在VAD及随后的高剂量化疗并接受自体干细胞支持后，原发性系统性AL淀粉样变性所致肾病综合征迅速缓解。

Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support.

作者信息

Sezer O, Schmid P, Shweigert M, Heider U, Eucker J, Harder H, Sinha P, Radtke H, Possinger K

机构信息

Universitätsklinikum Charité, Department of Internal Medicine, Haematology/Oncology, Humboldt University, Berlin, Germany.

出版信息

Bone Marrow Transplant. 1999 May;23(9):967-9. doi: 10.1038/sj.bmt.1701765.

DOI:10.1038/sj.bmt.1701765

PMID:10338056

Abstract

In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal lambda light chains were identified in serum and urine. A low percentage of monoclonal plasma cells was detected in the bone marrow. The patient received four cycles of VAD and subsequent high-dose chemotherapy (HDCT) with melphalan (200 mg/m2) followed by autologous peripheral blood stem cell transplantation. Proteinuria rapidly diminished during chemotherapy. Three months after HDCT, the patient has no edema, and no signs of plasma cell dyscrasia are currently detectable. Using VAD before starting HDCT may improve the condition of patients with amyloidosis and reduce transplantation-related morbidity and mortality.

摘要

在一名肾病综合征患者中，肾活检显示有AL淀粉样蛋白沉积。血清和尿液中鉴定出单克隆λ轻链。骨髓中检测到低比例的单克隆浆细胞。该患者接受了四个周期的VAD方案及随后的马法兰（200 mg/m²）大剂量化疗（HDCT），之后进行了自体外周血干细胞移植。化疗期间蛋白尿迅速减少。HDCT后三个月，患者无水肿，目前未检测到浆细胞异常增生的迹象。在开始HDCT之前使用VAD可能改善淀粉样变性患者的病情，并降低移植相关的发病率和死亡率。