Ren H, Poston R S, Hruban R H, Baumgartner W A, Baughman K L, Hutchins G M
Department of General Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Mod Pathol. 1993 Jul;6(4):402-7.
Several aspects of giant cell myocarditis remain controversial, including the natural history of the disease and the nature of the giant cells. We have observed three patients who had long survival with chronic active giant cell myocarditis. The first patient was a 59-yr-old female who had a 10-yr history of complete heart block which was found at autopsy to have been caused by giant cell myocarditis. The second patient is a 36-yr-old female who received a heart transplant 5 yr after a biopsy proven episode of active myocarditis, and examination of the explanted heart revealed giant cell myocarditis. The third patient was a 41-yr-old male who received a heart transplant 2 yr after developing progressive heart failure, and the explanted heart had giant cell myocarditis. On immunohistochemical study of the three hearts, the giant cells stained with the macrophage markers lysozyme and KP-1 (CD-68). Staining of the same cells with desmin and actin was focally positive in a punctate pattern, correlating with the ultrastructural presence of myofibrils within giant cell phagolysosomes. The associated lymphocytic infiltrate stained primarily for the T-cell markers CD-3, CD-45RO, and CD-43 whereas only a few of the lymphocytes stained with the B-cell marker CD-20. The long histories of cardiac dysfunction in the three patients show that giant cell myocarditis may have a protracted course. The morphologic studies show that the giant cells are of histiocytic origin but can contain phagocytosed components of myocytes, observations that may account for the controversy surrounding the nature of the giant cells in giant cell myocarditis.
巨细胞性心肌炎的几个方面仍存在争议，包括该疾病的自然史以及巨细胞的性质。我们观察了三名患有慢性活动性巨细胞性心肌炎且长期存活的患者。第一名患者是一名59岁女性，有10年完全性心脏传导阻滞病史，尸检发现其由巨细胞性心肌炎引起。第二名患者是一名36岁女性，在活检证实为活动性心肌炎发作5年后接受了心脏移植，对切除的心脏检查发现有巨细胞性心肌炎。第三名患者是一名41岁男性，在出现进行性心力衰竭2年后接受了心脏移植，切除的心脏有巨细胞性心肌炎。对这三颗心脏进行免疫组织化学研究时，巨细胞用巨噬细胞标志物溶菌酶和KP-1（CD-68）染色。同一细胞用结蛋白和肌动蛋白染色呈局灶性点状阳性，这与巨细胞吞噬溶酶体内肌原纤维的超微结构存在相关。相关的淋巴细胞浸润主要用T细胞标志物CD-3、CD-45RO和CD-43染色，而只有少数淋巴细胞用B细胞标志物CD-20染色。这三名患者心脏功能障碍的长期病史表明，巨细胞性心肌炎可能病程迁延。形态学研究表明，巨细胞起源于组织细胞，但可含有吞噬的心肌细胞成分，这些观察结果可能解释了围绕巨细胞性心肌炎中巨细胞性质的争议。
