Godskesen Line, Abildgaard Niels, Kjeldsen Jens, Krag Aleksander
Odense University Hospital, Odense C, Denmark.
BMJ Case Rep. 2014 Apr 3;2014:bcr2013203360. doi: 10.1136/bcr-2013-203360.
A previously healthy 43-year-old man presented with dyspnoea, 15 kg weight loss, severe hepatomegaly and alkaline phosphatase at 5400 U/L. Examinations seemed to suggest cirrhosis, but blood samples did not show any signs of underlying liver disease. Liver biopsy revealed amyloid light chain (AL) amyloidosis and bone marrow showed multiple myeloma (MM). The patient was treated with drugs of choice cyclophosphamide, bortezomib and dexamethasone. He responded well to the treatment and so far achieved partial response. Previously MM was associated with poor prognosis but due to improved treatment for AL the patient can achieve a progression-free period with good quality of life.
一名既往健康的43岁男性出现呼吸困难、体重减轻15千克、严重肝肿大，碱性磷酸酶为5400 U/L。检查似乎提示肝硬化，但血样未显示任何潜在肝病迹象。肝活检显示为轻链（AL）淀粉样变性，骨髓检查显示为多发性骨髓瘤（MM）。该患者接受了首选药物环磷酰胺、硼替佐米和地塞米松治疗。他对治疗反应良好，目前已达到部分缓解。既往MM预后较差，但由于对AL的治疗有所改善，该患者可实现无进展生存期，生活质量良好。
