PATHOLOGICAL SPECTRUM OF LIVER-DISEASES IN SICKLE-CELL DISEASE
PATHOLOGICAL SPECTRUM OF LIVER-DISEASES IN SICKLE-CELL DISEASE
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DOI：
10.1007/bf01318115
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发表时间：
1986-03-01
影响因子：
3.1
通讯作者：
TATTER, D
中科院分区：
文献类型：
--
作者：
OMATA, M;JOHNSON, CS;TATTER, D
大多数镰状细胞性贫血及其变体的肝脏疾病的病理学研究进行了回顾性尸检标本，并且，由于突出的组织学特征的窦内镰状和枯否细胞吞噬红细胞，肝功能障碍归因于肝内镰状红细胞在这种血红蛋白病。我们比较了19例肝活检患者的肝组织学和32例死于血红蛋白病并发症患者的尸检标本。在前者中，9例患者有病毒性肝炎的组织学证据。其中4例患者同时具有B型肝炎表面抗原的血清学和免疫组化证据。胆道树梗阻2例，酒精性肝硬化和结节样肉芽肿各1例。只有一名患者，谁从败血性休克恢复，显示缺血性坏死。5例胆囊切除术中偶然活检，未发现明显病变，14例尸检标本显示缺血性坏死，与活检组比较有显著性差异。10例除有大量铁沉积外，无明显形态学改变。慢性活动性肝炎2例，弥漫性纤维化2例，肝硬化、急性病毒性肝炎、胆汁淤积和巨细胞性肝炎各1例。肝内镰状化和红细胞吞噬在几乎所有的标本中可见，与肝脏疾病或转氨酶升高无关。除了感染性休克的病人，缺血性坏死只发现在死后材料。这些组织学特征可能代表红细胞破坏，而不是这些患者肝脏疾病的病因。
Most pathologic studies of liver disease in sickle cell anemia and its variants were performed retrospectively on autopsy specimens, and, because of the priminent histologic features of intrasinusoidal sickling and Kupffer cell erythrophagocytosis, hepatic dysfunction was attributed to the intrahepatic sickling of erythrocytes in this hemoglobinopathy. We compared the liver histology from 19 patients who had liver biopsies to the autopsy specimens from 32 patients who succumbed to the complications of the hemoglobinopathy. In the former, nine patients had histological evidence of viral hepatitis. Four of these patients had both serological and immunohistochemical evidence of hepatitis B surface antigen. The features of biliary tree obstruction were found in two cases and alcoholic cirrhosis and sarcoid granuloma in one case each. Only one patient, who had recovered from septic shock, showed ischemic necrosis. In five patients incidentally biopsied during cholecystectomy, no significantly lesions were found. Fourteen of the autopsy specimens showed ischemic necrosis, a result which was significantly different from the biopsy group. Ten cases had no significant morphologic changes other than heavy iron deposits. There were two cases with chronic active hepatitis, two with diffuse fibrosis, and one case each of cirrhosis, acute viral hepatitis, cholestasis, and giant cell hepatitis. Intrahepatic sickling and erythrophagocytosis were seen in almost all specimens and did not correlate with liver disease or transaminase elevation. Other than the patient with septic shock, ischemic necrosis was found only in postmortem material. These histological features may represent red cell destruction rather than the etiology of liver disease in these patients.