A patient with a sickling hemoglobinopathy may develop a fat embolism syndrome (FES). The diagnosis may be delayed if the diagnosis is not considered.
Fat embolism may occur in:
(1) sickle cell disease
(2) sickle cell beta thalassemia
(3) hemoglobin SC
(1) long bone fractures (unrelated to sickling hemoglobinopathy)
(2) avascular necrosis
(3) parvovirus B19 infection
(4) bone marrow necrosis in sickle cell crisis
Clinical features may include:
(3) general body aches
(4) encephalopathy (cerebral fat embolism)
(5) embolic skin lesions
(7) "starfield" pattern on MRI
(8) variable pulmonary hypertension
Fat embolism may be a cause of acute chest syndrome.
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Specialty: Hematology Oncology