Abstract
Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution.
Highlights
Sickle cell disease (SCD) is the most common hemoglobinopathy, and there are approximately 100,000 patients in the United States with the disease [1,2]
Sickle cell hepatopathy is a broad term encompassing numerous changes seen in SCD which may be caused by physiologic sickling and sequestration, iron overload, various hepatidides, or other multifactorial causes
Reported cases of sickle cell intrahepatic cholestasis (SCIC) have been routinely managed with red blood cell exchange transfusion (RBCEx)
Summary
Sickle cell disease (SCD) is the most common hemoglobinopathy, and there are approximately 100,000 patients in the United States with the disease [1,2]. Cases of SCIC in adults treated with supportive therapy, such as simple transfusion and peritoneal dialysis, were associated with 88% mortality (Table 1), compared to pediatric cases where survival was more commonly reported [17,18,19]. In 1980, use of partial red cell exchange in the management of an adult SCD patient with SCIC, resulting in survival, was first described [18]. Reported cases of SCIC have been routinely managed with red blood cell exchange transfusion (RBCEx). Management of chronic SCIC is less extensively characterized, due to a paucity of reported clinical cases, and to the clinical overlap with other progressive liver disease occurring in SCD patients, such as autoimmune hepatitis, iron overload, or viral hepatitis. Two reported patients with chronic SCIC have been successfully managed with orthotopic liver transplant (OLT) [5,12]. The significant risk of acute mortality or requirement for OLT in chronic disease supports the use of RBCEx therapy, if available
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