title: Moyamoya disease associated with hemoglobin Fairfax and beta-thalassemia.
abstract: Moyamoya disease is a rare cerebrovascular condition characterized by steno-occlusive disease of the major intracranial arteries at the base of the brain, and the appearance of innumerable, tiny, arterial collaterals that resemble a "puff of smoke." Although it has been associated with hematologic diseases such as sickle-cell disease, the association with other hemoglobinopathies is less frequently observed. We describe the association of a unique hemoglobinopathy (hemoglobin Fairfax) with beta-thalassemia and moyamoya disease in a 9-year-old girl with a history of stroke. To our knowledge, this is the first report of this unstable hemoglobin with moyamoya disease, and it emphasizes the potential for cerebral infarction due to the severe anemia of hemolytic disease.
authors.affiliation: Department of Radiology, Inova Fairfax Hospital, Falls Church, Virginia, USA. mardenf@alexian.net
authors.forename: Franklin A
authors.initials: FA
authors.lastname: Marden
authors.forename: Christopher M
authors.initials: CM
authors.lastname: Putman
authors.forename: John M
authors.initials: JM
authors.lastname: Grant
authors.forename: Jay
authors.initials: J
authors.lastname: Greenberg
mesh_terms: D000745:Anemia, Hemolytic, Congenital / Q000209:etiology; D000792:Angiography; D002545:Brain Ischemia / Q000209:etiology; D002339:Carotid Arteries / Q000473:pathology; D002648:Child; D005260:Female; D006453:Hemoglobinopathies / Q000097:blood / Q000150:complications* / Q000473:pathology; D006455:Hemoglobins, Abnormal / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009072:Moyamoya Disease / Q000097:blood / Q000150:complications* / Q000473:pathology; D020521:Stroke / Q000209:etiology; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000473:pathology
chemical_list: D006455:Hemoglobins, Abnormal