Dai Yong, Liu Chaoling, Chen Jiaping, Zeng Qigang, Duan Chenxia
Department of Respiratory Medicine, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine, Foushan, Guangdong Province, China.
Medicine (Baltimore). 2019 Jan;98(3):e14151. doi: 10.1097/MD.0000000000014151.
Clinical and radiologic manifestations of pleural amyloidosis are non-specific. And it can easily be missed or misdiagnosed. Meanwhile, few studies document amyloidosis presenting with pulmonary infarcts at the same time. Hereby, we report a case of immunoglobulin light chain amyloidosis (AL) pleural amyloidosis with pulmonary embolism rarely reported.
A 66-year-old male patient who suffered recurrent pleural effusion for more than 6 months and coughed for 2 months was admitted to hospital for clear diagnosis and treatment. He was previously engaged in a job which exposed him to dust and talcum powder for a long time. He underwent right thoracentesis and anti-infective treatment before admission. The patient's cough and shortness of breath were slightly relieved. He still experienced pleural effusion and had symptoms of cough and shortness of breath.
Chest X-ray demonstrated bilateral pleural effusion. Chest computed tomography (CT) angiography demonstrated left lower pulmonary embolism. The thorascopy showed hyperaemia and black tissue of the parietal pleura, which were biopsied. The pathological diagnosis was amyloidosis. The final diagnosis of this patient was AL pleural amyloidosis and left lower pulmonary embolism.
During the hospitalization, the patient underwent thoracentesis several times without any conclusive diagnosis. After the diagnosis of pleural amyloidosis, the patient was repeatedly advised to undergo bone marrow biopsy and pleurodesis which the patient refused. For pulmonary embolism, Nadroparin calcium combined Warfarin were administered as anticoagulative therapy.
The pulmonary embolism resolved 13 days after the anticoagulant therapy. The patient refused treatment for pleural effusion and requested for discharge. At the time of discharge, shortness of breath was relieved, and the pleural effusion had decreased. The patient was lost to follow-up.
Amyloidosis is a rare disease which can be ignored by many clinicians. It needs to be diagnosed promptly since the prognosis of amyloidosis is poor. Clinicians must improve relevant understandings of this kind of disease so as not to delay the diagnosis and treatment. We must be alert to the occurrence of embolic disease among amyloidosis patients. Last but not least, we should also think of the possibility of amyloidosis in patients with pulmonary embolism and recurrent pleural effusion.
胸膜淀粉样变性的临床和影像学表现不具有特异性。它很容易被漏诊或误诊。同时，很少有研究记录淀粉样变性同时伴有肺梗死的情况。在此，我们报告一例罕见的免疫球蛋白轻链淀粉样变性（AL）胸膜淀粉样变性合并肺栓塞的病例。
一名66岁男性患者，反复胸腔积液6个月余，咳嗽2个月，因明确诊断及治疗入院。他之前长期从事接触粉尘和滑石粉的工作。入院前曾行右胸腔穿刺及抗感染治疗。患者咳嗽及气短稍有缓解。但仍有胸腔积液，并有咳嗽及气短症状。
胸部X线显示双侧胸腔积液。胸部计算机断层扫描（CT）血管造影显示左肺下叶栓塞。胸腔镜检查显示壁层胸膜充血及黑色组织，进行了活检。病理诊断为淀粉样变性。该患者最终诊断为AL胸膜淀粉样变性及左肺下叶栓塞。
住院期间，患者多次接受胸腔穿刺，但未得出明确诊断。在诊断为胸膜淀粉样变性后，多次建议患者进行骨髓活检及胸膜固定术，但患者拒绝。对于肺栓塞，给予那屈肝素钙联合华法林进行抗凝治疗。
抗凝治疗13天后肺栓塞消失。患者拒绝治疗胸腔积液并要求出院。出院时，气短缓解，胸腔积液减少。患者失访。
淀粉样变性是一种罕见疾病，许多临床医生可能会忽视它。由于淀粉样变性预后较差，需要及时诊断。临床医生必须提高对这类疾病的相关认识，以免延误诊断和治疗。我们必须警惕淀粉样变性患者中栓塞性疾病的发生。最后但同样重要的是，我们也应考虑肺栓塞和反复胸腔积液患者发生淀粉样变性的可能性。
