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Severe thrombocytopenia in sickle cell crisis
U Allen1, H MacKinnon, A Zipursky
1Division of Hematology, Hospital for Sick Children, Toronto, Ontario, Canada.
Pediatric Hematology and Oncology
|January 1, 1988
Summary
Thrombocytopenia is rare in sickle cell disease. A child with sickle cell disease experienced severe low platelets during a vasoocclusive crisis, suggesting platelet consumption in sickle cell complications.
Area of Science:
- Hematology
- Pediatrics
- Vascular Biology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- Thrombocytopenia, or low platelet count, is uncommon in SCD patients.
- Vasoocclusive episodes are a hallmark of SCD, causing severe pain and organ damage.
Observation:
- A pediatric patient with homozygous SCD presented with life-threatening thrombocytopenia.
- The patient experienced a prolonged and debilitating vasoocclusive crisis.
- Notably, irreversibly sickled cells were absent in peripheral blood during the illness.
Findings:
- The absence of irreversibly sickled cells suggests their sequestration or consumption.
- The severe thrombocytopenia indicates significant platelet utilization.
- These findings suggest a mechanism of platelet and irreversibly sickled cell consumption at infarction sites during vasoocclusive crisis.
Implications:
- This case highlights a potential, albeit unusual, complication of SCD.
- Understanding platelet consumption in SCD may lead to new therapeutic targets.
- Further research is needed to elucidate the role of platelets in SCD pathophysiology.