FAT-EMBOLISM SYNDROME-ASSOCIATED WITH ASTHMA AND SICKLE CELL-BETA(-THALASSEMIA())

A 25-year-old African-American man with sickle cell-beta(+)-thalassemi a presented with acute asthma of 2 days' duration. The asthma was well controlled by 48 hr, and parenteral medications were changed to oral bronchodilators and steroids. Sixty hours after presentation, he devel oped pain similar to that of sickle cell vasooclusion, for which he re ceived small amounts of analgesics. He died approximately 84 hours aft er presentation. Autopsy showed extensive marrow necrosis and massive fat embolism. This is the first reported case of fat embolism syndrome associated with this genotype, the mildest of the clinically signific ant sickle cell hemoglobinopathies. The relationship between these eti ologic factors and indications for transfusions are discussed. (C) 199 4 Wiley-Liss, Inc.