ALBERT

Description: Abstract 4193 Introduction The management of red cell disorders especially Thalassaemias is a fine balance between adequate transfusion and maintainence of Ferritin levels within a threshold. To achieve this, UK thalassaemia guidelines advice : Study Is a retrospective audit to check if the above standards are met. The study period was 1 year (Jan –Dec 2008) ;the cohort being 12 patients(ages ranging from 22 to 46),10 with Beta thalassaemia major, 1 thalassaemia intermedia and 1 with erythropoietic porphyria. Data 1.pretransfusion Hb 64%transfusions had a mean pretransfusion Hb〉10. 2. Mean Ferritin levels : Treatment allocation 3 patients: had cardiac + liver iron overload & ferritin〉5000, were initially on Deferasirox, but later swapped to desferol and deferiprone combination based on MR T2*results. 3 patient s : had cardic and liver iron overload -2 had associated endocrine dysfunction with Ferritins 〉2000;the first was already on Desferol, Deferiprone was added to this. The second was on Desferol +Deferiprone combination with little response, was changed to Deferasirox increased upto 30 mg/kg, however the Ferritin levels showed a progressive rise and patient was considered for trial (Deferasirox40 mg/kg Vs Desferol). 1 patient had a Ferritin330 mls/kg/year) and pancytopenia on Desferol was considered for Splenectomy. 1patient: with clostrophobia was reluctant to undergo MR scanning, was commenced on Desferol + Deferoprone combination based on ferritin levels〉4000. Monitoring: All patients had monthly complete blood count,liver, kidney and endocrine function tests; and annual MR T2* imaging of heart / liver and ophthalmology and audiology assesments. Those with cardiac iron overload had cardiology followup .Those with liver iron overload had annual alphafetoprotein levels and Liver ultrasound. 3.Splenectomy : 42%(5/12)patients had a transfusion requirement of 〉200mls/kg/year. DISCUSSION Of the 12 patients:1 had regular 3 weekly transfusions,regardless of the Hb;3 patients were transfused on prewritten regimes designed at least 2 years earlier and lacked modification to current iron status.8 patients were transfused on a symptomatic basis-the most common symptom being ‘tiredness’-2 had left ventricular ejection fraction1500 in 75% of the patients could be improved by :strictly adhering to pretransfusion Hbs 9 to 10.5,reviewing prewritten transfusion regimes in the context of current iron overload,careful exclusion of other causes of symptoms ;avoiding transfusing patients purely on a 3 / 4 weekly basis .Lastly Splenectomy needs consideration in those with transfusion requirments〉200mls/kg/yr. Disclosures: No relevant conflicts of interest to declare.