Charlotte F, Bachir D, Nénert M, Mavier P, Galactéros F, Dhumeaux D, Zafrani E S
Service d'Anatomie et de Cytologie Pathologiques, Hôpital Henri Mondor, Créteil, France.
Arch Pathol Lab Med. 1995 Jan;119(1):46-52.
Hepatic lesions in sickle cell disease have been studied essentially in autopsy series. Previous reports on living patients are rare and concern a limited number of cases. The aim of the present study is to report the clinical, biochemical, and hepatic histological findings in 26 living patients with sickle cell disease and hepatobiliary disease.
Twenty-six of 510 patients with sickle cell disease, in whom liver tissue was available for histological analysis, were selected. In 21 patients, biopsy was obtained during laparotomy for cholecystectomy; 5 patients underwent needle biopsy for hepatomegaly and/or liver test abnormalities.
Twenty of the 21 cholecystectomized patients, as well as the 5 other patients, had liver vascular lesions consisting of sinusoidal dilatation (23 cases), perisinusoidal fibrosis (19 cases), and acute ischemic necrosis (5 cases). It is of interest that the 21 cholecystectomized patients had clinical signs of complicated cholelithiasis, and that 20 of them had gallbladder stones, with common bile duct lithiasis in only 1 case. In the 25 patients without common bile duct obstruction, symptoms might have been due to vascular lesions, but it must also be noted that in the cholecystectomized patients they did not persist or recur following surgery. In one cirrhotic patient, marked sinusoidal lesions might have favored severe hepatocellular failure that led to liver transplantation. In another patient, fatal hepatocellular insufficiency was possibly due to ischemia. The nonvascular lesions that were observed, ie, chronic persistent or mildly active hepatitis (11 cases) and cirrhosis (2 cases), were always associated with vascular lesions.
These results suggest that in sickle cell disease: (1) hepatic lesions are mainly vascular; (2) these lesions can be responsible for acute and/or chronic ischemia that may be severe; (3) symptoms suggestive of acute cholecystitis and/or biliary tract obstruction might be, at least in part, explained by vascular lesions; and (4) biliary tract surgery indications should be considered more carefully.
镰状细胞病的肝脏病变主要是在尸检系列中进行研究的。以往关于在世患者的报道很少，且涉及的病例数量有限。本研究的目的是报告26例患有镰状细胞病和肝胆疾病的在世患者的临床、生化及肝脏组织学检查结果。
从510例有肝脏组织可供组织学分析的镰状细胞病患者中选取了26例。21例患者在剖腹胆囊切除术时进行了活检；5例患者因肝肿大和/或肝功能检查异常接受了经皮肝穿刺活检。
21例接受胆囊切除术的患者中的20例，以及另外5例患者，存在肝脏血管病变，包括肝血窦扩张（23例）、肝血窦周围纤维化（19例）和急性缺血性坏死（5例）。有趣的是，21例接受胆囊切除术的患者有复杂性胆石症的临床体征，其中20例有胆囊结石，仅1例有胆总管结石。在25例无胆总管梗阻的患者中，症状可能归因于血管病变，但还必须注意的是，在接受胆囊切除术的患者中，这些症状在术后未持续或复发。在1例肝硬化患者中，明显的肝血窦病变可能促成了严重的肝细胞衰竭，进而导致肝移植。在另1例患者中，致命的肝细胞功能不全可能是缺血所致。观察到的非血管性病变，即慢性持续性或轻度活动性肝炎（11例）和肝硬化（2例），总是与血管性病变相关。
这些结果提示，在镰状细胞病中：（1）肝脏病变主要是血管性的；（2）这些病变可导致可能严重的急性和/或慢性缺血；（3）提示急性胆囊炎和/或胆道梗阻的症状可能至少部分由血管性病变解释；（4）应更仔细地考虑胆道手术指征。
