Monti G, Galli M, Invernizzi F, Pioltelli P, Saccardo F, Monteverde A, Pietrogrande M, Renoldi P, Bombardieri S, Bordin G
I Divisione di Medicina Generale, Saronno Hospital, Milan, Italy.
QJM. 1995 Feb;88(2):115-26.
In a multi-centre retrospective study, we compared clinical and laboratory data in 913 patients with cryoglobulinaemias, divided as: (i) essential cryoglobulinaemias; (ii) cryoglobulinaemias secondary to connective tissue diseases (CTD), lymphoproliferative or other haematological diseases (LPD), chronic liver diseases (CLD), and 'other diseases'. Purpura was the commonest presenting feature in all groups and was more common in essential cryoglobulinaemias (p < 0.0001). Meltzer's triad (purpura, arthralgia, weakness) was less frequent, but similarly distributed. Renal involvement was randomly distributed. Neurological impairment was less frequent in cryoglobulinaemias secondary to CLD (p < 0.002). Raynaud's phenomenon, arthritis and sicca syndrome were more frequent in cryoglobulinaemias secondary to CTD. Essential cryoglobulinaemias had a significantly higher percentage of serum complement C4 < 8 mg/dl (p < 0.004), of detectable rheumatoid factor activity (p < 0.0002), and of type II cryoglobulins (p < 0.0001). Liver involvement was evident at presentation in 32.6% of essential cryoglobulinaemias, 27.1% of cryoglobulinaemias secondary to LPD and 12.2% of cryoglobulinaemias secondary to CTD. Antibodies to hepatitis B surface (HBsAg) and core (HBc) antigens were more frequent in cryoglobulinaemias secondary to CLD; anti-HBs antibodies were randomly distributed. Antibodies to hepatitis C (HCV) were tested for in 224 patients, and prevalence was high in all the groups, but lower in cryoglobulinaemias secondary to CTD (p < 0.0001). Type II and type III essential cryoglobulinaemias differed significantly in renal involvement (p < 0.0001), cryocrit > 3% (p < 0.0001), C4 < 15 mg/dl (p < 0.001), HBsAg prevalence (p < 0.01) and purpura (p < 0.05). Despite the high prevalence of HCV markers in all groups, the role of HCV in essential cryoglobulinaemia is not well defined; HBV seems to play only a marginal role.
在一项多中心回顾性研究中，我们比较了913例冷球蛋白血症患者的临床和实验室数据，这些患者分为：（i）原发性冷球蛋白血症；（ii）继发于结缔组织病（CTD）、淋巴增殖性疾病或其他血液系统疾病（LPD）、慢性肝病（CLD）及“其他疾病”的冷球蛋白血症。紫癜是所有组中最常见的临床表现，在原发性冷球蛋白血症中更常见（p<0.0001）。梅尔策三联征（紫癜、关节痛、乏力）较少见，但分布相似。肾脏受累情况呈随机分布。继发于CLD的冷球蛋白血症患者神经功能障碍较少见（p<0.002）。雷诺现象、关节炎和干燥综合征在继发于CTD的冷球蛋白血症中更常见。原发性冷球蛋白血症患者血清补体C4<8mg/dl的比例显著更高（p<0.004），可检测到类风湿因子活性的比例更高（p<0.0002），II型冷球蛋白的比例更高（p<0.0001）。32.6%的原发性冷球蛋白血症患者、27.1%继发于LPD的冷球蛋白血症患者和12.2%继发于CTD的冷球蛋白血症患者在就诊时即有肝脏受累表现。乙型肝炎表面（HBsAg）和核心（HBc）抗原抗体在继发于CLD的冷球蛋白血症中更常见；抗-HBs抗体分布无明显差异。对224例患者检测了丙型肝炎（HCV）抗体，所有组中的患病率均较高，但继发于CTD的冷球蛋白血症中患病率较低（p<0.0001）。II型和III型原发性冷球蛋白血症在肾脏受累情况（p<0.0001）、冷球蛋白浓度>3%（p<0.0001）、C4<15mg/dl（p<0.001）、HBsAg患病率（p<0.01）和紫癜（p<0.05）方面存在显著差异。尽管所有组中HCV标志物的患病率都很高，但HCV在原发性冷球蛋白血症中的作用尚不明确；HBV似乎仅起边缘作用。
