Advertisement

Sickle Cell Intrahepatic Cholestasis with Acute Liver Failure and Acute Kidney Injury: Favourable Outcome with Exchange Transfusion

  • Prabir Maji
  • Rohan MalikEmail author
  • Rakesh Lodha
  • Arvind Bagga
Scientific Letter
  • 53 Downloads

To the Editor: An 11-y-old girl presented with 2 wk of jaundice, right upper abdominal pain and previous history of two episodes of jaundice. Examination revealed markedly deep icterus and hepatosplenomgaly. Laboratory investigation showed evidence of hemolysis (hemoglobin 9.1 g/dl, reticulocytosis 19%, fragmented RBCs, Lactate dehydrogenase 1674 U/L, negative direct and indirect Coombs test), liver dysfunction (total serum bilirubin 68 mg/dl, direct 58 mg/dl, Asparate aminotransferase 100 IU/L, Alanine aminotransferase 200 IU/L, Alkaline phosphatase 660 IU/L, prothrombin time - International normalised ratio 1.6) and Acute kidney injury (AKI) (urea/creatinine 52/1.5 mg/dl, microscopic hematuria). Serology for Dengue, Chikungunya, Hepatitis viruses, Malaria and Leptospira was negative. Within 24 h she progressed to encephalopathy (Grade II), coagulatopathy (INR 2.4), further hemolysis (Hb 5 g/dl), anuria and metabolic acidosis. Considering Acute Wilsonian crises which presents with this constellation of findings (Acute liver failure, Coomb’s negative hemolysis, AKI), plasmapheresis (2 cycles) and hemodialysis was initiated. Meanwhile, investigations for Wilsons disease were normal and repeat blood smear demonstrated multiple sickle shaped cells. Acute sickle cell intrahepatic cholestasis (SCIC) was considered. Single volume exchange transfusion (EBT) was done. Post exchange HPLC showed HbS 6.1% (both parents had sickle cell trait). Hydroxyurea was started. She gradually improved over next 45 d and renal and liver function normalized.

Acute SCIC is a rare and usually fatal complication of sickle cell disease (SCD) and should be suspected when a patient presents with very high level of conjugated bilirubin and right upper quadrant pain. The progression to AKI, coagulopathy and liver failure is rapid unless timely interventions are instituted and hence the index of suspicion should be high. The pathophysiology is, extensive adhesion of sickled RBCs to endothelium causing sluggish flow, sinusoidal congestion, ischemia, infarction and hepatic dysfunction [1, 2, 3]. Exchange blood transfusion has been shown to be beneficial by lowering the fraction of HbS [4]. However, even after aggressive EBT and liver transplantation, outcomes may be poor [5]. In light of the published poor outcome in SCIC with encephalopathy, liver failure and renal dysfunction, the case is unique with successful outcome with exchange transfusion, Hydroxyurea and hemodialysis.

Notes

Compliance with Ethical Standards

Conflict of Interest

None.

References

  1. 1.
    Costa DB, Miksad RA, Buff MS, Wang Y, Dezube BJ. Case of fatal sickle cell intrahepatic cholestasis despite use of exchange transfusion in an African-American patient. J Natl Med Assoc. 2006;98:1183–7.Google Scholar
  2. 2.
    Sheehy TW. Sickle cell hepatopathy. South Med J. 1977;70:533–8.CrossRefGoogle Scholar
  3. 3.
    Banerjee S, Owen C, Chopra S. Sickle cell hepatopathy. Hepatology. 2001:331021–8.Google Scholar
  4. 4.
    Ahn H, Li CS, Wang W. Sickle cell hepatopathy: clinical presentation, treatment and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005;45:184–90.CrossRefGoogle Scholar
  5. 5.
    Blinder MA, Geng B, Lisker-Melman M, et al. Successful orthotopic liver transplantation in an adult patient with sickle cell disease and review of the literature. Hematol Rep. 2013;5:1–4.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2019

Authors and Affiliations

  1. 1.Division of Gastroenterology, Hepatology and Clinical Nutrition, Department of PediatricsAIIMSNew DelhiIndia
  2. 2.Division of Pulmonology and Intensive Care, Department of PediatricsAIIMSNew DelhiIndia
  3. 3.Division of Nephrology, Department of PediatricsAIIMSNew DelhiIndia

Personalised recommendations