A Suspected Case of Cerebral Fat Embolism Triggering a Drug-resistant Status Epilepticus in a HbS/β⁺-Thalassaemia Patient

Main Article Content

Marta Bortolotti
Gianluca Costamagna
Marta Mancarella
Delia Gagliardi
Silvia Lanfranconi
Alessia Marcon
Margherita Migone De Amicis
Nereo Bresolin
Stefania Corti
Giovanna Graziadei

Keywords

sickle cell disease, cerebral fat embolism, status epilepticus, eritroexchange

Abstract

Sickle cell disease (SCD) refers to a group of hereditary disorders associated with clinical manifestations of variable severity. C In particular, Fat Embolism Syndrome, a rare and devastating complication of SCD, may preferentially involve non-homozygous patients and patients with mild disease. 

Downloads

Download data is not yet available.
Abstract 169 | PDF Downloads 41 HTML Downloads 5

References

1. Tsitsikas DA, Gallinella G, Patel S, Seligman H, Graves P, Amos RJ. 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 Rev. 2014;28:23–30.
2. Dang NC, Johnson C, Eslami‐Farsani M, Haywood LJ. Bone marrow embolism in sickle cell disease: A review. Am J Hematol. 2005;79:61–67.
3. Tsitsikas DA, May JE, Gangaraju R, Abukar J, Amos RJ, Marques MB. Revisiting fat embolism in sickle syndromes: diagnostic and emergency therapeutic measures. Br J Haematol. 2019;186:e112–e115.
4. Kammeyer R, Devnani R, Mehta R. Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease. Am J Hematol. 2016;91:539–542.
5. Bailey K, Wesley J, Adeyinka A, Pierre L. Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review. J Intensive Care Med. 2019;34:797–804.
6. Gangaraju R, May JE, Williams Ill LA, Reddy VB, MacLennan P, Marques MB. Fat embolism syndrome due to bone marrow necrosis in patients with hemoglobinopathies: A life-threatening complication mimicking thrombotic thrombocytopenic purpura. Am J Hematol. 2019;94:e64–e66.
7. Kuo KH, Pan YJ, Lai YJ, Cheung WK, Chang FC, Jarosz J. Dynamic MR Imaging Patterns of Cerebral Fat Embolism: A Systematic Review with Illustrative Cases. Am J Neuroradiol. 2014;35:1052–1057.
8. Medina FJ, Marquez JC, Castillo M. Cerebral fat embolism detection with susceptibility-weighted images in sickle cell disease. Neuroradiol J. 2012;25:411–414.
9. Mijalski C, Lovett A, Mahajan R, Sundararajan S, Silverman S, Feske S. Cerebral Fat Embolism. Stroke. 2015;46:e251–e253.
10. Onteddu S, Hinduja A, Shihabuddin B. Sickle Cell Crisis Presenting as Convulsive Status Epilepticus. Neurology. 2012;78:P01.066.
11. Aguirre-Bermeo H, Millón J, Martínez-Domeño A, Marruecos-Sant L. Status epilepticus in a patient with sickle cell disease. Med Clin. 2011;137:428–429.
12. Alvarez V, Drislane FW. Is Favorable Outcome Possible After Prolonged Refractory Status Epilepticus? J Clin Neurophysiol. 2016;33:32–41.
13. Hermann B, Brisson H, Langeron O, Pyatigorskaya N, Paquereau J, Robert H, Stender J, Habert M, Naccache L, Monsel A. Unexpected good outcome in severe cerebral fat embolism syndrome. Ann Clin Transl Neurol. 2018;5:988–995.