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Mild splenic sequestration crises in sickle-hemoglobin C disease.
Clinical Pediatrics
|June 1, 1984
Summary
Acute splenic sequestration crisis (ASSC) can occur in sickle-hemoglobin C disease, even without high altitude. These events may be milder than in sickle cell anemia.
Area of Science:
- Hematology
- Pediatrics
- Genetics
Background:
- Acute splenic sequestration crisis (ASSC) is a known complication of sickle cell anemia.
- ASSC is infrequently reported in sickle-hemoglobin C (SC) disease, typically associated with high altitude exposure.
Observation:
- This report details three pediatric cases of ASSC in sickle-hemoglobin C disease.
- The observed ASSC episodes were generally milder compared to those in sickle cell anemia.
Findings:
- ASSC can occur in sickle-hemoglobin C disease without hypoxic triggers like high altitude.
- Potential predisposing factors identified include mononucleosis syndrome and hypovolemic shock due to epistaxis.
Implications:
- Clinicians should consider ASSC in children with sickle-hemoglobin C disease, even in the absence of typical triggers.
- Recognizing milder forms of ASSC in SC disease is crucial for timely diagnosis and management.