Sun Yang, Zhao Hong, Song Laifeng, Wang Qingzhi, Chu Yan, Huang Jie, Hu Shengshou
State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
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Zhonghua Bing Li Xue Za Zhi. 2015 Feb;44(2):123-7.
To identify clinical and pathological features of giant cell myocarditis.
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.
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.
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.
明确巨细胞性心肌炎的临床和病理特征。
收集3例巨细胞性心肌炎患者的临床表现及随访数据。记录患者心脏移植术后心脏的大体、组织病理学、免疫组织化学及超微结构检查结果。
大体检查显示，所有3例均可见心肌多灶性受累，心腔不同程度扩张。组织学检查显示有明显的局灶性炎性浸润及多核巨细胞。所有病例中，多核巨细胞CD68和CD11b免疫染色呈阳性，但CD163呈阴性。透射电子显微镜显示，多核巨细胞由多个巨噬细胞与黏附淋巴细胞及分泌细胞融合而成。临床上，所有3例患者心脏移植术后整体病情均有改善。1例患者术后4个月发生急性细胞排斥反应（2R级），经治疗后恢复。1例患者移植术后2周心脏活检发现多核巨细胞。
巨细胞性心肌炎是一种成人罕见疾病，心脏移植可改善临床结局。心肌炎病变中的多核巨细胞来源于巨噬细胞，可能参与免疫反应。心内膜心肌活检对巨细胞性心肌炎的诊断很重要。
