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首页> 外文期刊>Journal of International Medical Research >Hepatic veno-occlusive disease may be a rare characteristic of hepatic involvement in systemic amyloidosis: case report and literature review
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Hepatic veno-occlusive disease may be a rare characteristic of hepatic involvement in systemic amyloidosis: case report and literature review

机译：肝静脉闭塞病可能是肝脏受累的罕见特征，肝脏淀粉样蛋白症：病例报告和文献综述

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摘要

Systemic amyloidosis is a rare disease and patients with hepatic amyloidosis often present with hepatomegaly. Hepatomegaly can also be a feature of hepatic veno-occlusive disease (HVOD). We report here a case of systemic amyloidosis in a patient who was suspected of having HVOD. On the basis of computed tomography findings in the abdomen, HVOD was initially suspected in a 63-year-old man with the chief complaint of upper abdominal pain, ascites, and weight loss. Multiple patchy purpura and nerve symptoms were identified and these were due to amyloidosis. An increase in proteinuria and immunoglobulin κ light-chain levels, and thickening of the ventricular wall supported the diagnosis of systemic light-chain amyloidosis involving the liver, heart, kidney, skin, and nerves. This diagnosis was confirmed by histological examination of a bone marrow core biopsy with Congo red dye. Sequential treatment of bortezomib and dexamethasone led to good results in the patient. Findings of this rare case indicate that HVOD can be diagnosed without a definite history of hematopoietic stem cell transplantation or pyrrolizidine alkaloid ingestion, but more evidence is required to make an accurate diagnosis. Importantly, we speculate that HVOD is a rare characteristic of liver involvement in systemic amyloidosis.
机译：全身淀粉样症是一种罕见的疾病和肝脏淀粉样蛋白病的患者通常存在于肝肿大。肝肿瘤也可以是肝静脉闭塞疾病（HVOD）的特征。我们在此报告了被怀疑患有HVOD的患者的全身淀粉样蛋白病的案例。在腹部计算的断层摄影结果的基础上，HVOD最初怀疑在一个63岁的男子中，具有上腹部疼痛，腹水和减肥的主要抱怨。鉴定了多种斑斑的紫癜和神经症状，这些症状是由于淀粉样蛋白病。蛋白尿和免疫球蛋白κ轻链水平的增加，心室壁的增厚支持涉及肝脏，心脏，肾，皮肤和神经的全身轻链淀粉样蛋白病的诊断。通过与刚果红染料的骨髓核心活组织检查的组织学检查证实了该诊断。硼替佐米和地塞米松的顺序治疗导致患者的良好结果。这种罕见案例的结果表明，在没有明确的造血干细胞移植或吡咯嗪生物碱摄取的明确历史中，可以诊断HVOD，但需要更多的证据来进行准确的诊断。重要的是，我们推测HVOD是肝脏受累的罕见特征，对全身淀粉样蛋白病。

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