Autoimmune Liver Disease in Children with Sickle Cell Disease

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

“…Severe hyperbilirubinemia may spontaneously resolve with supportive care (particularly in children) or present with multisystem organ failure requiring intensive care . Sickle hepatopathy is also associated with sepsis, viral hepatitis, autoimmune disease, and transfusional iron overload . This is a case series of four young patients with hemoglobin SS (HbSS) whose successive presentations with complex hepatobiliary disease at two institutions underscore the protean presentations of SCD liver injury, the need to consider broad differential diagnoses, and fundamental management considerations.…”

Haploidentical stem cell transplantation cures autoimmune hepatitis and cerebrovascular disease in a patient with sickle cell disease

“…[1] Among SCD patients, autoimmune disorders, such as autoimmune hepatitis (AIH) or systemic lupus erythematosus, are not infrequent and are difficult to treat due to the high risk of adverse events associated with steroids or other immunosuppressive treatments. [2][3][4] Hematopoietic Stem Cell Transplantation (HSCT) is the most consolidated curative therapy for patients with SCD, but benefits and risks in both the short and long term must be carefully considered, taking into consideration disease variability and the recent appearance of new drugs. Despite the progressive broadening of indications for HSCT, [5] the majority of pediatric haematologists still offers it only to patients with a severe phenotype: cerebrovascular disease, recurrent vasoocclusive crises (VOC) or acute chest syndrome not responsive to Hydroxycarbamide, or sickle nephropathy.…”

HCV Infection in Thalassemia Syndromes and Hemoglobinopathies: New Perspectives

“…Moreover, due to the need of blood transfusions to manage the sickling crisis and anemia, patients affected by HCV infection frequently are exposed to higher risk of iron overload and hemosiderosis, leading to liverrelated morbidity and mortality (Moon et al, 2017). The hepatic involvement ranges in severity from liver dysfunction to liver failure, and occurs as the result of many factors: (a) sickling process (with acute hepatic vaso occlusion, hepatic sickle cell cholestasis, liver ischemia, and reperfusion injury, hemolysis, and cholelithiasis); (b) chronic viral hepatitis; (c) transfusion related hemosiderosis; and (d) autoimmune liver disease (Porter and Garbowski, 2013;Jitraruch et al, 2017;Theocharidou and Suddle, 2019).…”