PDF(Pubmed)
Abstract：
UNASSIGNED: Unicentric Castleman\'s disease (UCD), a lymphoproliferative disorder characterized by enlargement of the lymph nodes, is a rare cause of Amyloid-A amyloidosis. While patients usually present with impaired kidney function and proteinuria, heart involvement is neither common nor the main cause of signs and symptoms.
UNASSIGNED: We present a patient who was admitted to the hospital for impaired exercise capacity. Diagnostic work-up revealed severe left ventricular hypertrophy suggestive of cardiac amyloidosis. Although Congo red staining of endomyocardial biopsies was initially negative, subsequent immunohistochemical staining against serum amyloid A finally confirmed the diagnosis of cardiac amyloidosis. 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed a tumour located in dorsal of the duodenum. Fine-needle aspiration biopsy of the tumour was suggestive but could not confirm the presence of UCD beyond reasonable doubt. Rapid worsening of heart failure symptoms warranted urgent surgical tumourectomy, which resulted in immediate post-operative lowering of serum amyloid protein. However, post-operative cardiogenic shock could not be stabilized even with veno-arterial extracorporeal membrane oxygenation, and the patient eventually died. The UCD of the hyaline vascular (HV) subtype was confirmed by pathologic work-up of the excised tumour.
UNASSIGNED: This case report presents for the first time a patient with malignant cardiac Amyloid-A amyloidosis caused by unicentric Castleman\'s disease of the HV subtype. Since the disease progresses swiftly, rapid diagnosis is essential for potential curative treatment.
UNASSIGNED: We present a patient who was admitted to the hospital for impaired exercise capacity. Diagnostic work-up revealed severe left ventricular hypertrophy suggestive of cardiac amyloidosis. Although Congo red staining of endomyocardial biopsies was initially negative, subsequent immunohistochemical staining against serum amyloid A finally confirmed the diagnosis of cardiac amyloidosis. 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed a tumour located in dorsal of the duodenum. Fine-needle aspiration biopsy of the tumour was suggestive but could not confirm the presence of UCD beyond reasonable doubt. Rapid worsening of heart failure symptoms warranted urgent surgical tumourectomy, which resulted in immediate post-operative lowering of serum amyloid protein. However, post-operative cardiogenic shock could not be stabilized even with veno-arterial extracorporeal membrane oxygenation, and the patient eventually died. The UCD of the hyaline vascular (HV) subtype was confirmed by pathologic work-up of the excised tumour.
UNASSIGNED: This case report presents for the first time a patient with malignant cardiac Amyloid-A amyloidosis caused by unicentric Castleman\'s disease of the HV subtype. Since the disease progresses swiftly, rapid diagnosis is essential for potential curative treatment.
摘要：
■UnicentricCastleman病（UCD），一种以淋巴结肿大为特征的淋巴增生性疾病，是淀粉样蛋白A淀粉样变性的罕见原因。虽然患者通常表现为肾功能受损和蛋白尿，心脏受累既不常见，也不是体征和症状的主要原因。
■我们介绍了一名因运动能力受损而入院的患者。诊断检查显示严重的左心室肥大，提示心脏淀粉样变性。尽管心内膜活检的刚果红染色最初是阴性的，随后针对血清淀粉样蛋白A的免疫组织化学染色最终证实了心脏淀粉样变性的诊断。18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描显示肿瘤位于十二指肠背侧。肿瘤的细针抽吸活检具有暗示性，但无法确定UCD的存在。心力衰竭症状的快速恶化需要紧急手术肿瘤切除术，导致术后立即降低血清淀粉样蛋白。然而,即使使用静脉-动脉体外膜氧合也无法稳定术后心源性休克，病人最终死了.通过切除肿瘤的病理检查证实了透明血管（HV）亚型的UCD。
■该病例报告首次提出了由HV亚型的单中心Castleman病引起的恶性心脏淀粉样蛋白A淀粉样变性患者。由于疾病进展迅速，快速诊断对于潜在的治愈性治疗至关重要。
■我们介绍了一名因运动能力受损而入院的患者。诊断检查显示严重的左心室肥大，提示心脏淀粉样变性。尽管心内膜活检的刚果红染色最初是阴性的，随后针对血清淀粉样蛋白A的免疫组织化学染色最终证实了心脏淀粉样变性的诊断。18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描显示肿瘤位于十二指肠背侧。肿瘤的细针抽吸活检具有暗示性，但无法确定UCD的存在。心力衰竭症状的快速恶化需要紧急手术肿瘤切除术，导致术后立即降低血清淀粉样蛋白。然而,即使使用静脉-动脉体外膜氧合也无法稳定术后心源性休克，病人最终死了.通过切除肿瘤的病理检查证实了透明血管（HV）亚型的UCD。
■该病例报告首次提出了由HV亚型的单中心Castleman病引起的恶性心脏淀粉样蛋白A淀粉样变性患者。由于疾病进展迅速，快速诊断对于潜在的治愈性治疗至关重要。
