Tervaert J W Cohen, Van Paassen P, Damoiseaux J
Department of Clinical and Experimental Immunology, University Hospital Maastricht, P. Debyelaan 25, 6229 HX Maastricht, the Netherlands.
Ann N Y Acad Sci. 2007 Jun;1107:251-8. doi: 10.1196/annals.1381.027.
Mixed cryoglobulinemia (MC) are cryoprecipitable immunocomplexes. In type II MC, a combination of polyclonal and monoclonal immunoglobulins is found, whereas in type III a combination of polyclonal immunoglobulins is detected. MC is usually associated with hepatitis C (HCV) infection as has been found in studies that have been performed in countries with a high prevalence of HCV. Because HCV has an extremely low prevalence in the Netherlands (<0.1% of the population), we wondered whether HCV is also associated with MC in our regional referral center. To answer this question, we tested consecutive patients with type II MC for HCV antibodies and for HCV-mRNA by polymerase chain reaction (PCR). Between January 2000 and June 2005, 22 patients tested positive for type II MC. Seven patients had essential MC, 2 patients had MC due to a lymphoproliferative disease, 10 patients had MC in the context of a systemic autoimmune disease, and 3 patients had MC without a clear diagnosis. HCV antibodies were not detected in any of the 22 patients. Also, all samples tested negative for HCV-mRNA. During follow-up none of these patients developed an HCV infection. In summary, the estimated occurrence of HCV in 60-90% of patients with MC is not found in our region where MC is only infrequently associated with HCV. In a substantial proportion of our patients a really "essential MC" is observed. A search for yet unknown etiological factors is clearly needed in these patients, who frequently have severe renal involvement warranting aggressive immunosuppressive therapy.
混合性冷球蛋白血症(MC)是可冷沉淀的免疫复合物。在II型MC中,可发现多克隆和单克隆免疫球蛋白的组合,而在III型中可检测到多克隆免疫球蛋白的组合。MC通常与丙型肝炎(HCV)感染相关,这在HCV高流行国家所开展的研究中已得到证实。由于HCV在荷兰的流行率极低(<0.1%的人口),我们想知道在我们的区域转诊中心HCV是否也与MC相关。为回答这个问题,我们通过聚合酶链反应(PCR)对连续的II型MC患者检测了HCV抗体和HCV-mRNA。在2000年1月至2005年6月期间,22例患者II型MC检测呈阳性。7例患者患有原发性MC,2例患者因淋巴增殖性疾病患有MC,10例患者在系统性自身免疫性疾病背景下患有MC,3例患者患有MC但诊断不明确。22例患者中均未检测到HCV抗体。此外,所有样本的HCV-mRNA检测均为阴性。在随访期间,这些患者均未发生HCV感染。总之,在我们这个MC仅偶尔与HCV相关的地区,未发现60 - 90%的MC患者中估计存在的HCV感染情况。在我们相当一部分患者中观察到了真正的“原发性MC”。显然需要对这些经常有严重肾脏受累且需要积极免疫抑制治疗的患者寻找尚未明确的病因。