Koopman Pieter, Van Dorpe Jo, Maes Bart, Dujardin Karl
Department of Cardiology, Heilig Hart Ziekenhuis, Roeselare, Belgium.
Acta Cardiol. 2009 Dec;64(6):821-4. doi: 10.2143/AC.64.6.2044752.
We report an unusual case of a 47-year-old Caucasian woman who presented with severe dyspnoea as a manifestation of restrictive cardiomyopathy, found to be due to myocardial deposition of kappa light chains. Non-routine specific immunofluorescence stainings of endomyocardial biopsy specimens were key for the diagnosis of myocardial light chain deposition disease. We discuss non-amyloidotic cardiac immunoglobulin deposition disease in contrast to cardiac amyloidosis.
我们报告了一例不寻常的病例,一名47岁的白种女性,表现为严重呼吸困难,这是限制性心肌病的一种表现,发现是由于κ轻链在心肌沉积所致。心内膜活检标本的非常规特异性免疫荧光染色是诊断心肌轻链沉积病的关键。我们讨论了与心脏淀粉样变性相反的非淀粉样变性心脏免疫球蛋白沉积病。