Rimon D, Lurie M, Storch S, Halon D, Eisenkraft S, Laor A, Cohen L
Department of Medicine B, Carmel Hospital, Haifa, Israel.
Arch Intern Med. 1988 Mar;148(3):551-3.
Multiple myeloma and congestive heart failure developed in a patient with long-standing scleredema adultorum. Staining of the myocardium, performed after her death, was positive for acid mucopolysaccharide and negative for amyloid. To the best of our knowledge, this is the first case in which acid mucopolysaccharide has been demonstrated in the myocardium, thus explaining the cardiomyopathy of scleredema adultorum. Review of the world literature enabled us to identify a statistically significant increased prevalence of plasma cell dyscrasia among patients with protracted scleredema. In all patients, plasma cell dyscrasia appeared years after the onset of scleredema. Immunofluorescent studies were negative for immunoglobulin deposition. We assume, therefore, that the plasma cell dyscrasia was secondary to scleredema.
一名患有成人硬肿病多年的患者出现了多发性骨髓瘤和充血性心力衰竭。在她死后对心肌进行染色，结果显示酸性粘多糖呈阳性，淀粉样蛋白呈阴性。据我们所知，这是首例在心肌中证实存在酸性粘多糖的病例，从而解释了成人硬肿病的心肌病。对世界文献的回顾使我们能够确定，长期硬肿病患者中浆细胞异常增生的患病率在统计学上显著增加。在所有患者中，浆细胞异常增生均出现在硬肿病发病数年之后。免疫荧光研究显示免疫球蛋白沉积为阴性。因此，我们认为浆细胞异常增生是硬肿病的继发表现。
