Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

Abstract

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.

Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients

“…The reported incidence of hepatic crises is about 10% in adults with SCD [4], which is higher than in our series. The severity of crises in our patients agrees with previous reports [3,4,6,8,11], with a lower mortality in children than adults [10,11], probably because of fewer co-morbidities: iron overload, alcohol, drugs (such as cocaine), or viral hepatitis [18]. However, our patients’ good outcome may also be due to our strategy of early exchange transfusion in all children with hepatic crisis (liver pain and elevated ALT and/or conjugated bilirubin levels), as soon as a biliary complication is excluded.…”

“…Despite several studies on hepatic complications in adults with SCD [3,4,6,8,15], reports in children are few and are mainly case series [10,11,12]. Here we report hepatobiliary manifestations in a very large cohort of SCD children ( n = 616), ranging from highly frequent and usually poorly symptomatic cholelithiasis to rare and life-threatening complications.…”

“…In children, severe liver disease is rarely reported but might be underdiagnosed and insufficiently treated, because of misinterpretation of liver tests and possible confusion between hemolysis and liver disease markers [9]. Large series are rare, reporting mild elevation of transaminases and cholelithiasis as the main hepatobiliary abnormalities [9], and most studies are case series [10,11,12]. Here we assessed the prevalence of hepatobiliary manifestations in our cohort of more than 600 patients.…”

Epstein-Barr virus induced sickle hepatopathy

“…The clinical entities represented under the rubric of sickle hepatopathy (Table 1) encompass a disease spectrum; clinical manifestations vary depending on the relative degrees of cell trapping, ischemia, and intracanalicular cholestasis. [1][2][3][4][5][6] There is overlap among these entities, so precise classification is often challenging. Patient 1 had a clinical course best classified as hepatic sequestration, whereas Patient 2 experienced intrahepatic cholestasis with multi-organ failure.…”

“…Sickle hepatopathy of childhood encompasses a group of disorders ranging in severity from mild liver pain to multi-organ failure (Table 1). [1][2][3][4][5][6] These diverse clinical manifestations are thought to reflect varying degrees of erythrocyte sickling, Kupffer cell hyperplasia, sinusoidal obstruction, hepatocyte ischemia, and intracanalicular cholestasis. 2,5,7 Sickle hepatopathy can arise spontaneously or may be triggered by infection or autoimmune diseases.…”