15例原发性淀粉样变性的临床与病理分析

目的 加强对有突出肾损害的原发性淀粉样变性的认识，提高诊断水平。方法 对１５例有突出肾损害的原发性淀粉样变性病例资料从多脏器受累情况。骨髓穿刺及活检。血／尿蛋白电泳和电泳及治疗预后等方面进行总结和分析。结果 肾脏、消化、循环和血液系统有不同程度受累，部分病例有骨髓浆细胞增生，血／尿中检出单克隆蛋白。结论本病肾损害有一定特点，尤其特异性的肾脏病理改变可明确诊断 。

Primary Systemic Amyloidosis Clinical and Pathological Analysis in Fifteen Patients

Objective To improve our understanding of the clinical and pathological features, especially renal damage, of primary systemic amyloidosis (AL) for early diagnosis and better treatment. Methods Fifteen records of AL were reviewed. Results in this group of patients, many aspects were analyzed, including multi - organ involvement, findings of bone marrow examination, serum and urine electrophoresis and immunoelectrophoresis, as well as treatment and prognosis. Conclusion AL is closely related to other plasma cell dyscrasias. It usually has multi - organ involvement, in which the renal damage is common and its pathologic findings are essential to diagnosis.