Autopsy findings and pattern of mortality in undiagnosed sickle cell disease patients


  • Bhavna Gamit Department of Pathology, Government Medical College, Surat, Gujarat
  • Amita Patel Department of Pathology, Government Medical College, Surat, Gujarat
  • Pinal Shah Department of Pathology, Government Medical College, Surat, Gujarat
  • Suresh Padsala Department of Pathology, Government Medical College, Surat, Gujarat



Sickle cell disease, Sudden and unexpected death, Vaso-occlusive crisis, Autopsy


Background: Sickle cell disease (SCD) has a high mortality rate with unexpected sudden death. So, the purpose of the study was to analyze clinical and/or autopsy findings at the time of death among sickle cell disease (SCD) patients.

Methods: This is a retrospective study of SCD patients who died between January 2011 to December 2011 and the morphological evidence of the cause of death was studied in a tertiary care hospital of south Gujarat. The clinical data, including the age, gender, symptoms and the major autopsy findings and cause of death were obtained for each patient that included histopathological examination.

Results: A total of 472 autopsies were performed, out of which sickled erythrocytes were detected in 40 cases. The mean age at death was 30 years and a male/female ratio of 3:1 and peak mortality was in the 2nd and 3rd decades of life. The common causes of death in this study include vaso-occlusive crisis (45%), infection (40%). The terminal infection was heralded by upper respiratory tract (43.7%) and by gastroenteritis (31.2%). Other causes of death included intracranial haemorrhage (2.5%) and cirrhosis (2.5%). Among the cases of SCD, 10% of deaths were non-haemoglobinopathy related such as fall (accidental), organophosphorus poisoning and snake bite.

Conclusion: This study is to precise analysis of causes of death is needed to focus and improve morbidity and mortality in sickle cell disease especially in highly prevalent area and will impact on the overall survival of these patients.


Stuart MJ, Nagel RL. Sickle cell disease. Lancet. 2004;364:1343-60.

Rees DC, Williams TM, Gladwin MT. Sickle cell disease. Lancet. 2010;376:2018-31.

Kate SL. Health Problems of Tribal Population groups from the State of Maharashtra. Indian Journal of Medical Sciences. 2001;55:99-108.

Shukla RN, Solanki BR, Parande AS. Sickle Cell disease in India. Blood. 1958;13:552-8.

Lehman H, Cutbush M. Sickle cell trait in southern India. Brit Med J. 1952;1:404-5.

Bhatia HM, Rao VR. Genetic atlas of Indian Tribes, Bombay: Institute of Immunohaematology (ICMR); 1987.

Colah RB, Mukherjee MB, Martin S, Ghosh K. Sickle cell disease in tribal populations in India. Indian J Med Res. 2015;141:509-15.

Sears DA. Sickle Cell trait. In: Embury SH, Hebbel RP, Mohandas N, Steinberg MH, eds. Sickle cell disease: basic principles and clinical practice. New York, Raven Press. 1994:381-94.

National health mission, state health society, Health and family welfare department, government of Gujarat.

Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MHm et al. Mortality in sickle cell disease: Life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-44.

Darbari DS, Kple-Faget P, Kwangyan J, Rana S, Castro O. Circumstances of death in adult sickle cell disease patients. Am J of Haematol. 2006;81(11): 858-63.

Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. Br Med J. 1982;285(6342):633-5.

Perronne V, Roberts-Harewood M, Bachir D, Roudot-Thoraval F, Delord J-M, Thuret I, et al. Pattern of mortality in sickle cell disease in adults in France and England. The Haematology Journal. 2002;3(1):56-60.

Liesner RJ, Vandenberghe EA. Sudden death in sickle cell disease. Journal of the Royal Society of Medicine. 1993;86(8):484-5.

Manci 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.

Ogun GO, Ebili H, Kotila TR. Autopsy findings and pattern of mortality in Nigerian sickle cell disease patients. The Pan African Medical Journal. 2014;18:30.

Gray A, Anionwu EN. Patterns of mortality in sickle cell disease in United Kingdom. Journal of clinical pathol. 1991;44:459-63.

Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011;87(3):191-207.

Minter KR, Gladwin MT. Pulmonary Complications of Sickle Cell Anemia. American Journal of Respiratory and Critical Care Medicine. 2001;164(11):2016-19.

Pillai LV, Husainy S, Gosavi S, Vaidya N. Sudden unexpected death in an undiagnosed sickle disease. Indian J Crit Care Med. 2005;9:92-5.

Gladwin MT, Sachdev V. Cardiovascular abmormalities in sickle cell disease. J Am Coll Cardiol. 2012;59:1123-33.

Al-Salem AH, Qaisaruddin S, Al-Dabbous I, Bhamidipati P, Abu Srair H, Amman H et al. Cholelithiasis in children with sickle cell disease. Pediatr Surg Int. 1996;11:471-3.

Bond LR, Hatty SR, Horn ME, Dick M, Meire HB, Bellingham AJ. Gall stones in sickle cell disease in the United Kingdom. Br Med J (Clin Res Ed). 1987;295(6592):234- 6.

Carlos T, Escoffery DM, Suzanne ES. Autopsy findings in sickle cell disease. Jusksonville med. 2000;6:1-7.

Hussain I, Ahmed H. Hepatobiliary Manifestations of Sickle Cell Anemia. Gastroenterol Res and Elmer Press. 2010;3(1):1-8

Martin N, Nwabuko CO, Chinenye N, Emeka P. Comparative Study of autopsy findings in sickle cell disease (SCD) At South Eastern tertiary hospital, Nigeria. IOSR Journal of Dental and Medical Sciences. 2013;5(3):59-62.

Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK et al. Surgery and Anesthesia in Sickle Cell Disease. Blood. 1995;86(10):3676-84.

Firth PG, Alvin C. Sickle Cell Disease and Anesthesia. Anesthesiology. 2004;101(3):766-85.

Patel DK, Patel S, Mashon RS, Dash PM, Mukherjee MB. Diverse phenotypic expression of sickle cell hemoglobin C disease in an Indian family. Ann Hematol. 2011;90(3):357-8.

Behrens RJ, Cymet TC. Sickle cell disorder: Evaluation, treatment, and natural history. Hospital physician. 2000:17-28.

Yadav R, Gupta RB, Bharadwarj VK, Singh MPSS. Morbidity profile of sickle cell disease in central India. Proceeding of National Symposium on Tribal Health. 2006.

Gupta RB. Sickle cell disease load in Madhya Pradesh. 18. RMRCT Update. Newslett Regional Med Res Centre Tribals Jabalpur. 2006;3:1-6.

Kar BC, Satapathy RK, Kulozik AE, Kulozik M, Sirr S, Serjeant BE, et al. Sickle cell disease in Orissa State, India. Lancet. 1986;22;2(8517):1198-201.

El-Mouzan MI, Al-Awamy BH, Al-Torki MT, Niazi GA. Variability of sickle cell disease in the eastern province of Saudi Arabia. J Pediatr. 1989;114(6):973-6.

Al-Awamy BH, Niazi GA, El-Mauzan ML, Altorki MT, Naeem MA. Relationship of hemoglobin F and a-thalassemia to severity of sickle cell anemia in the eastern province of Saudi Arabia. Ann Trop Pediatr. 1968;6:251-65.

Serjeant GR. Sickle cell disease. Lancet. 1997;350:725-30.

Chopra R, Al-Mulhim AR, Al-Baharani AL. Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia. American Journal of Hematology. 2005;79(3):180-6.

Guazzi M, ABorlaug B, Pulmonary Hypertension Due to Left Heart Disease Circulation. 2012;126:975-90.

Graham JK, Mosunjac M, Hanzlick RL, Mosunjac M. Sickle cell lung disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review. Am J Forensic Med Pathol. 2007;28(2):168-72.

Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008;359:2254.

Siddiqui AK, Ahmed S. Pulmonary manifestations of sickle cell disease. Postgrad Med J. 2003;79:384.

Powars D, Weidman JA, Odom-Maryon T, Niland JC, Johnson C. Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore). 1988;67(1):66-76.

Pham PT, Pham PC, Wilkinson AH, Lew SQ. Renal abnormalities in sickle cell disease. Kidney Int. 2000;57(1):1-8.




How to Cite

Gamit, B., Patel, A., Shah, P., & Padsala, S. (2017). Autopsy findings and pattern of mortality in undiagnosed sickle cell disease patients. International Journal Of Community Medicine And Public Health, 3(5), 1094–1100.



Original Research Articles