Sickle cell trait (SCT) is estimated to occur in 6% to 10% of Africans, and the United Kingdom (UK) prevalence rate has been estimated at 3.2%. Although sudden death in sickle cell disease is well known, its occurrence in SCT is rare and requires extremes of physiological stress.
We present a case of a 29-year-old black woman who died suddenly during a period of religious fasting. Her medical history was unremarkable, and there was no family history of sickle cell disease. At postmortem, she was found to be dehydrated, and macroscopically, the main abnormal findings were congested lungs and a small spleen. Histological examination revealed extensive vascular congestion with red blood cell sickling in both lungs, the liver, and the spleen. Electrophoresis on a postmortem blood sample confirmed the clinical suspicion that the patient was a carrier of SCT.
The case highlights a novel scenario of SCT associated sudden death. We discuss the potential pathophysiological mechanisms that may have led to the patient's demise. We also remind pathologists to consider this diagnosis as potentially contributing to the cause of death in apparently fit young people of ethnic origin during episodes of physiological stress.
From the Department of Histopathology, The Royal London Hospital, Whitechapel, London, United Kingdom.
Manuscript received June 21, 2007; accepted November 15, 2007.
Reprints: Olaf J. Biedrzycki, BSc, MBBS, MRCPath, DMJ(Path), MFFLM, Department of Histopathology, The Royal London Hospital, Whitechapel, London E1 1BB, United Kingdom. E-mail: firstname.lastname@example.org.