LETTER TO THE EDITOR Fatal type B lactic acidosis in a patient with end-stage liver disease related to homozygous sickle cell disease Véronique Masy 1 & Etienne Sokal 2 & Nadejda Ranguelov 3 & Bénédicte Brichard 4 & Pierre-François Laterre 1 & Philippe Hantson 1 Received: 9 September 2019 /Accepted: 2 October 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019 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 condi- tion, with multi-organ failure as a terminal event. The follow- ing 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 ful- minant 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 seg- mental glomerulosclerosis connected with sickle cell ne- phropathy. A few months later, she developed a progres- sive liver disease with jaundice. Liver biopsy could rule out rejection and was in favor of sickle cell–related sinu- soidal and centrilobular damage. She had a progressive worsening of liver function, with a predominant cholestatic pattern, and of her chronic renal failure. She was treated by blood exchange transfusions to lower hemoglobin S levels. The recent hospital admission was justified by the worsening of kidney injury leading to intermittent hemo- dialysis. Additionally, she presented coagulation disorders in relationship with hepatic and renal failure, but also with a thrombocytopenia from both central and peripheral ori- gin. A catheter-related infection due to Staphylococcus epidermidis caused bacteremia. She complained of head- ache and a brain computed tomography revealed a limited subarachnoid hemorrhage at the convexity of the right frontal lobe, without evidence of intracranial hypertension. She then presented four episodes of generalized tonic- clonic seizures and was transferred to the ICU after having received a total of 16 mg midazolam and a loading dose of 750 mg intravenous levetiracetam. She was put on me- chanical ventilation, continuous venovenous hemofiltration (CVVH), and continuous electroencephalo- gram (EEG) monitoring. Admission laboratory investiga- tions revealed total bilirubin 59.9 mg/dL, ALAT 120 IU/L, INR 1.9, creatinine 5.39 mg/dL, platelet count 26,000/μL, hemoglobin S 0.7%, and arterial lactate 7.6 mmol/L. Hemodynamic conditions were stable without vasopres- sors. The continuous EEG monitoring confirmed the con- trol of epileptic activity and levetiracetam therapy (750 mg b.i.d) was maintained; the patient became progressively responsive to pain and voice. By contrast, lactic acidosis continued to progress despite bicarbonate administration and CVVH, and the patient died 24 h after ICU admission. There was no direct evidence for cardiogenic, hypovole- mic, or septic shock. Just before death, blood was drawn for lactate/pyruvate determination and a very high lactate/ pyruvate ratio was found (lactate 30.73 mmol/l, pyruvate 0.35 mmol/l, L/P = 87.8); additionally, hypoglycemia was noted with a ratio of blood 3-hydroxybutyric acid/ acetoacetate of 6.03. Mitochondrial toxicity was suspected as hyperlactatemia due to dysoxia seemed unlikely. The only medication that was introduced recently was leveti- racetam. Among the other medications simultaneously pre- scribed, none was considered a potential inducer of type B * Philippe Hantson philippe.hantson@uclouvain.be 1 Department of Intensive Care, Cliniques universitaires St-Luc, Université catholique de Louvain, Avenue Hippocrate, 10, 1200 Brussels, Belgium 2 Department of Pediatrics, Pediatric Gastroenterology and Hepatology Unit, Cliniques universitaires St-Luc, Université catholique de Louvain, Brussels, Belgium 3 Department of Pediatrics, Cliniques universitaires St-Luc, Université catholique de Louvain, Brussels, Belgium 4 Department of Pediatric Hemato-oncology, Cliniques universitaires St-Luc, Université catholique de Louvain, Brussels, Belgium Annals of Hematology https://doi.org/10.1007/s00277-019-03822-8