Jang Suk-Tae, Sohn Il-Suk, Jin Eun-Sun, Cho Jin-Man, Kim Chong-Jin, Lim Sung-Jig
Department of Cardiology, East-West Neo Medical Center of Kyunghee University, Seoul, Korea.
J Korean Med Sci. 2009 Apr;24(2):354-6. doi: 10.3346/jkms.2009.24.2.354. Epub 2009 Apr 20.
The monoclonal gammopathies (MG) are monoclonal neoplasms related to each other by virtue of their development from common progenitors in the B lymphocyte lineage. Cardiac dysfunction in patients with MG is not well established. We experienced a case of cardiac dysfunction associated with MG identified by echocardiography and biopsy. Fifty nine year-old man was admitted because of dyspnea for several months. Echocardiography revealed diastolic dysfunction showing restrictive physiology with elevated left ventricular filling pressure. Bone marrow (BM) studies and immunoelectrophoresis were compatible with monoclonal gammopathy of undetermined significance. Endomyocardial, BM, and enteral biopsies for ruling out for amyloidosis (Congo-red stain) were negative. This is the case of non-amyloidotic light chain deposition cardiomyopathy.
单克隆丙种球蛋白病(MG)是一类单克隆肿瘤,由于它们起源于B淋巴细胞谱系中的共同祖细胞而相互关联。MG患者的心脏功能障碍尚未完全明确。我们遇到了一例通过超声心动图和活检确诊的与MG相关的心脏功能障碍病例。一名59岁男性因数月来的呼吸困难入院。超声心动图显示舒张功能障碍,呈现限制性生理特征,左心室充盈压升高。骨髓(BM)检查和免疫电泳结果与意义未明的单克隆丙种球蛋白病相符。为排除淀粉样变性(刚果红染色)而进行的心肌内膜、BM和肠活检均为阴性。这是一例非淀粉样轻链沉积性心肌病病例。