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Mortality Rates and autopsy findings in fat embolism syndrome complicating sickle cell disease
  1. Sayna Samaee1,
  2. Sepideh Samaee1,
  3. Diana Mihalca1,
  4. Laura Fitzgerald2,
  5. Adeel Ahmed1,
  6. John Hall1,
  7. Dimitris A Tsitsikas1
  1. 1Haematology, Homerton Healthcare NHS Foundation Trust, London, UK
  2. 2Homerton Healthcare NHS Foundation Trust, London, UK
  1. Correspondence to Dr Dimitris A Tsitsikas, haematology, Homerton Healthcare NHS Foundation Trust, London, E9 6SR, UK; di.ts{at}


Fat embolism syndrome is a rare but underdiagnosed complication of sickle cell disease associated with high morbidity and mortality. It affects predominantly patients with a previously mild course of their illness and those of non-SS genotypes while there is possibly an association with infection with human parvovirus B19 (HPV B19). Here, we present the mortality rates and autopsy findings of all reported cases to date. A systematic review has revealed 99 published cases in the world literature with a mortality rate of 46%. Mortality varied greatly according to the time of reported cases with no survivors in the 1940s, 1950s or 1960s and no deaths since 2020. 35% of cases had previously undiagnosed sickle cell disease and the latter was only identified at autopsy after developing fat embolism with a fatal outcome. 20% of cases reported after 1986 tested positive for HPV B19 with an associated mortality of 63% whereas in cases that have not documented HPV B19 infection the mortality was 32%. The organs most often staining positive for fat were the kidneys, lungs, brain and heart whereas ectopic haematopoietic tissue was found in 45% of the examined lung specimens.

  • Autopsy
  • Anemia, Sickle Cell
  • Hematologic Diseases
  • Hemoglobinopathies
  • Hematology

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  • Handling editor Runjan Chetty.

  • Contributors SaS collected data and wrote the paper, SeS, LF, DM, AA and JH collected data and critically reviewed the manuscript and DAT designed the project and critically reviewed the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.