Clinico-pathological study of sickle cell anemia in Basrah

الملخص EN

Basrah is located in the world’s map of geographical distribution of sickle cell anemia (SCA) that carries significant morbidity.

Data on the true gene frequency, clinical spectrum of the problem and hematological and biochemical profiles of patients that could define the natural history of the problem in our country are scarce.In the present study the medical files of patients with SCA admitted to Basrah Teaching hospitals (1990-1998) were review review.

Data on age, sex, residence, clinical presentations, mean hemoglobin level, frequency of readmissions and blood transfusions were collected.

The clinical presentation of SCA were found to be protean.

The most frequent one was acute painful crisis (40%) following by aplastic crisis (10%) and hemolytic crisis (9%).

Infection comprised 30% of total presentation including urinary tract infection, osteomyelitis and gastrointeritis.

Splenomegaly, hepatomegaly and hypersplensim were reported in different frequencies (50%, 42% and 20% respectively).

Splenic sequestration was the the least common presentation (2%).

The mean hemoglobin level was 7.5± 1.2 gm % and the total frequencies of readmission and blood transfusion were 80-92 respectively.

It is concluded that the clinical severity of SCA in Basrah tends generally to be more sever than Mosul and Baghdad cases.

Different hemoglobin haplotypes, possible interaction between sickle cell gene and other red blood cells genetic marker such as thalassemia and GsPD deficiency and environmental factors are probable etiological for more sever clinical presentation in Basrah .