Lo King-Yik, Chen Chen-Yin, Lee Chih-Shiung
Department of Urology, Tian-Sheng Memorial Hospital, Ping-Tong, Taiwan.
Ren Fail. 2009;31(2):149-52. doi: 10.1080/08860220802595906.
Essential mixed cryoglobulinemia (type II) has turned out to be secondary to hepatitis C virus (HCV) infection in the large majority of patients. Interferon might be anticipated to be effective only in HCV-associated cryoglobulinemias. We found that interferon was highly effective in an HCV-positive patient with true essential type II mixed cryoglobulinemia. The patient presented with symptomatic cryoglobulinemic vasculitis without underlying immunologic, infectious, or neoplastic diseases. Tests for HCV viremia, a reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assay, and anti-HCV antibodies (third-generation assays) were positive before therapy. The patient had severe cryoglobulinemic vasculitis with purpura, peripheral neuropathy, and membranous proliferative glomerulonephritis. The cryocrit before therapy was 6 percent in the patient. Recombinant interferon alfa-2a (Roferon-A, Hoffmann-LaRoche, Basel, Switzerland) was administered at a dose of 3 million units per day for three months and 3 million units every other day for the subsequent nine months, a protocol adopted for HCV-associated cryoglobulinemia. The patient had a complete clinical response, with the disappearance of serum cryoglobulins and all signs of cutaneous vasculitis and with the normalization of kidney-function results and urinary values in the patient with nephropathy. The patient has remained in complete remission for more than one year since the withdrawal of therapy. True essential mixed cryoglobulinemia with HCV infection complicated with glomerulonephritis represents a therapeutic challenge.
在大多数患者中,原发性混合性冷球蛋白血症(II型)已被证明继发于丙型肝炎病毒(HCV)感染。预计干扰素仅对HCV相关的冷球蛋白血症有效。我们发现,干扰素对一名患有真正原发性II型混合性冷球蛋白血症的HCV阳性患者非常有效。该患者表现为有症状的冷球蛋白血症性血管炎,无潜在的免疫、感染或肿瘤性疾病。治疗前,HCV病毒血症检测、逆转录聚合酶链反应(RT-PCR)检测和抗HCV抗体(第三代检测)均呈阳性。该患者患有严重的冷球蛋白血症性血管炎,伴有紫癜、周围神经病变和膜增生性肾小球肾炎。治疗前患者的冷球蛋白比容为6%。采用了针对HCV相关冷球蛋白血症的方案,给予重组干扰素α-2a(罗扰素,瑞士巴塞尔霍夫曼-罗氏公司),剂量为每日300万单位,持续三个月,随后九个月隔日300万单位。患者获得了完全的临床缓解,血清冷球蛋白消失,皮肤血管炎的所有体征消失,肾病患者的肾功能结果和尿液值恢复正常。自停止治疗以来,该患者已完全缓解超过一年。伴有HCV感染并并发肾小球肾炎的真正原发性混合性冷球蛋白血症是一个治疗难题。