Experience with thalassemia major in Al Baha

In a two-year retrospective review of the thalassemia program in our hospital, relevant clinical and laboratory items of information were extracted and analyzed. There were 12 regular attendants; seven males and five females, with a mean age of 6.9 +/- 3.3 years and a mean age at the time of diagnosis of 18.6 +/- 8.7 months. Mean hemoglobin at diagnosis was 6.5 +/- 1.1 g/dL and MCV was 67.9 +/- 4.6 fL. Predominant hemoglobin was HbF [30% to 98%]. Presenting features included pallor, abdominal distention, hepatomegaly, splenomegaly and occasional fever. Mean pretransfusion hemoglobin was 8.3 +/- 1.5 g/dL and mean post-transfusion hemoglobin was 12.2 g/dL. Only two [16.7%] had weight less than the 5[th] percentile; six [50%] had weight >/= 25[th] percentile while eight [66.7%] had height >/= 10[th] percentile. Mean weight gain per year was 1.3 +/- 0.82 kg and linear growth rate was 4.27 +/- 1.66 cm. One patient was positive for hepatitis C virus [detected by antibody] and another was positive for both hepatitis B [both antigen and antibody] and C. Apart from admissions for routine blood transfusion, none of the patients were hospitalized for any other morbid events in two years. High serum ferritin level [1482 +/- 766 mg/L] despite subcutaneous desferrioxamine remains a problem