Abstract

Hematological profile of homozygous sickle cell disease patients attending RHDMC from Central India is presented. Central India has a huge population of sickle cell disease patients. Though predicted SS in the region is 22-44 %, 81 homozygous of sickle cell patients reported during study period of Jan 2003-Dec 2005. The clinical course of these patients is characterized in most of the cases by relatively long period without any symptoms punctuated by acute clinical events. Hematological profile of these 81 patients with age ranging from 6 month to 64 years is presented. There are 44 males and 37 females with an average age of 14.55yrs in males and 18.13 yrs females. Males out number females in pediatric age group where as females with SCD are attending hospital more in reproductive age group. Very few patients are reported after the age of 30 yrs. Average hemoglobin in males is 7.11 ± 2.13 gms/dl and in females 6.75 ± 1.85 gms/dl with parallel low RBC count.Hemoglobin rise is seen after 14 years of age in males and females. Age related rise in MCV is more noted in females after the age of 5 as compared to males. No age or sex related difference was seen in MCHC values. Hb A(2) levels for males is 2.13 ± 0.95% and for females 2.04 ± 0.91 %.Hb F in males is 19.58 + 5.86% and in females is 20.99 + 4.9%. There is no age and sex related difference in Hb F levels. Moderate to severe anemia with high Hb F dominate Central Indian sickle cell disease patient's hematological profile. The hematological profile in Central India is similar to the profile in other parts of India and Saudi Arabia but different from Jamaica and Africa.

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