Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto, Japan.
Department of Internal Medicine, Sakura-jyuji Yatsushiro Rehabilitation Hospital, Yatsushiro, Japan
BMJ Case Rep. 2022 Aug 18;15(8):e250338. doi: 10.1136/bcr-2022-250338.
We report a case of cardiac amyloid A (AA) amyloidosis due to unicentric Castleman disease (UCD) in a patient whose cardiac function was restored 15 years after surgical resection of the mesenteric lymph node lesion. A man in his 40s had recurrent palpitations and fainting spells. ECG revealed Increased C-reactive protein, interleukin-6 and serum AA levels, and marked concentric thickening of the left ventricular (LV) wall with diastolic restrictive filling pattern were observed. Duodenal biopsy revealed AA amyloid deposits. He had a mesenteric tumour, comprising many plasma cells. He was diagnosed with plasma cell-type UCD associated with secondary AA amyloidosis. C-reactive protein, interleukin-6 and serum AA levels were normalised 2 months postresection. Episodes of lethal ventricular arrhythmias decreased. LV wall thickness was gradually reduced. Approximately 15 years postresection, the LV wall thickness nearly normalised and ventricular arrhythmias disappeared. Better outcomes are expected following surgical tumour resection.
我们报告了一例由单中心Castleman 病（UCD）引起的心脏淀粉样 A（AA）淀粉样变性病例，该患者在肠系膜淋巴结病变切除 15 年后心脏功能得到恢复。一名 40 多岁的男性反复出现心悸和晕厥。心电图显示 C 反应蛋白、白细胞介素-6 和血清 AA 水平升高，左心室（LV）壁呈同心性增厚，伴舒张限制性充盈模式。十二指肠活检显示 AA 淀粉样沉积物。他有一个肠系膜肿瘤，包含许多浆细胞。他被诊断为与继发性 AA 淀粉样变性相关的浆细胞型 UCD。切除后 2 个月，C 反应蛋白、白细胞介素-6 和血清 AA 水平恢复正常。致命性室性心律失常发作减少。LV 壁厚度逐渐减少。切除后约 15 年，LV 壁厚度几乎恢复正常，室性心律失常消失。手术切除肿瘤后预计会有更好的结果。
