Abstract Intro: Sickle cell disease (SCD) affects more than 8 million people worldwide and liver disease is an important cause of morbidity and mortality. Some of the variants of acute sickle cell hepatopathy include acute sickle cell hepatic crisis, hepatic sequestration, intrahepatic cholestasis, and overt liver failure (Allali S et Al, Hepatobiliary Complications in Children with Sickle Cell Disease). Intrahepatic cholestasis (IC) accounts for less than 5% of all sickle cell (SC) hepatopathy and is considered a rare complication. It has an estimated mortality rate of 40% (Edwards CL et al, Intrahepatic cholestasis in sickle cell disease). IC is diagnosed clinically and supported by laboratory findings of marked conjugated hyperbilirubinemia, coagulopathy, leukocytosis with subsequent multiorgan failure. Management of SCIC has not been formalized. Some strategies considered have been initial simple blood transfusion or exchange transfusions. We present a case of SCIC that improved with exchange transfusion. This case adds to a growing evidence of rare occurrence of SCIC and emphasizes the need for elucidating formal guidelines on the management of SCIC. Case: A 27 year old male presented to the hospital for chest pain. The patient reported a history of presumed SC anemia with no SC crises since the age of six, he is not on any disease modifying medications and does not follow a hematologist. There was no confirmation that he has SC disease and notes from his primary care physician mentions that he has SC trait. On admission, the patient was noted to be hemodynamically stable but hypertensive suspected secondary to pain and hypoxic, saturating 96% on 4L nasal cannula. The physical exam was unremarkable except for 10/10 sternal chest pain with no radiation. Labs revealed leukocytosis 17k without bandemia, hemoglobin 7.7, MCV 72, platelets 172,000. Reticulocyte count 8%. Differential shows 1-2+ sickle cells. LDH 4500. Haptoglobin 11. Triglycerides 109. Ferritin greater than 16,500 with percent sat 93. Hyponatremia 125, anion gap acidosis of 19, elevated total bilirubin 6.6, with AST 5231 and ALT 2296. Hepatitis panel negative. EKG normal sinus rhythm. Troponins were unremarkable. Imaging revealed normal chest x-ray however CT chest revealed mild faint ground glass most confluent within the left lower lobe. CT abdomen and pelvis was unremarkable. Ultrasound of the liver with doppler showed mild hepatomegaly with normal waveforms. Hemoglobin electrophoresis resulted hemoglobin S 93.4%, hemoglobin A 2 3.7%, hemoglobin A 0%, hemoglobin 2.9% with fractionation pattern consistent with homozygous HbSS. The patient was admitted to the intensive care unit for SC crisis, concerning for vasocclusive disease in the liver and possible ground glass opacities in the LLL, and to be possibly started on exchange transfusion. He was started on IV hydration, pain management, folic acid supplementation and administered one unit of packed red blood cells. The patient had a central line placed for exchange transfusion due to presumptive SCIC. A liver biopsy was obtained and pathology shows liver with preserved hepatic architecture, marked lobular cholestasis, sinusoidal dilation and congestion with many sickled red blood cells and nucleated red blood cells, and patchy centrilobular necrosis. Overall, the findings are consistent with acute hepatic SCIC. Post red cell exchange, the hemoglobin electrophoresis resulted with a HbS of less than 20%. The patient symptomatically improved, with liver function test and total bilirubin returning to baseline. Discussion: The importance of this paper is to report a fatal variant and uncommon SC hepatic crisis. Review of the current literature regarding effective management has not been well elucidated. This patient improved with red blood cell exchange after a trial of simple transfusion had not achieved improvement. Some strategies considered have been initial simple blood transfusion, alternatively, if there is no improvement in liver disease, then exchange transfusion could be considered as the next intervention, with a goal HbS20-30% (Martí-Carvajal AJ et al, Interventions for treating intrahepatic cholestasis in people with sickle cell disease). However these recommendations are not well studied and mostly based on case reports and observational data. Further research is needed to elucidate optimal management of SCIC to establish a formal standard of care.
Jandir et al. (Mon,) studied this question.