Nakayama S, Yabe H, Nagai K
Department of Immunohematology, Kobe City General Hospital.
Rinsho Ketsueki. 1990 Dec;31(12):1924-8.
A 31-year-old man was admitted for investigation of proteinuria and hematuria. Physical examination on admission revealed systemic lymphoadenopathy, no hepatosplenomegaly, and ankle edema. Hemoglobin was 14.3 g/dl, platelet 21.4 x 10(4)/microliters and WBC 40,800/microliters which contained 86% mature lymphoid cells. Immunological phenotyping of peripheral lymphoid cells gave positive reactions for CD19, and CD20, and negative reaction for smlg. Urinary protein excretion was 8.3 g/dl in 24h. Serum total protein was 4.1 g/dl with albumin of 2.5 g/dl. Serum IgG was 302 mg/dl, IgA 43 mg/dl, and IgM 56 mg/dl. Renal biopsy showed characteristic features of membranoproliferative glomerulonephritis (MPGN). He was diagnosed as having nephrotic syndrome associated with B-cell chronic lymphocytic leukemia (B-CLL), and was treated with prednisolone and cyclophosphamide without effect. Therefore, he was treated with 18 MU of recombinant-alpha-2a-interferon (IFN-alpha)/day. This treatment resulted in almost normal WBC and differential counts, and urinary protein excretion of 3g in 24h 2 months later. After IFN-alpha treatment was discontinued, WBC count and the amount of urinary protein again increased. He was again treated with IFN-alpha at the dose of 9.0 MU/day three times a week, and is now well without any complaints. This is the first case report in which IFN-alpha was effective in a patient with nephrotic syndrome associated with B-CLL. We think that IFN-alpha therapy is worth trying in similar cases.
一名31岁男性因蛋白尿和血尿入院检查。入院时体格检查发现全身淋巴结肿大,无肝脾肿大及踝关节水肿。血红蛋白为14.3g/dl,血小板21.4×10⁴/微升,白细胞40800/微升,其中86%为成熟淋巴细胞。外周淋巴细胞免疫表型分析显示CD19和CD20呈阳性反应,表面膜免疫球蛋白呈阴性反应。24小时尿蛋白排泄量为8.3g/dl。血清总蛋白为4.1g/dl,白蛋白为2.5g/dl。血清IgG为302mg/dl,IgA为43mg/dl,IgM为56mg/dl。肾活检显示膜增生性肾小球肾炎(MPGN)的特征性表现。他被诊断为与B细胞慢性淋巴细胞白血病(B-CLL)相关的肾病综合征,接受泼尼松龙和环磷酰胺治疗无效。因此,给予他每天18MU的重组α-2a干扰素(IFN-α)治疗。2个月后,这种治疗使白细胞及分类计数几乎恢复正常,24小时尿蛋白排泄量降至3g。停用IFN-α治疗后,白细胞计数和尿蛋白量再次增加。他再次接受每周3次、每次9.0MU的IFN-α治疗,目前情况良好,无任何不适主诉。这是首例关于IFN-α对与B-CLL相关的肾病综合征患者有效的病例报告。我们认为在类似病例中,IFN-α治疗值得一试。