Bulfoni A
Minerva Med. 1995 May;86(5):193-8.
Mixed cryoglobulinemia is a cryoprecipitable immune-complex mediated disease, in which cryoglobulins are type II and III of the immunochemical classification of Brouet. Essential mixed cryoglobulinemia, in opposition to secondary, is characterized by the absence of well-defined underlying disease and clinically it shows the classical triad of Meltzer and Franklin. Several reports point to the frequent, through various, liver involvement in essential-defined mixed cryoglobulinemia. Particularly, the clinical and biological evidence of liver disease is commonly soft, in spite of the common bioptic evidence of persistent or chronic active hepatitis. The problem about which comes first in this association had been widely investigated, including the possible pathogenetic role of hepatotropic viruses. Initially some studies focused attention on hepatitis B virus, but now its importance is de-emphasized because of critical epidemiological review, results of HBV-DNA in cryoprecipitates, electron microscopy data. The recent opportunity of diagnostic tests for hepatitis C virus gave proof of its great importance in apparently essential mixed cryoglobulinemia. In fact, many researches have found a variously high prevalence of anti HCV antibodies and HCV-RNA in serum and cryoprecipitates, with selective concentration in the latter. Alpha interferon treatment has been suggested as first choice drug in the management of HCV related cryoglobulinemia. The mechanism is postulated to be primarily due to its antiviral activity. Up to date optimal dose and treatment period needs to be established.
混合性冷球蛋白血症是一种可冷沉淀的免疫复合物介导的疾病,其中冷球蛋白属于布罗埃免疫化学分类中的II型和III型。与继发性混合性冷球蛋白血症相对,原发性混合性冷球蛋白血症的特点是缺乏明确的潜在疾病,临床上表现为梅尔策和富兰克林经典三联征。多项报告指出,在原发性混合性冷球蛋白血症中,肝脏经常通过多种方式受累。特别是,尽管肝活检常见持续性或慢性活动性肝炎的证据,但肝脏疾病的临床和生物学证据通常并不明显。关于这种关联中哪个因素先出现的问题已得到广泛研究,包括嗜肝病毒可能的致病作用。最初,一些研究关注乙型肝炎病毒,但由于严格的流行病学审查、冷沉淀中HBV-DNA的结果以及电子显微镜数据,现在其重要性已被淡化。丙型肝炎病毒诊断检测的最新进展证明了其在明显原发性混合性冷球蛋白血症中的重要性。事实上,许多研究发现血清和冷沉淀中抗HCV抗体和HCV-RNA的患病率各不相同且较高,且在后一种情况中有选择性浓缩。α干扰素治疗已被建议作为治疗丙型肝炎病毒相关冷球蛋白血症的首选药物。其机制据推测主要归因于其抗病毒活性。迄今为止,仍需要确定最佳剂量和治疗周期。