DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20171853

Pattern of mortality in sickle cell disease: an autopsy study

Pinkal Shah, V. M. Bhagat, Komal Patel, Chandani Patel

Abstract


Background: Sickle haemoglobin is highly prevalent in western India. Sickle cell disease (SCD) is the generic term for the group of haemoglobinopathies caused by the occurrence of haemoglobin S (Hbs) in the homozygous form –sickle cell anaemia (Hbss) or as the heterozygous combination of Hbs with another abnormal haemoglobin such as Hbsc or beta –thalassaemias (Hbs b-thal). While doing autopsy in case of death with no apparent cause, the possibility of death may be due to vaso-occlusive crisis in sickle cell disease should be kept in mind. The findings at autopsy are variation of features which may or may not be directly connected to death. The goal is to draw awareness among physicians and relatives on need of autopsy as to minimize future unexpected death from complication or crisis and to enhance knowledge on both parties.

Methods: This was a study of autopsy specimens received between January 2015 to December 2015 at tertiary care hospital.

Results: Total of 679 autopsy cases were received, out of which sickled erythrocytes were detected in 25 cases. The mean age at death was 30 years, a male/female ratio of 1.5:1 and peak mortality was in the 2nd to 4th decades of life. The commonest presentation was sudden death. The cause of death in middle aged patients were vaso occlusive crisis, in paediatric patients were infection and in older patients were chronic organ damage.

Conclusions: Early diagnosis, prompt treatment and extended screening programme are necessary in prevalent tribal belt of western India to reduce morbidity and mortality. we should also introduce awareness programmes in tribal belt of western India.


Keywords


Autopsy, Sickle cell disease, Sudden death, Vaso occlusive crisis

Full Text:

PDF

References


Davies SC, Brozoic M. The presentation, management and prophylaxis of sickle cell disease. Blood Rev. 1989;3:29-44.

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

Shukla RM, Solanki BR. Sickle Cell Trait in Central India. The Lancet. 1985;1:297-8.

Kumar V, Abbas AK, Nelson F, Robbins and Cotran. Pathological Basis of Disease, 7th edition. 2008;628-32.

National health mission, state health society, Health and family welfare department, government of Gujarat. Available at: https://nrhm.gujarat.gov.in/.

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. Haemato J. 2002;3(1):56-60.

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.

Liesner RJ, Vandenberghe EA. Sudden death in sickle cell disease. J Royal Soc Med. 1993;86(8):484-5.

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

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):1198201.

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. Amer J Hemato. 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

Manish B. Shrigiriwar, Pankaj S. Ghormade, Chaitanya V. Tingne. Case report: Death due to sickle cell anaemia: Autopsy diagnosis. J Indian Academy Forensic medicine. 2013;35(4):383-5.