ASSOCIATION OF SICKLE-CELL DISEASE, PRIAPISM, EXCHANGE-TRANSFUSION AND NEUROLOGICAL EVENTS - ASPEN SYNDROME

Priapism and acute neurological events are believed to be unrelated co mplications of sickle cell hemoglobinopathy. We describe a syndrome ba sed on our experience and a review of the literature of significant ne urological events after partial exchange transfusion to treat priapism in sicklemic patients. Severe headache is often the initiating sympto m of this complex. The ensuing neurological events range from seizure activity to obtundation requiring ventilatory support. The proposed pa thophysiology of these neurological events is related to cerebral isch emia after an acute increase in per cent total hemoglobin, concomitant decrease in per cent hemoglobin S and subsequent release of vasoactiv e substances during penile detumescence. We have termed this constella tion of events the ASPEN syndrome, an eponym for association of sickle cell disease, priapism, exchange transfusion and neurological events. Early recognition and aggressive medical management resulted in compl ete reversal of neurological sequela.