Features of the ASPEN Syndrome -
A
ssociation of:
(1)
S
ickle cell disease
(2)
P
riapism, usually major
(3)
E
xchange transfusion
(4)
N
eurological event(s), including headache, lethargy, and seizures. In severe cases obtundation requiring ventilatory support may occur. The neurological syndrome may be delayed several days after the transfusions.
Possible explanations:
(1) Abrupt cerebral hypoxemia associated with a too-rapid exchange rate, sudden increase in hemoglobin, decrease in cerebral blood volume and decreased cerebral blood flow.
(2) Release of vasoactive substances, cytokines, etc from the penile blood vessels into the general circulation.
Management:
(1) The exchange transfusion should be performed gradually and with close observation.
(2) Seizures should be treated using standard therapy.
(3) Imaging studies may be needed to exclude stroke.
(4) Increased intracranial pressure needs to be identified and treated aggressively.
The differential diagnosis includes an acute neurological event unrelated to the exchange transfusion.
