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.
Fat embolism syndrome (FES) due to extensive bone marrow necrosis (BMN) in sickle cell disease (SCD) is a potentially under-diagnosed complication associated with severe morbidity and mortality. We identified 58 cases reported in the world literature to date. Typically, patients presented with a seemingly uncomplicated vaso-occlusive crisis (VOC) and subsequently deteriorated rapidly with a drop in their haemoglobin and platelets, development of respiratory failure, encephalopathy and varying degrees of involvement of other systems. Overall mortality in the reported cases was 64% but differed according to the use of transfusion and was 29%, 61% and 91% for patients receiving exchange, top-up or no transfusion respectively. Patients most at risk appear to be those with a "milder" form of SCD as 81% of patients had a genotype other than HbSS and the majority had no history of significant sickle-related complications. Human parvovirus B19 (HPV B19) infection was documented in 24% of cases.
Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK. Electronic address: dimitris.tsitsikas@homerton.nhs.uk.
Dimitris A
DA
Tsitsikas
Department of Pharmacy and Biotechnology, University of Bologna, S. Orsola-Malpighi Hospital - Microbiology, Bologna, Italy.
Giorgio
G
Gallinella
Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Sneha
S
Patel
Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Henry
H
Seligman
Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Paul
P
Greaves
Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Roger J
RJ
Amos
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics / Q000821:virology; D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D004620:Embolism, Fat / Q000209:etiology* / Q000401:mortality; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010322:Parvoviridae Infections / Q000150:complications; D016732:Parvovirus B19, Human; D063868:Patient Outcome Assessment; D012306:Risk; D055815:Young Adult
Bone marrow necrosis; Fat embolism; Parvovirus B19; Sickle cell