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 5th percentile; six (50%) had weight >/=25th percentile while eight (66.7%) had height >/=10th percetile. 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 routing blood transfusion, none of the patients were hospitalzed for any other morbid events in two years. High serum ferritin level (1482 +/- 766 mg/L) despite subcutaneous desferrioxamine remains a problem.