Cerebral Fat Embolism due to Sickle Cell Crisis with Concurrent Parvovirus B-19 Infection
Anam Farrukh 1, Maryam Haq2, Toluwanimi Shaanu2, Hamza Ahmed2, Ahmad Al-Awwad2
1Independent, 2University of Oklahoma
Objective:
To describe the case of a 22-year-old man with heterozygous sickle cell disease who developed cerebral fat embolism from a sickle cell crisis with concurrent Parvovirus B-19 infection.
Background:
Cerebral fat embolism is known to occur most commonly after long bone fractures, but it can also be caused by sickle cell crisis. It paradoxically occurs mainly in heterozygous sickle cell disease, with concurrent Parvovirus B-19 infection being a further risk factor. This is postulated to be due to heterozygous mutations that allow for a semi functioning spleen causing inflammation which results in the formation and release of fat globules into the circulation.
Design/Methods:
Case report and review of literature.
Results:
22-year-old man with heterozygous sickle cell disease (HgbSC mutation) presented in a sickle cell crisis manifested by neurological deterioration and respiratory failure with a Glasgow Coma Scale (GCS) of 8 (E2, V1T, M5). He received an emergent red blood cell exchange transfusion on arrival. MRI brain showed numerous scattered punctate areas of diffusion restriction in the bilateral cerebral cortex, deep white matter, and bilateral basal ganglia with associated micro hemorrhaging and T2/FLAIR hyperintensities, concerning for cerebral fat embolism. Further workup showed a profoundly elevated serum ferritin as well as positive Parvovirus B-19 DNA PCR. Patient received supportive care in the intensive care unit for two weeks after which he was transferred to the floor where he remained for another week before being discharged home in good condition.
Conclusions:
Parvovirus B-19 infection increases the risk of developing cerebral fat embolism in patients with heterozygous sickle cell disease who present in a sickle cell crisis.