Annals of Hematology

, Volume 98, Issue 11, pp 2627–2628 | Cite as

Fatal type B lactic acidosis in a patient with end-stage liver disease related to homozygous sickle cell disease

  • Véronique Masy
  • Etienne Sokal
  • Nadejda Ranguelov
  • Bénédicte Brichard
  • Pierre-François Laterre
  • Philippe HantsonEmail author
Letter to the Editor

Hepatic involvement by sickle cell disease (SCD) can result in a variety of symptoms ranging from mild to life-threatening. Acute intrahepatic cholestasis is a rare but often fatal condition, with multi-organ failure as a terminal event. The following observation is suggesting that extreme hyperbilirubinemia may be associated with energetic failure.

A 16-year-old girl was admitted to the intensive care unit (ICU) for seizures. She had a complicated medical past history in relation to homozygous SCD. At the age of 3 months, she received a liver transplantation for a fulminant hepatic failure due to neonatal hemochromatosis. At the age of 15, she developed a chronic renal failure with a corticoresistant nephrotic syndrome due to a membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis connected with sickle cell nephropathy. A few months later, she developed a progressive liver disease with jaundice. Liver biopsy could rule out rejection and was in favor of sickle...


Compliance with ethical standards


An informed consent was obtained from the relatives.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.


  1. 1.
    Finsterer J, Scorza FA (2017) Effects of antiepileptic drugs on mitochondrial functions, morphology, kinetics, biogenesis, and survival. Epilepsy Res. 136:5–11CrossRefGoogle Scholar
  2. 2.
    Lipka K, Bülow HH (2003) Lactic acidosis following convulsions. Acta Anaesthesiol Scand. 47:616–618CrossRefGoogle Scholar
  3. 3.
    Karacaoglu PK, Asma S, Korur A et al (2016) East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients. Ann Hematol. 95:993–1000CrossRefGoogle Scholar
  4. 4.
    Manci EA, Culberson DE, Yang YM, Gardner TM, Powell R, Haynes J Jr, Shah AK, Mankad VN, Investigators of the Cooperative Study of Sickle Cell Disease (2003) Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 123:359–365CrossRefGoogle Scholar
  5. 5.
    Haydek JP, Taborda C, Shah R, Reshamwala PA, McLemore M, Rassi FE, Chawla S (2019) Extreme hyperbilirubinemia: an indicator of morbidity and mortality in sickle cell disease. World J Hepatol. 11:287–293CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Intensive Care, Cliniques universitaires St-LucUniversité catholique de LouvainBrusselsBelgium
  2. 2.Department of Pediatrics, Pediatric Gastroenterology and Hepatology Unit, Cliniques universitaires St-LucUniversité catholique de LouvainBrusselsBelgium
  3. 3.Department of Pediatrics, Cliniques universitaires St-LucUniversité catholique de LouvainBrusselsBelgium
  4. 4.Department of Pediatric Hemato-oncology, Cliniques universitaires St-LucUniversité catholique de LouvainBrusselsBelgium

Personalised recommendations