Saadoun David, Sellam Jérémie, Ghillani-Dalbin Pascale, Crecel Richard, Piette Jean-Charles, Cacoub Patrice
Department of Internal Medicine, Centre National de la Recherche Scientific Unité Mixte de Recherche 7087, Paris, France.
Arch Intern Med. 2006 Oct 23;166(19):2101-8. doi: 10.1001/archinte.166.19.2101.
Data on essential mixed cryoglobulinemia (MC) are scarce, and most date back to studies before 1989 (ie, before the discovery of hepatitis C virus [HCV] infection). Our objective was to describe the spectrum of MC in the era of HCV infection.
Retrospective study from a single university hospital's database of 1434 patients who tested positive for MC between January 1989 and December 2003.
One hundred thirty-three patients (9%) with persistent MC without HCV were included in the study. Sixty-five of 133 patients who fulfilled the criteria for MC vasculitis were compared with 118 patients with HCV-related MC vasculitis. The patients without HCV had increased frequencies of renal involvement and B-cell non-Hodgkin lymphoma (NHL), lower gammaglobulin levels, and higher death rates. Twenty-three of the patients had B-cell NHL (primarily of the lymphoplasmocytic and marginal zone types), and 8 patients had Sjögren syndrome. In multivariate analysis, a cryoglobulin level higher than 0.6 g/L (odds ratio [OR], 1.44) and the presence of MC vasculitis (OR, 4.3) and hypogammaglobulinemia (OR, 6.7) were independently associated with B-cell NHL. After a mean follow-up of 49.4 months, 18 (14%) of 133 patients had died, primarily of sepsis. In multivariate analysis, age at diagnosis older than 60 years (OR, 1.06) and renal involvement (OR, 5.20) were independently associated with death.
Patients with non-HCV-related MC vasculitis have a poor outcome and have a 4-fold increased risk of developing B-cell NHL.
关于原发性混合性冷球蛋白血症（MC）的数据稀缺，且大多数可追溯到1989年之前的研究（即丙型肝炎病毒[HCV]感染被发现之前）。我们的目的是描述HCV感染时代MC的情况。
对一家大学医院数据库中1989年1月至2003年12月期间MC检测呈阳性的1434例患者进行回顾性研究。
133例（9%）持续MC且无HCV感染的患者纳入研究。将133例符合MC血管炎标准的患者中的65例与118例HCV相关的MC血管炎患者进行比较。无HCV感染的患者肾脏受累和B细胞非霍奇金淋巴瘤（NHL）的发生率更高，γ球蛋白水平更低，死亡率更高。23例患者患有B细胞NHL（主要为淋巴浆细胞型和边缘区型），8例患者患有干燥综合征。多变量分析显示，冷球蛋白水平高于0.6 g/L（比值比[OR]，1.44）、存在MC血管炎（OR，4.3）和低γ球蛋白血症（OR，6.7）与B细胞NHL独立相关。平均随访49.4个月后，133例患者中有18例（14%）死亡，主要死于败血症。多变量分析显示，诊断时年龄大于60岁（OR，1.06）和肾脏受累（OR，5.20）与死亡独立相关。
非HCV相关的MC血管炎患者预后较差，发生B细胞NHL的风险增加4倍。
