胸膜淀粉样病变1例并文献复习

A case of amyloidosis of pleural and literature review

目的提高胸膜淀粉样病变的诊断率。方法报道1例原发性淀粉样病变，主要累及胸膜、心脏，并复习相关文献，对其病因、发病机制、临床表现、诊断、治疗及预后进行分析。结果本例原发性系统性淀粉样病变患者无家族史及慢性疾病史，胸膜病理诊断原发性淀粉样病变AL型，其他组织如皮下脂肪、骨髓未见淀粉样物质沉淀。结论对于反复出现不明原因的顽固性胸腔积液的鉴别诊断，应考虑本病的可能；为治疗大量顽固性胸腔积液，可行胸膜腔黏连术缓解症状。

Objective To improve the diagnostic rate of amyloidosis of pleura. Methods One case of amyloidosis of pleura was reported and the literatures were reviewed on the etiopathology, clinical features, diagnosis, treatment and prognosis. Results The patient had no family history and other chronic diseases. Pleura was taken by pleural biopsy and was eventually found to have localized amyloidosis. The amyloidosis was found to be of AL amyloidosis. Multiple biopsy specimens taken from subcutaneous fat showed no amyloid deposition. Conclusions Amyloidosis should be considered in those patients with recurring intractable pleural effusion with unknown reasons, they should be treated through pleural adhesions to relieve symptoms.