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Colonic pseudo-obstruction in sickle cell disease
Huxley Knox-Macaulay1, Mohiyaddin Ayyaril, Nadeem Nusrat
1Department of Haematology, College of Medicine, Sultan Qaboos University and Sultan Qaboos University Hospital, Al-Khod, Sultanate of Oman.
Southern Medical Journal
|February 27, 2003
Summary
Sickle cell disease can cause chronic or acute colonic pseudo-obstruction. These cases highlight a rare gastrointestinal complication of sickle cell disorders, with unclear pathogenesis but potential hydroxyurea response.
Area of Science:
- Hematology
- Gastroenterology
- Genetics
Background:
- Sickle cell disease (SCD) encompasses a group of inherited red blood cell disorders.
- Gastrointestinal complications are recognized in SCD, but large-bowel pseudo-obstruction is rarely described.
- The pathogenesis of pseudo-obstruction in SCD is not well understood.
Observation:
- A young Arab woman with sickle cell-beta0-thalassemia presented with acute, then chronic, colonic pseudo-obstruction.
- The woman's condition showed partial improvement with hydroxyurea treatment.
- An Arab man with sickle cell anemia experienced acute colonic pseudo-obstruction, with complete recovery.
Findings:
- These cases represent the first documented instances of large-bowel pseudo-obstruction in sickle cell hemoglobinopathies.
- No evidence of microvascular or macrovascular occlusion was found in the woman's case.
- The pseudo-obstruction in both patients appeared to be a complication of their underlying sickle cell disease.
Implications:
- This study expands the spectrum of known gastrointestinal manifestations of sickle cell disease.
- Further research is needed to elucidate the pathogenesis of colonic pseudo-obstruction in SCD.
- Understanding this complication may lead to improved diagnostic and therapeutic strategies for affected patients.