CAJ | 학술논문

目的探讨巨细胞心肌炎的临床、病理特征及超微结构改变,评价心脏移植及移植后免疫抑制剂的治疗效果.方法对阜外心血管病医院心脏移植的3例巨细胞心肌炎进行回顾性研究并随访.心脏离体后立即进行肉眼检查、测量和摄像记录,组织化学及免疫组织化学染色,光镜观察组织形态学表现,并对特异性蛋白进行定位标记及半定量检测,透射电镜观察超微结构特点;移植术后心内膜心肌活检评价排斥反应程度.结果 3例均为男性,平均年龄42.3岁(34 ～ 47岁),患者自出现临床症状至心脏移植时间为4～9个月,均有严重的心律失常(3/3),其中2例临床诊断为扩张型心肌病.移植术后均使用免疫抑制剂治疗,1例术后4个月出现急性细胞性排异反应(2R级),治疗后消失.1例术后2周心肌中查见多核巨细胞.巨细胞心肌炎组织学以多核巨细胞肉芽肿为特征.免疫组织化学显示多核巨细胞CD68(+)、CD11b(+)、CD163(-),肉芽肿病灶中的淋巴细胞以CD8+T细胞为主.电镜观察到多核巨细胞为细胞融合形成,并与淋巴细胞及含有分泌颗粒的细胞相黏附.结论巨细胞心肌炎是一种病因不明、易发猝死的罕见病.组织学呈巨细胞肉芽肿性心肌炎表现.免疫组织化学及电镜观察证实多核巨细胞系多个巨噬细胞融合而成,并参与免疫反应.心内膜心肌活检有助本病诊断.心脏移植及免疫抑制剂治疗可以显著延长患者生存时间.
목적 탐토거세포심기염적림상、병리특정급초미결구개변,평개심장이식급이식후면역억제제적치료효과.방법 대부외심혈관병의원심장이식적3례거세포심기염진행회고성연구병수방.심장리체후립즉진행육안검사、측량화섭상기록,조직화학급면역조직화학염색,광경관찰조직형태학표현,병대특이성단백진행정위표기급반정량검측,투사전경관찰초미결구특점;이식술후심내막심기활검평개배척반응정도.결과 3례균위남성,평균년령42.3세(34 ～ 47세),환자자출현림상증상지심장이식시간위4～9개월,균유엄중적심률실상(3/3),기중2례림상진단위확장형심기병.이식술후균사용면역억제제치료,1례술후4개월출현급성세포성배이반응(2R급),치료후소실.1례술후2주심기중사견다핵거세포.거세포심기염조직학이다핵거세포육아종위특정.면역조직화학현시다핵거세포CD68(+)、CD11b(+)、CD163(-),육아종병조중적림파세포이CD8+T세포위주.전경관찰도다핵거세포위세포융합형성,병여림파세포급함유분비과립적세포상점부.결론 거세포심기염시일충병인불명、역발졸사적한견병.조직학정거세포육아종성심기염표현.면역조직화학급전경관찰증실다핵거세포계다개거서세포융합이성,병삼여면역반응.심내막심기활검유조본병진단.심장이식급면역억제제치료가이현저연장환자생존시간.
Objective To identify clinical and pathological features of giant cell myocarditis.Methods Clinical presentation and follow-up data of three patients with giant cell myocarditis were collected.Gross,histopathological,immunohistological and ultrastructural findings of extransplantated hearts of the patients were documented.Results Grossly,multifocal involvement of the myocardium with variably dilated cardiac chambers were observed in all 3 cases.Histological examination revealed pronounced focal inflammatory infiltrates with multinucleated giant cells.Multinucleated giant cells were positive for CD68 and CD11b immunostains but were negative for CD163 in all cases.Transmission electron microscopy showed that the multinucleated giant cells derived from fusion of several macrophages with adherent lymphocytes and secretary cells.Clinically,the overall patient condition improved in all three cases after heart transplantation.One patient experienced acute cellular rejection (2R level) 4 months after transplantation,but recovered after treatment.One patient developed multinucleated giant cells observed in heart biopsy two weeks after transplantation.Conclusions Giant-cell myocarditis is a rare disease of adult,and cardiac transplantation could improve the clinical outcome.Multinucleated giant cell in the myocarditis lesions were derived from macrophages,likely participating in the immune response.Endomyocardial biopsy is important for the diagnosis of giant cell myocarditis.