[Iron overload in sickle cell anemia: a study of 94 patients]

Sickle cell disease is an autosomal, recessive hemoglobinopathy characterized by hemolytic anemia. Red blood cell transfusions are uncommon therapeutic mainstay in sickle cell disease and repeated transfusions can result in iron overload. The predicted risks of iron overload and organ failure increase with both the duration of disease requiring transfusion therapy and the number of transfusions. To assess the state of iron overload in patients with sickle cell anemia according to their number of transfusions. The medical records of 94 patients with sickle cell anemia [46 had homozygous sickle cell disease, 41 had sickle-beta thalassemia, 7 had compound heterozygous hemoglobin: 4 SC and 3 SOArab] were retrospectively reviewed for the following: clinical exam, serum ferritin level, liver function tests, abdominal ultrasound exam and heart Doppler. 61% of our patients are from the Northern- west of the country. The average age is 18.29 years [2 to 62 years] and the sexratio is 0.62. In addition to parental consanguinity which is found in 28.72% of the cases. The average level of ferritin is 660.35 ng/ml. 41.5% of the patients have a high status of ferritin witch ranged from 521.4 to 3360 ng/ml. There is not a significant difference of ferritin level according to age, sex and a phenotype of sickle cell anemia. However, it is higher among the transfused patients with a same phenotype [p<0.05]. We found a correlation between serum ferritin levels and the number of transfusions [r =+0.74]. Splenectomy has a preventive role because it allowed stopping the transfusion in 65% of the cases. The evaluation of organ dysfunction has found a hepatomegaly in 29% of the cases, half of witch were have a high status of serumferritin [> 1000 ng/ml]. Left ventricular hypertrophy associated to valvulopathy was classified in 10% of the cases. Iron overload in sickle cell anemia, though relying on transfusion, remains moderate. The repetitive assessment of serum ferritin level is considered as the best test though it does not evaluate an organic dysfunction. To evaluate them better, other tests are requiring: magnetic resonance imaging and Tc-Squid biosusceptometers

[Beta thalassemia intermedia: about 36 cases]

Thalassemia intermedia empasses a mild clinical and biological spectrum. The aim is to report the clinical and biological features and treatment of this disease. It is a retrospective study about 36 thalassemia intermedia patients [17 males, 19 females]. Epidemiological, haematological aspects and treatment were reported. The diagnosis was carried out at a relatively old age 15 years [1-72].The thalassemia intermedia was characterized by mild facial deformities, splenomegaly and moderate anemia [Hb=9.1 g/dl]. The mean serum ferritin was 518 ng/ml [25-1800].Three phenotypes are caracterised: heterozygosis beta thalassemia, beta° thalassemia and beta° thalassemia. Clinical complications were hypersplenism, extra medullary hematopoiesis, leg ulcers, thrombosis and pulmonary hypertension. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenious [47%]. Evolution of this disease was generally good with a long lifespan at 31 years [6-83]. Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious