HAEMA (ΑΙΜΑ)
The Journal of the Hellenic Society of Haematology
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Diamond-Blackfan anaemia response to cyclosporin-A therapy six years after diagnosis
Nagwa Y. Fayea, Christofer J.H. Fryer, Sami K. Feliban, Adil AH. Abbas
Princess Norah Oncology Centre, King Khalid National Guard Hospital, P.O.Box 9515, Jeddah 21423, Saudi Arabia
Haema 2003; 6(3): 401-403
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Abstract. A boy with Diamond - Blackfan anaemia (DBA) diagnosed in infancy was started on cyclosporin A (CSA) therapy 10 mg/kg/day at 6 years of age after failure of prednisone therapy with increase of blood transfusion requirement and development of haemosiderosis. The patient exhibited a dramatic response to CSA with increase in reticulocyte count initially, followed by increase in haemoglobin level after splenectomy with minimal complications. Desferroxamine was started and the serum ferritin was reduced to <1000 mg/dl. He has remained transfusion independent for three years after starting CSA. Attempts to reduce the dose of CSA were followed by dramatic drop in his haemoglobin level and reticulocyte count. He is currently maintained on CSA and has no major side effects. CSA level was kept between 250-400 mcg/ml. His haemoglobin level ranged between 10-12 gm/dl and the reticulocyte count between 2-6%. These results suggest that CSA therapy should be considered in patients with DBA anaemia, in whom steroid therapy has failed, even years after diagnosis.
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