Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis, IN, USA.
Department of Pathology, Albert Einstein College of Medicine, Bronx, NY, USA.
Am J Clin Pathol. 2022 Jan 6;157(1):73-81. doi: 10.1093/ajcp/aqab096.
Recent data on hepatic histopathology in patients with sickle cell disease (SCD) are lacking.
A total of 39 liver biopsies from SCD patients from 4 medical institutes were systematically evaluated.
The average age of patients was 27 years; 23 were female. The majority of the patients had hemoglobin SS (33), 3 had hemoglobin SC, and 3 sickle cell trait. Elevated liver functional tests and evaluation for cirrhosis were the main indications for biopsy. At the time of biopsy, most had elevated liver transaminases or hepatomegaly. The most common histopathologic abnormalities were Kupffer cell erythrophagocytosis (76.9%), hemosiderosis (74.4%), sinusoidal dilatation (71.8%), and intrasinusoidal sickled red cells (69.3%). Portal inflammation, lobular inflammation, and bile duct injury were mild to minimal and present in a minority of cases. Advanced fibrosis was present in 28.2% of the cases.
The typical histopathologic features seen in patients with SCD include Kupffer cell erythrophagocytosis, hemosiderosis, sinusoidal dilatation, and intrasinusoidal sickled red cells in a pauci-inflammatory or uninflamed background. Necrosis is less common than reported in older literature. Pathologists should be aware that significant portal and lobular inflammation, interface activity, and bile duct injury are unusual and may be suggestive of other etiologies.
目前缺乏镰状细胞病（SCD）患者肝脏组织病理学的最新数据。
系统性评估了来自 4 个医学研究所的 39 例 SCD 患者的肝活检。
患者的平均年龄为 27 岁，23 例为女性。大多数患者为血红蛋白 SS（33 例），3 例为血红蛋白 SC，3 例为镰状细胞特征。肝功能升高和肝硬化评估是进行活检的主要指征。在进行活检时，大多数患者的肝转氨酶升高或肝肿大。最常见的组织病理学异常是库普弗细胞含铁血黄素沉积（76.9%）、血色素沉着症（74.4%）、窦状扩张（71.8%）和窦内镰状红细胞（69.3%）。门管区炎症、肝小叶炎症和胆管损伤轻至中度，且在少数病例中存在。晚期纤维化见于 28.2%的病例。
SCD 患者的典型组织病理学特征包括库普弗细胞含铁血黄素沉积、血色素沉着症、窦状扩张和镰状红细胞在炎症反应较轻或无炎症背景下的窦内沉积。坏死较旧文献报道的少见。病理学家应该意识到，显著的门管区和肝小叶炎症、界面活动和胆管损伤是不常见的，可能提示其他病因。
