Cavalcanti R, Pol S, Carnot F, Campos H, Degott C, Driss F, Legendre C, Kreis H
Service de Transplantation and Hépatologie, Hôpital Necker, Paris, France.
Transplantation. 1994 Aug 15;58(3):315-6.
Peliosis hepatis and hepatic sinusoidal dilatations are rare vascular liver diseases that occur at increased frequency in kidney transplant recipients. We retrospectively evaluated in kidney transplant recipients the natural history of vascular liver diseases, their impact on patient and graft survival, and the influence of AZA withdrawal. Between 1970 and 1990, vascular liver disease was diagnosed in 32 cadaver kidney transplant recipients 1-128 months after transplantation (mean 41 months). Diagnosis was based on histology in all cases. Patients received conventional immunosuppression (high dose steroids and AZA). Twenty patients had a minor form (sinusoidal dilatations or focal peliosis), while 12 had a major form (diffuse peliosis) of vascular hepatic disease. Two patients were lost to follow-up and 1 died at the time of diagnosis. In 12 patients (group 1), AZA dosage remained unchanged, while it was interrupted at the time of diagnosis in 17 patients (group 2). Five group 1 patients underwent serial liver biopsies, which showed persistence of vascular hepatic disease in 3 (with regenerative nodular hyperplasia in 1) and disappearance in 2 patients. Eight group 2 patients underwent serial liver biopsies, which showed disappearance of vascular hepatic disease in 6 patients and persistence in 2. Moreover, regenerative nodular hyperplasia was noted in 1 case, perisinusoidal fibrosis in 1 case, and cirrhosis in 6 cases. Three patients of group 1 and 11 patients of group 2 returned to dialysis a mean of 21 and 39 months after diagnosis, respectively. Eight patients died and death was clearly associated with major peliosis in 2 cases. In kidney transplant recipients, vascular hepatic disease may be associated with high mortality, especially in major forms. Our findings indicate that peliosis hepatis may lead to severe fibrosing liver lesions. The course of vascular hepatic disease is not clearly modified by AZA withdrawal.
肝紫癜和肝血窦扩张是罕见的肝脏血管疾病,在肾移植受者中发病率增加。我们回顾性评估了肾移植受者中肝脏血管疾病的自然病史、其对患者和移植物存活的影响以及停用硫唑嘌呤(AZA)的影响。1970年至1990年间,32例尸体肾移植受者在移植后1至128个月(平均41个月)被诊断出患有肝脏血管疾病。所有病例的诊断均基于组织学检查。患者接受常规免疫抑制治疗(大剂量类固醇和AZA)。20例患者为轻度形式(血窦扩张或局灶性紫癜),而12例患者为重度形式(弥漫性紫癜)的肝脏血管疾病。2例患者失访,1例在诊断时死亡。12例患者(第1组)的AZA剂量保持不变,而17例患者(第2组)在诊断时停用了AZA。第1组的5例患者接受了系列肝脏活检,其中3例显示肝脏血管疾病持续存在(1例伴有再生结节性增生),2例消失。第2组的8例患者接受了系列肝脏活检,其中6例显示肝脏血管疾病消失,2例持续存在。此外,1例出现再生结节性增生,1例出现窦周纤维化,6例出现肝硬化。第1组的3例患者和第2组的11例患者分别在诊断后平均21个月和39个月恢复透析。8例患者死亡,其中2例死亡与重度紫癜明显相关。在肾移植受者中,肝脏血管疾病可能与高死亡率相关,尤其是重度形式。我们的研究结果表明,肝紫癜可能导致严重的肝纤维化病变。停用AZA并不能明显改变肝脏血管疾病的病程。