Bauer T W, Moore G W, Hutchins G M
Am J Med. 1980 Dec;69(6):833-7. doi: 10.1016/s0002-9343(80)80008-8.
In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. We studied 70 patients with sickle cell disease (57 SS, 12 SC and one S-thalassemia (S-thal) hemoglobin) autopsied at The Johns Hopkins Hospital. They ranged in age from five months to 75 years (average 21 years) and 35 (50 percent) were female, In 64 patients (91 percent), livers were enlarged and had distention of Kupffer cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27 percent) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34 percent) and were explained in 12, eight by cardiac dysfunction and four by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20 percent), only one of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in seven patients and cardiac cirrhosis in one. Cirrhosis with hemochromatosis was present in three patients and 30 others had parenchymal iron accumulation. Thus, unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequently encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.
在临床研究中，已注意到镰状细胞病危象常伴有肝功能障碍，但这些短暂发作是否会导致不可逆的形态学改变尚不确定。我们研究了在约翰霍普金斯医院接受尸检的70例镰状细胞病患者（57例SS型、12例SC型和1例S-地中海贫血（S-thal）血红蛋白型）。他们的年龄从5个月至75岁不等（平均21岁），35例（50%）为女性。64例患者（91%）肝脏肿大，库普弗细胞扩张并吞噬镰状红细胞；其中10例情况严重。19例患者（27%）的肝血窦因镰状红细胞而明显扩张，似乎出现了阻塞。24例患者（34%）存在局灶性实质坏死，其中12例得到了解释，8例由心功能不全导致，4例由败血症导致。14例患者（20%）出现了修复性改变、门脉纤维化和再生结节，尽管输血频繁，但只有1例有已知的病毒性肝炎病史。7例患者出现不明原因的肝硬化，1例出现心源性肝硬化。3例患者出现伴有血色素沉着症的肝硬化，另外30例有实质铁沉积。因此，这些患者经常出现无法解释的肝坏死、门脉纤维化、再生结节和肝硬化。这种肝病谱似乎最好被理解为镰状细胞病反复出现血管阻塞、坏死和修复的结果。
