Hematol Oncol Clin North Am. 2022 Dec;36(6):1187-1199. doi: 10.1016/j.hoc.2022.08.001.
Patients with sickle cell disease and/or (rarely) trait are at increased risk for developing recurrent episodes of priapism, also known as stuttering priapism, and major ischemic priapism. Treatment of acute ischemic priapism is reactive; whereas ideal management consists of preventative approaches to ultimately promote the best improvement in patient's quality of life. Leg ulcers in patients with sickle cell disease (SCD) are quite common, with ∼20 % of patients with HBSS reporting either having an active or a past ucler. They can be confused with venous ulcers, with lower extremity hyperpigmentation confounding further the diagnosis. Several factors believed to contribute to the development of leg ulcers in patients with SCD are discussed in this article. Sickle cell liver disease (SCLD) occurs because of a wide variety of insults to the liver that happen during the lifetime of these patients. SCLD includes a range of complications of the hepatobiliary system and is increasing in prevalence with the aging adult sickle population. Liver nodular regenerative hyperplasia (NRH) is more common than realized and underappreciated as a diagnosis and requires liver biopsy with reticulin staining. Undiagnosed, the insidious damage from liver NRH can lead to noncirrhotic portal hypertension or cirrhosis.
镰状细胞病和/或（罕见）特征的患者发生复发性阴茎异常勃起（也称为顿挫性阴茎异常勃起）和主要缺血性阴茎异常勃起的风险增加。急性缺血性阴茎异常勃起的治疗是反应性的；而理想的治疗方法包括预防措施，最终促进患者生活质量的最佳改善。镰状细胞病（SCD）患者的腿部溃疡很常见，约 20%的 HBSS 患者报告有活动或过去的溃疡。它们可能与静脉溃疡混淆，下肢色素沉着进一步混淆诊断。本文讨论了被认为导致 SCD 患者腿部溃疡发展的几个因素。镰状细胞肝病（SCLD）是由于这些患者一生中发生的各种对肝脏的损伤引起的。SCLD 包括一系列肝胆系统并发症，随着成人镰状细胞人群的老龄化，其患病率也在增加。肝结节性再生性增生（NRH）比实际情况更为常见，且作为一种诊断方法被低估，需要进行肝活检和网状纤维染色。如果未被诊断，肝 NRH 的隐匿性损害可能导致非肝硬化性门静脉高压或肝硬化。
