Kim Dong-Hyeok, Kim Yong-Hyun, Song Woo-Hyuk, Ahn Jeong-Cheon
Division of Cardiology, Department of Internal Medicine, Korea University Ansan Hospital, Ansan, Korea.
Echocardiography. 2014 Apr;31(4):E115-9. doi: 10.1111/echo.12477. Epub 2013 Dec 17.
Primary cardiac lymphoma (PCL) is a very rare malignancy although cardiac involvement with the disseminated disease is not uncommon. We present a case of a 43-year-old man with PCL that initially presented as marked thickening of all cardiac walls and was mistakenly diagnosed as an atypical type of hypertrophic cardiomyopathy. The diagnosis of PCL was made with a delay of 9 months after the initial presentation, when atypical lymphocytes staining positive for CD79a and CD20 were demonstrated in the rapidly growing mediastinal and neck mass. Anthracycline-based chemotherapy and anti-CD20 immunotherapy resulted in a remarkable reduction in cardiac wall thickness and mediastinal mass. The first lesson to be learnt from this case is that PCL can present as a diffuse infiltrative disease without a mass. The second lesson is that prompt exploratory thoracotomy should not be delayed when the diagnosis is elusive.
原发性心脏淋巴瘤(PCL)是一种非常罕见的恶性肿瘤,尽管心脏受播散性疾病累及并不少见。我们报告一例43岁男性原发性心脏淋巴瘤病例,该患者最初表现为所有心壁明显增厚,被误诊为非典型肥厚型心肌病。原发性心脏淋巴瘤的诊断在初次就诊9个月后才得以明确,当时在快速生长的纵隔和颈部肿块中发现了CD79a和CD20染色阳性的非典型淋巴细胞。基于蒽环类药物的化疗和抗CD20免疫治疗使心壁厚度和纵隔肿块显著减小。从此病例中吸取的第一个教训是,原发性心脏淋巴瘤可表现为无肿块的弥漫浸润性疾病。第二个教训是,当诊断难以明确时,不应延迟及时进行 exploratory thoracotomy(此处可能有误,推测应为“exploratory thoracotomy”,即“剖胸探查术”)。