化。Brentuximabvendoin开始了,病人现在已经完全缓解,30个月后。从以英语报告的76例口头CTCL中,六个是转化的MF。最常见的受影响的部位是舌头和腭,最常见的表现是红斑或溃疡性肿瘤,斑块,或与吞咽困难和疼痛相关的结节。口腔CTCL通常在CTCL的初始诊断后数年发生,并且预示预后差,在口腔病变发展后平均存活仅超过1年。
Oral and laryngeal cutaneous T-cell lymphoma (CTCL) is rare and usually associated with poor prognosis. Here, we discuss 2 cases of oral CTCL that developed in heavily pretreated patients and provide a
review of the literature. The first case is of a 46-year-old African American male with rapidly progressive disease, presenting with a lesion on his hard palate 6 months after being diagnosed with a CD4+CD8+ CTCL. His cutaneous disease was widespread with tumors on >80% of his body surface area. Unfortunately, the patient died 2 ½ years after his CTCL diagnosis and 7 months after developing the oral CTCL lesion. The second case is of a 38-year-old African American male with stage IIb CD3+CD4+CD30+ mycosis fungoides (MF), who developed a tumor on the hard palate 6 months after diagnosis. He received palliative radiation to the oral lesion and multiple lines of systemic therapy for pulmonary, laryngeal, esophageal, and gastric involvement. Biopsy of the gastric lesions showed a CD30+ T-cell lymphoma with the same clonal peak as in his skin but with large cell transformation. Brentuximab vendoin was started, and the patient is now in complete remission, 30 months later. From the 76 cases of oral CTCL that have been reported in the English language, six were of transformed MF. The most common sites affected were the tongue and palate, and the most common presentation were erythematous or ulcerated tumors, plaques, or nodules associated with dysphagia and pain. Oral CTCL typically occurs years after the initial diagnosis of CTCL and portend a poor prognosis with an average survival of just over 1 year after development of oral lesions.