P-096: ACUTE SICKLE CELL INTRAHEPATIC CHOLESTASIS: RARE DISEASE WITH UNUSUAL PRESENTATION, LESSONS LEARNT FROM AN INTERESTING CASE.

Abstract

Purpose: Management of Sickle cell disease in India the is evolving. The pediatric fatal disease is shifting to adult chronic organ disease. Sickle hepatopathy is an umbrella term which covers hepatic sickle crisis, acute hepatic sequestration crisis although uncommon but manageable. Acute sickle cell intrahepatic cholestasis (SCIC) is a rare phenomenon, difficult to treat and has very high mortality. The pathogenesis of Acute sickle cell intrahepatic cholestasis involves sickling within the hepatic sinusoids leading to vascular stasis, hypoxia, and ballooning of the hepatocytes, resulting in intracanalicular cholestasis. We describe a difficult case of Acute sickle cell intrahepatic cholestasis which has a subacute course, challenges faced, and lessons learnt from managing it. Materials and methods: A 14-year-old girl with sickle cell anemia who was non-compliant with hydroxyurea treatment presented to us with complaints of pain in the right side of abdomen, drowsiness, lethargy, nausea and loss of appetite. She was transfused twice at another center before reaching our center. On examination, she was disoriented, febrile, icteric, severely pale with active bleeding from gums and mouth and moderate hepatosplenomegaly. Hemoglobin was 7.2 grams/ dl with reticulocyte count of 2%, with neutrophilic leukocytosis and normal platelet count. Hemoglobin S was 42%. Serum bilirubin was 68 mg/dl with direct fraction of 65.3mg/dl. SGPT and SGOT were 263 and 202 IU/dl respectively and prothrombin time was raised with INR of 1.79. Viral hepatitis A, B, C and E were ruled out. Bacterial infections were ruled out. The possibility of Acute sickle cell intrahepatic cholestasis was considered, and the child was managed with partial exchange transfusion till Hemoglobin S was brought to <10%. She also received fresh frozen plasma in view of raised prothrombin time and active bleeding. Results: She did not improve until 14 days after admission.Gradually after 20 days she started talking and eating. However, liver enzymes and bilirubin normalized only after 40 days. She was discharged in stable condition and is continued on follow up. Conclusion: Sickle cell hepatopathy is an umbrella diagnosis for all the varied causes of hepatic dysfunction ranging from sickling crisis to transfusion transmitted viral infections. In our case, viral hepatitis was ruled out conclusively.A very rare condition Acute sickle cell intrahepatic cholestasis was considered in view of the clinical presentation serum bilirubin was extremely high and mildly elevated transaminases. However. Hepatic sequestration was unlikely based on the hemoglobin level and reticulocyte count. Differentiating the varied causes in resource constrained centers can be often a daunting task and multipronged management needs to be initiated at the earliest to ensure a positive outcome. The authors do not declare any conflict of interest