Nakanuma Y, Wada M, Kono N, Miyamura H, Ohta G
Virchows Arch A Pathol Anat Histol. 1981;393(2):123-32. doi: 10.1007/BF00431069.
A 43-year-old woman with a history of photosensitivity died of hepatic failure following 3 and a half months of unexplained jaundice. The liver was black, showed mild fibrosis and conspicuous pigment deposition in the cytoplasm of the hepatocytes, Kupffer cells and portal macrophages, and within dilated lumina of bile canaliculi and of ductules. The pigment disclosed a striking birefringence and numerous slender electron-dense crystals on electron microscopy. Similar crystals were also found within the cytoplasm of the ductular epithelium. Despite absence of cirrhosis observed in almost all previously described fatal cases the diagnosis of erythropoietic liver with marked protoporphyrin deposition so far have not been described in the cases observed at autopsy.
一名有光敏史的43岁女性，在出现不明原因黄疸3个半月后死于肝衰竭。肝脏呈黑色，有轻度纤维化，肝细胞、库普弗细胞和门脉巨噬细胞的细胞质以及胆小管和小胆管扩张的管腔内有明显的色素沉积。该色素在电子显微镜下显示出显著的双折射和大量细长的电子致密晶体。在小胆管上皮细胞的细胞质中也发现了类似的晶体。尽管在几乎所有先前描述的致命病例中均未观察到肝硬化，但尸检病例中迄今尚未描述有明显原卟啉沉积的造血性肝病的诊断。
