CAJ | 학술논문

目的探讨多中心网状组织细胞增生症(MRH)的临床特征.方法分析10例MRH患者的临床表现、影像及病理特征,并复习相关文献.结果 ①10例患者中7例为女性,年龄33～84岁.②以全身皮肤多发性丘疹结节(10例)和侵蚀性关节炎(10例)为突出表现,部分累及其他系统,表现为肌炎(4例)、肺部结节(3例)、喉腔结节(1例)和浆膜炎(1例).③组织活检病理表现为大量CD68阳性细胞质呈颗粒毛玻璃样外观的多核巨细胞和组织细胞浸润.④10例患者均应用糖皮质激素联合免疫抑制剂治疗,7例丘疹结节减轻,3例关节肿痛减轻.结论 MRH是一种系统性疾病,有特征性临床症状,独特的组织病理,提高认识可减少对该病的误诊,确实有效的治疗措施尚待进一步研究.
목적 탐토다중심망상조직세포증생증(MRH)적림상특정.방법 분석10례MRH환자적림상표현、영상급병리특정,병복습상관문헌.결과 ①10례환자중7례위녀성,년령33～84세.②이전신피부다발성구진결절(10례)화침식성관절염(10례)위돌출표현,부분루급기타계통,표현위기염(4례)、폐부결절(3례)、후강결절(1례)화장막염(1례).③조직활검병리표현위대량CD68양성세포질정과립모파리양외관적다핵거세포화조직세포침윤.④10례환자균응용당피질격소연합면역억제제치료,7례구진결절감경,3례관절종통감경.결론 MRH시일충계통성질병,유특정성림상증상,독특적조직병리,제고인식가감소대해병적오진,학실유효적치료조시상대진일보연구.
Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH).Methods The clinical manifestations,image and histologic characteristics of ten patients with MRH were collected,and the relevant literatures were reviewed.Results Seven cases were women.The median (quartile) age was 46.5 (44.5-55.3) years.Clinical symptoms included papulonodular lesions on the skin (ten cases),erosive polyarthritis (ten cases),myositis (four cases),lung nodules (three cases),larynx nodules (one case) and serositis (one case).Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and "glassy" cytoplasm.All of the ten patients were treated with corticosteroids and disease-modifying antirheumatic drugs,skin lesions of seven patients were ameliorated and arthritis was improved in three patients.Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings.Deep understanding of this disease can avcid.isdiagnose.Further studies are needed to explore effective treatment.