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Acute splenic sequestration in hemoglobin sickle O-Arab disease
The Johns Hopkins Medical Journal
|June 1, 1980
Summary
Sickle O-Arab disease (Hb S/O-Arab) can cause dangerous splenic sequestration in infants. Splenectomy stabilized hemoglobin levels, preventing further life-threatening complications in this case.
Area of Science:
- Hematology
- Pediatric Medicine
- Genetics
Background:
- Sickle O-Arab disease (Hb S/O-Arab) is a hemoglobinopathy.
- It shares similarities with homozygous sickle cell disease (Hb SS).
- Acute splenic sequestration is a known complication.
Observation:
- A white infant with Hb S/O-Arab experienced three episodes of acute splenic sequestration.
- Hemoglobin levels dropped significantly during these episodes.
- Radionucleotide spleen scan indicated functional hyposplenism.
Findings:
- Splenectomy led to hemoglobin stabilization between 0.7-8.5 g/dl.
- This suggests splenectomy effectively managed the sequestration crises.
Implications:
- Splenectomy may be a viable treatment for recurrent splenic sequestration in Hb S/O-Arab.
- Patients with Hb S/O-Arab and Hb SS are at risk for infections and osteomyelitis.
- Early intervention and monitoring are crucial for managing these conditions.