Study of sickle cell anaemia with clinical and hematological correlation

Vasundhara M., Ramdas R., Bhagya Lakshmi A., Satish Kumar S., Subash R.


Background: Sickle cell anaemia is a hemoglobinopathy with the production of abnormal hemoglobin, HbS which when exposed to hypoxemia produces sickling of RBC. Severity of the disease depends on the percentage of HbS. Rise in the levels of Hb F is associated with less intense clinical course. Drugs targeted at increasing levels of HbF have prognostic significance. The aim of the study is to analyse the age, sex distribution, clinical, hematological and electrophoretic appearances and to compare with other studies.

Methods: 300 sickle cell positive patients, both male and females who attended OPD were studied with all the clinical findings  and hematological parameters. 52 cases were subjected to electrophoresis.

Results: Males were 154 and females were 146. Degree of anaemia was severe in females when compared to males. Of 52 cases subjected for electrophoresis 36 were homozygous, 15 were heterozygous and 1 case Sickle thalassemia. 7 cases showed HbF above 5%.

Conclusions: Most of the patients were under 40 yrs suggesting decreased survival after that age.  An increased level of HbF was associated with better prognosis suggesting the need to target at drugs which increase HbF.


Sickle cell anaemia, Haematological parameters, Hb electrophoresis, HbF

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Egmond E, Rieber, Veliz G, Pollock S. Red Cells in Sickle Cell crisis. Observations on the pathophysiology of crisis. Blood. 1977;49:967-79.

Zanette AM, Gonçalves MS, Bahia RC, Nogueira LV, Arruda SM. Sickle cell anemia: delayed diagnosis in Bahia, Brazil--a largely Afro-descendant population. Ethnicity & Disease. 2011;21:243.

Lehman H, Cutbush M. Sickle cell trait in southern india. BMJ. 1952;1:404-5.

Gill FM, Brown A, Gallagher D, Diamond S, Goins E, Grover R, et al. Newborn experience in the Cooperative Study of Sickle Cell Disease. Pediatr Suppl. 1989;83:827.

Wild BJ, Bain BJ, Dacie, Lewis, 11 edn, Practical Hematology Sir John.V.Dacie 10th edn. Churchill and Livingstone, 310-317

Patel AB, Athavale AM. 2004. Sickle cell disease in Central India. Ind. J. Paediatr. 2004;71(9):789-93.

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

Gill FM, Sleeper LA, Weiner SJ, Brown AK, Bellevue R, Grover R, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Blood. 1995;86:776:83.

Saxena A, Phadke SR. Thalassemia control by carrier screening. The Indian scenario. Current science. 2002:83(3):291-5.

Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, et al. Pain in sickle cell disease: Rates and risk factors. N Engl J Med. 1991;325:11.

Marsenic O, Couloures KG, Wiley JM. Proteinuria in children with sickle cell disease. Nephrol Dial Transplant. 2008;23(2):715–20.

Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, et al. Cerebrovascular Accidents in Sickle Cell Disease: Rates and Risk Factors. Blood.1998;91(1):288–94.

Powars DR. The natural history of sickle cell disease: The first ten years. Semin Hematol. 1974;12:267.

Bailey K, Orris JSM, Thomas P, Serjeant GR. Fetal haemoglobin and early manifestations of homozygous sickle cell disease. Archives of Disease in childhood. 1992;67:517-20.

Diggs LW, Ahmann CF, Bibb J. Incidence and significance of sickle cell trait. Ann Intern Med. 1933;7:769-78.

Sears DA. The morbidity of sickle cell trait: a review of the literature. Am J Med. 1978;64:1021-36.

Kiryluk K, Jadron A, Gupta M, Radhakrishnan J. Sickle cell trait and gross hematuria. Kidney Int. 2007;71:706-10.

Allison AC. Protection afforded by sickle cell trait against subtertian malarial infection. Br Med J. 1954;1:290-4.