Death due to sickle cell anaemia, an autopsy diagnosis: a study at a tertiary care hospital
Keywords:
Sickle cell crisis, Sudden and unexpected deaths, Autopsy studyAbstract
Background: Sickle cell disease (SCD) is the generic term for the group of inherited haemoglobinopathies caused by the occurrence of Haemoglobin S (HbS) in the homozygous or heterozygous form in combination of Hbs with another abnormal haemoglobin such as HbSC or beta-thalassaemias (HbS-thal). Sickle cell syndromes are remarkable for their clinical heterogeneity, including their presentations as sudden and unexpected deaths due to a sickle cell crisis. Less numbers of deaths are reported due to this cause because of ignorance of autopsy surgeon in considering this disease as a cause of death despite of its high prevalence. While doing autopsy in cases of deaths with no apparent cause and physical over activity medical officer must keep in mind the possibility of death due to vaso-occlusive crisis in sickle cell disease.
Methods: The study covers a period of one year (January 2013 - December 2013) and it is a study of cases of autopsy carried out in a tertiary care hospital of South Gujarat.
Results: A total of 607 cases examined, out of which sickled red blood cells were detected in 17 cases. The respective records were reviewed. Out of 17 cases, 13 cases were male and 4 cases were females. The youngest person was 15 years female and oldest was 70 years male.
Conclusion: Sickle cell crisis is one of the causes of sudden unexplained deaths. The present study highlights the role of autopsy in such cases. Community awareness and marriage counseling programs are also helpful in preventing sickle cell disease.
References
Pauling L. Sickle cell anemia: a molecular disease. Science. 1949;110:543.
Kamble M, Chaturvedi P. Epidemiology of sickle cell disease in a rural hospital of central India. Indian Paediatr. 2000;37:391-6.
Davies SC, Brozoic M. The presentation, management and prophylaxis of sickle cell disease. Blood Rev. 1989;3:29-44.
Beutler E. The sickle cell disease and related disorders. In: Beutler E, Williams WJ, eds. William’s Hematology, 5th ed. New York: McGraw Hill; 1995: 616-665.
Balgir RS. Hematological profile of twenty five tribal compound cases of haemoglobinopathies and G6PD deficiency in rural Orissa. Indian J Med Sci. 2008;62(9):362-71.
Government of Gujarat. Sickle cell disease control and research project, Gujarat. In: Government of Gujarat, eds. A Project. Gujarat, India: Commisionarate of tribal development and Commisionarate of Health and Family Welfare; December 2007.
Lehmann H, Cutbush M. Sickle cell trait in southern India. Br Med J. 1952;1:404-5.
Shukla RN, Solanki BR, Parande AS. Sickle cell disease in India. Blood. 1958;13:552-8.
Italia Y. A sickle cell disease book for health workers and sickle cell disease control programs. In: Italia Y, eds. A Book. Gujarat, India: Commisionarate of Health and Family welfare. Government of Gujarat; 2006: 1-3.
Frenette PS, Atweh GF. Sickle cell disease: old discoveries, new concepts, and future promise. J Clin Invest. 2007;117:850-8.
Mitchell BL. Sickle cell trait and sudden death - bringing it home. J Natl Med Assoc. 2007;99(3):300-5.
Kate SL. Health problems of the tribal population groups from the state of Maharashtra. Indian J Med Sci. 2001;55(2):99-108.
Manci EA, Culberson DE, Yang YM, Gardner TM, Povell R, Haynes J Jr, et al. Causes of death in Sickle cell disease: an autopsy study. Br J Haematol. 2003;123(2):359-65.
Lalitha V. Pillai, Saiffuddin Hussaini, Sameer Gosavi, Narendra Vaidya. Sudden unexpected death in an undiagnosed sickle disease. Indian J Crit Care Med. 2005;9(2):92-5.
Orah S. Platt. The acute chest syndrome of sickle cell disease. N Engl J Med. 2000;34(25):1904-7.
Ernest Beulter. Disorders of haemoglobin. In: Kurt J. Isselbacher, Jean D. Wilson, Joseph B. Martin, Dennis Kasper, Stephen L. Hauser, Anthony S. Fauci, et al., eds. Harrison’s Principles of Internal Medicine. 14th ed. New York: McGraw Hill; 1998: 645-649.
Manic EA, Culberson DE, Yang YM, Gardner TM, Powell R, Haynes J Jr, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003;123(2):359-65.
Orah S. Platt, Donald J. Brambilla. Mortality in sickle cell disease - life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-44.
Gilbert-Barness E. Sudden and unexplained death in infants. In: Enid Gilbert-Barness, eds. Potters Pathology of the Fetus, Infant and Child. 2nd ed. USA: Mosby Elsevier; 2007: 841-847.