Your browser doesn't support javascript.
loading
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.
Tsitsikas, Dimitris A; Gallinella, Giorgio; Patel, Sneha; Seligman, Henry; Greaves, Paul; Amos, Roger J.
Afiliación
  • Tsitsikas DA; Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK. Electronic address: dimitris.tsitsikas@homerton.nhs.uk.
  • Gallinella G; Department of Pharmacy and Biotechnology, University of Bologna, S. Orsola-Malpighi Hospital - Microbiology, Bologna, Italy.
  • Patel S; Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
  • Seligman H; Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
  • Greaves P; Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
  • Amos RJ; Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Blood Rev ; 28(1): 23-30, 2014 Jan.
Article en En | MEDLINE | ID: mdl-24468004
ABSTRACT
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.
Asunto(s)
Palabras clave

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Médula Ósea / Embolia Grasa / Anemia de Células Falciformes Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies / Systematic_reviews Límite: Adolescent / Adult / Child / Female / Humans / Male / Middle aged Idioma: En Revista: Blood Rev Asunto de la revista: HEMATOLOGIA Año: 2014 Tipo del documento: Article

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Médula Ósea / Embolia Grasa / Anemia de Células Falciformes Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies / Systematic_reviews Límite: Adolescent / Adult / Child / Female / Humans / Male / Middle aged Idioma: En Revista: Blood Rev Asunto de la revista: HEMATOLOGIA Año: 2014 Tipo del documento: Article
...