Sickle cell hepatopathy
Sickle cell hepatopathy
Description, Causes and Risk Factors:
Sickle cell hepatopathy encompasses a range of hepatic pathology arising from a wide variety of insults to the liver in patients with sickle cell disease. It occurs predominantly in patients with homozygous sickle cell anemia, and to a lesser extent in patients with sickle cell trait, HbSC disease and HbS/ß-Thalassemia. The hepatic disease may primarily be caused by the sickling process, but more commonly arises as a consequence of the multiple transfusions that these patients require in their lifetime. The term multitransfusion hepatopathy may therefore be more appropriate for these latter patients.
The main hepatic complications of multiple transfusions include acute and chronic infection with hepatitis B and C and iron overload. A further potential consequence of the chronic hemolysis is the development of pigment stones, with consequent cholecystitis and acute and chronic biliary obstruction from choledocholithiasis.
Unfortunately, much of the literature on sickle cell hepatopathy lacks depth, because there are few sizeable or controlled studies, and most studies provide only incomplete information regarding coexisting chronic infection with hepatitis B or C, hepatic iron deposition, and chronic biliary obstruction from choledocholithiasis. It is therefore difficult to evaluate the contributing effects of these various insults in the final pathologic and clinical picture in these patients. As will be discussed subsequently, sickle cell anemia may lead to several unique liver syndromes.
Furthermore but less common, hepatic injury can be directly related to the sickling process and hence the term most often used to describe these syndromes is sickle cell hepatopathy e.g., acute hepatic crisis and hepatic sequestration crisis. Sickle cell intrahepatic cholestasis is a rare form of sickle cell hepatopathy. It is potentially fatal with a poor prognosis. Therefore, early identification is essential. It is thought to represent a severe form of hepatic crisis.
Symptoms may include:
RUQ (right upper quadrant) abdominal pain.
Signs may include:
AST (aspartate aminotransferase) and ALT (alanine aminotransferase) rarely exceed 300 IU/L.
Serum bilirubin levels usually less than 15 mg/dL.
Differential diagnosis may include: sickle cell intrahepatic cholestasis, acute viral hepatitis, cholecystitis, and choledocholithiasis with common bile duct obstruction.
Computerized Tomography: Diffuse hepatomegaly.
Magnetic Resonance Imaging: Transfusion dependent patients usually show decreased signal intensity in the liver, pancreas, and spleen due to iron deposition.
Liver & spleen scan.
Ultrasound: Bile gallstones, increased echogenicity due to iron deposition.
Liver biopsy is important in establishing the correct diagnosis and in instituting appropriate management, though it should be borne in mind that the patient may have another distinct liver disease co-existing with sickle-cell disease.
The goals of treating sickle cell hepatopathy are to relieve pain; prevent infections, eye damage, and strokes; and control complications if they occur. The treatments include medicine, blood transfusions, and specific treatment for complications.
Do not forget to look for liver injury.
Rule out other common causes of acute liver injury.
Distinguish between acute syndrome and chronic multitransfusion hepatopathy.
Do not transfuse unless necessary and if so, do exchange.
Be aware of zinc deficiency.
Avoid hepatotoxic drugs.
Abnormal LFTs and bilirubin are coming in sickle cell hepatopathy.
Some researchers believe that bone marrow transplants may offer a cure in a small number of cases. Researchers are looking for new treatments for sickle cell hepatopathy, including gene therapy and safer and more effective bone marrow transplants.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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