Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support
Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support
复制标题
VAD 和随后的自体干细胞支持大剂量化疗后，可快速逆转原发性全身性 AL 淀粉样变性导致的肾病综合征
DOI：
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发表时间：
1999
影响因子：
4.8
通讯作者：
K. Possinger
中科院分区：
文献类型：
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作者：
O. Sezer;P. Schmid;M. Schweigert;U. Heider;J. Eucker;H. Harder;P. Sinha;H. Radtke;K. Possinger
1例肾病综合征患者，肾活检发现AL淀粉样物沉积。血清和尿液中均可检测到单抗λ轻链。骨髓中可见低比例的单克隆性浆细胞。患者接受了四个周期的VAD和随后的大剂量化疗(HDCT)和马法兰(200 mg/m2)，然后是自体外周血干细胞移植。化疗期间蛋白尿迅速减少。HDCT三个月后，患者没有水肿，目前也没有浆细胞异染症的迹象。在开始HDCT前使用VAD可能会改善淀粉样变性患者的病情，降低移植相关的发病率和死亡率。
In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal λ 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.
影响因子：
3.6
作者：
R. Kyle;M. Gertz
通讯作者：
R. Kyle;M. Gertz