title: Sudden Death in Sickle Cell Anaemia: Report of Three Cases with Brief Review of Literature.
abstract: Vaso-occlusive crisis in sickle cell anaemia is one of the commonest presentations and a leading cause of death. Death can be sudden and unexpected. Herein we present three cases of sickle cell anaemia with sudden death within 3 days of hospitalisation. All the three cases presented with fever and jaundice. Two cases presented consecutively in the same year within a span of 5 months while the other case had presented 2 years prior to these two cases. Infection was the precipitating event in two cases and pregnancy with infection in one. One case in addition had 'right upper quadrant syndrome' and one case had 'acute chest syndrome' (ACS) due to bone marrow fat embolism. Postmortem liver biopsy of all the three cases showed dilated and congested sinusoids with sickled RBCs, kupfer cell prominence with erythrophagocytosis. Lung biopsy of case with ACS showed vessels occluded with bone marrow elements indicating bone marrow fat embolism.
authors.affiliation: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 605006 India.
authors.forename: Manickam
authors.initials: M
authors.lastname: Niraimathi
authors.affiliation: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 605006 India.
authors.forename: Rakhee
authors.initials: R
authors.lastname: Kar
authors.affiliation: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 605006 India.
authors.forename: Sajini Elizabeth
authors.initials: SE
authors.lastname: Jacob
authors.affiliation: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 605006 India.
authors.forename: Debdatta
authors.initials: D
authors.lastname: Basu
keywords: Bone marrow fat embolism; Sickle cell anaemia; Sudden death; Vaso-occlusive crisis