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Hepatic sickling crisis mimicking recurrent cholangitis
S Mehta1, A Nagral, V K Sucheta
1Division of Digestive Diseases and Clinical Nutrition, Tata Memorial Hospital, Mumbai.
Summary
Sickle cell disease can cause hepatic sickling crisis, a condition diagnosed via liver biopsy. Hydroxyurea therapy effectively treated the patient's symptoms, highlighting biopsy's diagnostic role.
Area of Science:
- Hematology
- Hepatology
- Pathology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- Hepatic complications in SCD can be severe and challenging to diagnose.
- Hepatic sickling crisis is a specific vaso-occlusive event affecting the liver in SCD patients.
Observation:
- A 22-year-old male with homozygous sickle cell disease presented with fever, right upper quadrant pain, and jaundice.
- Clinical presentation suggested an acute hepatic event.
- Liver biopsy was performed for diagnostic clarification.
Findings:
- Liver biopsy confirmed the presence of sickled red blood cells within the hepatic sinusoids, diagnosing hepatic sickling crisis.
- The patient's symptoms significantly improved following treatment with hydroxyurea therapy.
- Hepatic sickling crisis can be definitively diagnosed through liver biopsy, not solely as a diagnosis of exclusion.
Implications:
- Liver biopsy is a valuable tool for diagnosing hepatic sickling crisis in sickle cell disease.
- Early diagnosis and appropriate treatment, such as hydroxyurea, can lead to favorable outcomes.
- This case underscores the importance of considering specific hepatic complications in SCD management.