Bombardieri S, Ferri C, Di Munno O, Pasero G
Ric Clin Lab. 1979 Oct-Dec;9(4):361-8. doi: 10.1007/BF02904572.
Twenty-one of 30 patients with essential mixed cryoglobulinemia (EMC) had evidence of liver involvement. The liver disease was characterized by the absence of clinical symptoms, hepatosplenomegaly, mild elevation of enzymes, abnormal BSP retention and low albumin levels. Histology, available in 12 patients, showed either chronic persistent or chronic active hepatitis or liver cirrhosis; 44% of the patients had HBsAg or HBsAb in sera and/or cryoglobulins, confirming the high frequency of exposure to hepatitis B virus (HBV) infection in EMC. However, liver lesions were similar in all patients, regardless of HBV exposure. Since other factors usually associated with chronic liver diseases were absent or apparently irrelevant, it is temptative to speculate that a 'cryoglobulinemic hepatitis' may exist as a distinct syndrome. The characteristic complement profile of the patients with EMC (low CH50 and C4, normal C3PA), not related to albumin levels, can help to differentiate this disease from chronic liver disease without cryoglobulins.
30例原发性混合性冷球蛋白血症(EMC)患者中有21例有肝脏受累的证据。肝脏疾病的特点是无临床症状、肝脾肿大、酶轻度升高、BSP潴留异常和白蛋白水平降低。12例患者的组织学检查显示为慢性持续性肝炎、慢性活动性肝炎或肝硬化;44%的患者血清和/或冷球蛋白中有HBsAg或HBsAb,证实EMC患者感染乙型肝炎病毒(HBV)的频率很高。然而,无论是否接触HBV,所有患者的肝脏病变都相似。由于通常与慢性肝病相关的其他因素不存在或明显无关,因此推测可能存在一种“冷球蛋白血症性肝炎”作为一种独特的综合征。EMC患者特征性的补体谱(CH50和C4降低,C3PA正常)与白蛋白水平无关,有助于将该疾病与无冷球蛋白的慢性肝病区分开来。