From the Guidelines
Sickle cell crisis can indeed cause elevated liver enzymes, also known as transaminitis, due to the obstruction of blood flow in the liver's sinusoids by abnormal sickled red blood cells, leading to ischemia, hypoxia, and hepatocyte damage. This is supported by the pathophysiology of sickle cell disease, where the abnormal beta globin gene (ßs) and formation of abnormal haemoglobin, HbS (a2 ßs2), can lead to polymerization and sickling of red blood cells, causing vascular occlusion and tissue damage 1. During a sickle cell crisis, the liver's sinusoids can become obstructed, resulting in hepatic injury and the release of transaminases into the bloodstream.
Some key points to consider in the management of sickle cell crisis and its impact on liver function include:
- The severity of sickle cell disease varies according to genotype and within genotype, with HbSS, HbSß0-thalassaemia, and HbSD disease being associated with severe sickle cell disease and early onset of painful crises 1
- Fetal haemoglobin (HbF) can reduce the polymerization of HbS molecules and offer some protection in sickle cell disease, with patients having high HbF levels (> 8%) tend to have a mild phenotype with fewer symptoms 1
- Hydroxycarbamide can raise the HbF level and is now recommended as standard therapy for many patients with sickle cell disease 1
- Monitoring liver function tests during and after sickle cell crises is important for assessing hepatic involvement and guiding management decisions, as repeated episodes of sickling in the liver can lead to sickle cell hepatopathy, a spectrum of liver abnormalities from acute hepatic crisis to chronic liver disease.
The liver dysfunction associated with sickle cell crisis is generally transient and resolves with appropriate management of the underlying condition, which includes hydration, pain control, and oxygen therapy if needed. However, in some cases, the transaminitis can be severe, and close monitoring of liver function is essential to prevent long-term liver damage. Therefore, it is crucial to monitor liver function tests in patients with sickle cell disease, especially during crises, to promptly identify and manage any hepatic involvement.
From the Research
Sickle Cell Crisis and Elevated Liver Enzymes
- Sickle cell disease (SCD) can cause microvascular occlusion and hemolytic anemia, leading to various complications, including liver disorders 2.
- Acute liver disorders in SCD can include acute vaso-occlusive crisis, liver infarction, and acute hepatic crisis, which may cause elevated liver enzymes 2.
- A case report of a patient with SCD presented with hyperhemolysis syndrome, which led to transaminitis, indicating that sickle cell crisis can cause elevated liver enzymes 3.
- Another case report described a patient with SCD who developed acute liver failure due to sickle cell hepatopathy, which was treated with exchange transfusion 4.
- A study found that liver involvement occurs in 39% of patients with SCD during acute vaso-occlusive crisis, with hepatocellular-type injury, cholestatic, or mixed patterns 5.
- Research also showed that liver stiffness increases acutely during sickle cell vaso-occlusive crisis, and this increase is associated with elevated alanine aminotransferase (ALT) and alkaline phosphatase levels 6.
Key Findings
- Sickle cell crisis can cause elevated liver enzymes, including ALT and alkaline phosphatase 3, 6.
- Liver involvement is common in SCD patients during acute vaso-occlusive crisis, with a prevalence of 39% 5.
- Transient elastography (TE) may be a useful marker for hepatic involvement during VOC, as it correlates with fibrosis and tricuspid regurgitation velocity (TRV) 6.
- Elevated liver enzymes and liver stiffness during VOC may indicate hepatic involvement and passive congestion 6.