Introduction: Sickle cell disease (SCD) is an inherited autosomal recessive disorder which results in the production of hemoglobin S
(HbS). It is characterized by a wide-range of complications that vary in severity. Vaso-occlusive crisis is the most common
complication and accounts for the majority of admissions, while acute chest syndrome and sepsis are responsible for most of sickle
cell-related mortalities. Acute pancreatitis is a serious cause of acute abdominal pain that necessitates urgent hospital admission.
Acute ischemic pancreatic is an uncommon but documented cause of pancreatitis associated with different disorders including
connective tissue diseases, vasculitis, and shock. Case: We report a rare case of a 7 years old Saudi male patient with sickle cell
disease who developed acute pancreatitis that presented with severe abdominal pain, vomiting, and strikingly elevated pancreatic
enzymes. The patient was successfully managed with exchange blood transfusion and discharged in stable condition after 4 days of
admission at the hospital. Conclusion: Acute pancreatitis seldomly reported in association with SCD, patients presented with
abdominal vaso-occlusive crisis should have this diagnosis ruled out clinically and laboratory. Exchange blood transfusion versus
conservative management should be studied on large scale of patients to set up a clear guideline in case of pancreatitis occurring in
patients with SCD.
Keywords: Sickle cell disease, acute pancreatitis, exchange transfusion