Sickle cell disease in young adult patients in basarh : Epidemiologic and major clinical characteristics

الملخص EN

Sickle cell disease (SCD) is a common disease in southern Iraq.

The aim of this study is to study the epidemiology and clinical behavior of SCD in Basrah.

This is a cross sectional hospital based study of 120 patients with SCD in Basrah from January 1999 to October 2001.

The total number of patients was 120,115 males and 5 females.

Majority of patients were from age range 15-18 year.

Half of them were from Abu-AL-khasib district.

Consanguineous marriages were present in 48.3% with positive family history of sickling disorder in 52.5% of cases.

Jaundice, painful crisis, splenomegaly, and hepatomegaly were reported in 55%, 50%, 40.8%, and 6.6% respectively.

Growth retardation and bone deformity occurred in 33.3%, 29.1% respectively.

Splenoctomy was done for hypersplenism in 8.3% and symptomatic gallstones were seen in 5.8%.

A vascular necrosis of femoral head reported in 3.3%.

Hospitalization history was positive in 54.1% of patients.

History of blood transfusion was reported in 62.5%.

The genotypes were sickle cell anemia (SCA) in 69.1%, 21.6% sickle /β+ thalassemia, 6.6% sickle cell trait, 1.6% sickle /β0 thalassemia, and 0.8% is sickle alpha thalassemia.

Hb F >10% reported in 70% (84 patients) with mean Hb F level of 16.3%±10.3%.

Target cells, irreversibly sickled cells and teardrop cells were present in 81.9%, 66.2% and 13.2% respectively of SCA.

Of those with SCA the anemia is normochromic normocytic in 61.4%, and hypochromic microcytic in 38.5%.

In conclusions, SCD in Basrah is not benign disease.

Despite the high HbF and persistent splenomegaly in to adult