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formation. These thrombi are composed of
Occlusion of the pulmonary vessels by emboli results in hypoxemia, sickling of red blood cells and further vaso-occlusion. This results in formation and release of more fat emboli.1
In addition to the lungs and kidneys, patients also usually have neurological involvement. Classically, petechial hemorrhages are seen throughout the cerebral white matter. The presence
of intravascular fat in the intracranial vessels is diagnostic for FES. MRI is highly sensitive for detecting cerebral involvement. Microinfarcts are typically seen, with cerebral edema thought to be secondary to toxicity from free fatty acids and subsequent breakdown of the blood brain barrier.8
Fat Emboli Syndrome has a high mortality rate, which has fortunately decreased
REFERENCES
1. Tsitsikas, Dimitris A., et al. “Bone marrow necrosis and fat embolism syndrome in sickle cell disease: Increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection.” Blood Reviews 28.1 (2014): 23-30.
2. L.J. Wade, L.D. Stevenson. Necrosis of the bone marrow with fat embolism in sickle cell anemia. Am J Pathol, 17 (1941), pp. 47–54.
3. N.C. Dang, C. Johnson, M. Eslami-Farsani, L.J. Haywood. Bone marrow embolism in sickle cell disease: a review. Am J Hematol, 79 (2005), pp. 61–67.
with the use of exchange transfusions.
At present, exchange transfusion is the fat emboli syndrome is suspected6 and should be initiated as soon as possible. Other treatment modalities have also been proposed, including high dose corticosteroids, use of dextrans, heparin, hypertonic dextrose, aspirin, and IV ethanol. However, studies have not clearly shown any of these treatments to be 9
In conclusion, fat emboli syndrome
is a rare complication in patients with hemoglobinopathies, more commonly
in those with milder forms, such as hemoglobin SC. The increased incidence in these patients has been attributed to higher blood viscosity. It occurs due to necrosis
of the bone marrow. Patients present with pain and quickly develop respiratory and neurological complications. Other organ systems are also affected, including the
diagnose and thus, is often only noted
at the time of autopsy. It is often fatal, although mortality has decreased over time, thanks to exchange transfusions. It
is therefore important to have a high index of suspicion for FES in a patient with a history of sickle cell disease or hemoglobin SC disease that presents with pain and develops hypoxia and/or confusion; and to institute exchange transfusion as soon as possible.
■ ISHA MISRA, MD is an Internal Medicine Resident at Christiana Care Health System in Newark, Del.
■ JUSTIN GLASGOW, MD, PhD is an Internal Medicine Physician and Hospitalist at Christiana Care Health System in Newark, Del.
■ FARID MOOSAVY, MD is a Pulmonologist and Critical Care Physician at Christiana Care Health System in Newark, Del.
risk patients. Ann Intern Med. 1983;99:438- 443.
8. Early Diagnosis of Cerebral Fat Embolism Syndrome by Diffusion-Weighted MRI (Starfield Pattern) P.M. Parizel, H.E. Demey, G. Veeckmans, F. Verstreken, P. Cras, P.G. Jorens and A.M. De Schepper. Stroke. 2001;32:2942-2944, originally published December 1, 2001.
9. Graff, Danielle M., et al. “Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection.” Case reports in hematology 2015 (2015).
CONTRIBUTING AUTHORS
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5.
E.A. Manci, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol, 123 (2003), pp. 359–365.
J.K. Graham, M. Mosunjac, R.L. Hanzlick,
M. Mosunjac. Sickle cell lung disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review. Am J Forensic Med Pathol, 28 (2007), pp. 168–172.
6 Gurd AR, Wilson RI. The fat embolism syndrome. J Bone Joint Surg Br. 1974;56B:408-416.
7. Schonfeld SA, Ploysongsang Y, DiLisio
R, et al. Fat embolism prophylaxis with corticosteroids. A prospective study in high-
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