IgG4相关性疾病(immunoglobulin G4 related disease,IgG4-RD)是一类病因、发病机制尚不明确的进行性自身免疫性疾病,其临床特点为受累器官弥漫性或局灶性肿大、硬化,血清IgG4水平升高,组织病理学以受累组织大量淋巴细胞和浆细胞浸润、...IgG4相关性疾病(immunoglobulin G4 related disease,IgG4-RD)是一类病因、发病机制尚不明确的进行性自身免疫性疾病,其临床特点为受累器官弥漫性或局灶性肿大、硬化,血清IgG4水平升高,组织病理学以受累组织大量淋巴细胞和浆细胞浸润、席纹状纤维化和闭塞性静脉炎为主要特征。本文报道一例以反复胸水首诊并累及全身多器官的IgG4-RD,为临床医生认识并重视本病诊治提供借鉴。展开更多
韦格纳肉芽肿(Wegener’s granulomation,WG)又名肉芽肿性血管炎(Granulomatosis with Polyangiitis,GPA)是一种累及小动脉、静脉及毛细血管,偶尔累及大动脉,病理以血管壁炎症为特征的系统性自身免疫性疾病。临床上既可表现为单个脏器受...韦格纳肉芽肿(Wegener’s granulomation,WG)又名肉芽肿性血管炎(Granulomatosis with Polyangiitis,GPA)是一种累及小动脉、静脉及毛细血管,偶尔累及大动脉,病理以血管壁炎症为特征的系统性自身免疫性疾病。临床上既可表现为单个脏器受累,也可表现为多脏器受累,其中肺、肾受累者多见,病情进展迅速,严重者危及生命[1],给患者的身体、生活、心理及经济负担带来了严重的危害,护理难度增大。因此,全方位评估患者多器官受累情况,给予针对性护理尤显重要。2019年3月我院对1例韦格纳肉芽肿并发多器官受累患者进行精准治疗和护理,取得不错成效。现报告如下。展开更多
A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the...A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial work-up. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, th e CD8+epidermotropic cytotoxic subset comprises rare, highly aggressive forms c haracterized by metastatic spread to unusual sites such as the oral cavity, lung s, testis and the central nervous system but usually not to the lymph nodes. The se cases seem to be distinct from mycosis fungoides with CD8+phenotype, which s hows a nonaggressive clinical behavior.展开更多
文摘IgG4相关性疾病(immunoglobulin G4 related disease,IgG4-RD)是一类病因、发病机制尚不明确的进行性自身免疫性疾病,其临床特点为受累器官弥漫性或局灶性肿大、硬化,血清IgG4水平升高,组织病理学以受累组织大量淋巴细胞和浆细胞浸润、席纹状纤维化和闭塞性静脉炎为主要特征。本文报道一例以反复胸水首诊并累及全身多器官的IgG4-RD,为临床医生认识并重视本病诊治提供借鉴。
文摘韦格纳肉芽肿(Wegener’s granulomation,WG)又名肉芽肿性血管炎(Granulomatosis with Polyangiitis,GPA)是一种累及小动脉、静脉及毛细血管,偶尔累及大动脉,病理以血管壁炎症为特征的系统性自身免疫性疾病。临床上既可表现为单个脏器受累,也可表现为多脏器受累,其中肺、肾受累者多见,病情进展迅速,严重者危及生命[1],给患者的身体、生活、心理及经济负担带来了严重的危害,护理难度增大。因此,全方位评估患者多器官受累情况,给予针对性护理尤显重要。2019年3月我院对1例韦格纳肉芽肿并发多器官受累患者进行精准治疗和护理,取得不错成效。现报告如下。
文摘A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient’s history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial work-up. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, th e CD8+epidermotropic cytotoxic subset comprises rare, highly aggressive forms c haracterized by metastatic spread to unusual sites such as the oral cavity, lung s, testis and the central nervous system but usually not to the lymph nodes. The se cases seem to be distinct from mycosis fungoides with CD8+phenotype, which s hows a nonaggressive clinical behavior.
