Agranulocytosis with deferiprone treatment of superficial siderosis.
Superficial siderosis of the central nervous system is a rare neurological disorder caused by deposits of haemosiderin on subplial brain matter. Characterised by a thin dark layer surrounding the brain stem, cerebellum and cortical fissures on the T2-weighted MRI, symptoms include sensorineural hearing loss and progressive gait ataxia. A specific aetiology for the blood in the subarachnoid space is identified in less than 50% of cases. While identification of a specific vascular defect allows for vascular repair, treatment options are limited for idiopathic superficial siderosis. Recently, a pilot safety study demonstrated promising results using an iron chelator, deferiprone. While this approach is promising, we present a potential serious complication of this therapy-the first report of agranulocytosis in the treatment of superficial siderosis following deferiprone therapy.
Internal Medicine Department, Madigan Army Medical Center, Tacoma, Washington, USA. nikhil.a.huprikar.mil@mail.mil
Nikhil
N
Huprikar
Marisa
M
Gossweiler
Maureen
M
Callaghan
Paul
P
Bunge
D000368:Aged; D000380:Agranulocytosis / Q000139:chemically induced*; D000077543:Deferiprone; D006801:Humans; D007502:Iron Chelating Agents / Q000009:adverse effects* / Q000627:therapeutic use; D008297:Male; D011728:Pyridones / Q000009:adverse effects* / Q000627:therapeutic use; D012806:Siderosis / Q000188:drug therapy*
D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone