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Neurologic events after partial exchange transfusion for priapism in sickle cell disease
W R Rackoff1, K Ohene-Frempong, S Month
1Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia.
Insights
Partial exchange transfusion for priapism in boys with sickle cell disease can lead to severe neurological issues. Promptly investigate neurological symptoms like severe headache in these patients.
Area of Science:
- Neurology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Priapism is a prolonged erection, often treated with exchange transfusion in SCD patients.
- Neurological complications following treatment are a concern.
Observation:
- Six boys (7-13 years) with homozygous SCD developed acute neurological abnormalities post-exchange transfusion for priapism.
- Symptoms included severe headache, seizures, and focal deficits, with some requiring intubation.
- Cerebral arteriography revealed vascular abnormalities like stenosis and occlusion.
Findings:
- Neurological complications arose 1-11 days after partial exchange transfusion.
- Pre-complication hemoglobin S levels ranged from 18% to 33%.
- Regular erythrocyte transfusions prevented recurrence when hemoglobin S was maintained at 30%-50%.
Implications:
- A potential causal link exists between partial exchange transfusion and neurological events in SCD.
- Early, thorough neurological evaluation, especially for severe headache, is crucial.
- Management strategies may need adjustment to mitigate these risks.
Abstract:
We describe six boys with homozygous sickle cell disease, aged 7 to 13 years, in whom acute, severe neurologic abnormalities developed 1 to 11 days after partial exchange transfusion was performed to treat priapism that was unresponsive to more conservative therapy. Hemoglobin levels were 10.5 to 13.4 gm/dl (mean 12.1 gm/dl), and hemoglobin S levels were 18% to 33% (mean 27%) before the onset of neurologic complications. Severe headache was the initial finding in five patients, four of whom had increased intracranial pressure and three of whom required tracheal intubation and hyperventilation. Four patients had seizures; three had focal neurologic deficits for more than 24 hours. Cerebral arteriography demonstrated vascular abnormalities, including irregularity, stenosis, and complete occlusion of vessels. Patients treated with regular erythrocyte transfusions had no recurrence of neurologic signs or symptoms when hemoglobin S levels were kept at 30% to 50%. The occurrence of serious neurologic complications after partial exchange transfusion in patients with homozygous sickle cell disease from three centers indicates the possibility of a causal relationship between the events. Early and thorough investigation of neurologic symptoms, especially severe headache, is warranted in this clinical setting.