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Fat embolism syndrome associated with asthma and sickle cell‐β + ‐thalassemia
Author(s) -
Johnson K.,
Stastny J. F.,
Rucknagel D. L.
Publication year - 1994
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830460418
Subject(s) - medicine , fat embolism , acute chest syndrome , hemoglobinopathy , thalassemia , sickle cell anemia , asthma , autopsy , pediatrics , pulmonary embolism , embolism , hemolytic anemia , gastroenterology , surgery , disease
Abstract A 25‐year‐old African‐American man with sickle cell‐β + ‐thalassemia 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 developed pain similar to that of sickle cell vasooclusion, for which he received small amounts of analgesics. He died approximately 84 hours after 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 significant sickle cell hemoglobinopathies. The relationship between these etiologic factors and indications for transfusions are discussed. © 1994 Wiley‐Liss, Inc.