PB2394 EVALUATION AND PROGRESS OF PATIENTS WITH SIGNIFICANT HEMOGLOBINOPATHIES IN GREEK PATIENTS WHO LIVE OUTSIDE OF ATHENS
Authors: Berdoukas V. , Kotsionis D. , Stamopoulou I. , Kattamis A. 
Published: June 2019

Background:

Greece has over 4500 patients with hemoglobinopathies, the majority of whom are thalassaemia major. More than 3000 of these patients live and are treated in areas outside of Athens, including on numerous islands. As the complications of thalassemia requires specaiist care, access to such care in many cases is limited. With cooperation with local centers of care, their physicians and local thalassemia societies we embarked on a project of travelling to those centers and providing evaluations and recommendations for management for any patients who wished to be reviewed. We were able to purchase a portable ultrasound that allowed echocardiography, equivalent in quality to office and hospital machines.

Aims:

The aim of the project was to a acquire a history from the patients, including known complications and information about their iron load status, iron chelation therapy, perform cardiac and general evaluation and to makre recommendations on their monitoring and therapy.

Methods:

Two Visits to Volos, Rhodes, Lesvos, and initial visits to Salonika, Kavala, Xanthi, Serres, Crete, Agrinio, Patras, Arta, Kalamata and Alexandroupoli have taken place. A total of 308 patients were reviewed initially and 111 twice. Most patients were reviewed by the cardiologist (DK) including echocardiography and the hematologist (VB). Data was collected by our clerical staff,including MRI and bone density findings when avaliable. Appropriate adjustments to the patients’ therapy were recommended e.g. for pulmonary hypertension and extramedullary erythropoiesis it was recommended that pre‐transfusion Hb be maintained above 110 g/L and for arrhythimas, anticoagulation to prevent stroke.. Revaluation was undertaken approximiately one year later in a number of centers to evaluate the benefit of the interventions. Ethics committee approval was obtained from the Lefkos Stavros Hospital, Athens.

Results:

From the tranfused patients at initial review, the mean pre‐transfusion Hb level was 100 g/L with a range of 80‐110 g/L (n = 280). The mean ferritin was 800 ng/ml (range 100‐18000)(n = 280). Mean LIC was 3.0 mg/g dry weight with a range of 0,9‐60 (n = 260). Cardiac T2∗ available in 192, showed 87.5% acceptable, 9.4% mild to moderate and 3.1% severe iron load. 17 (6.1%) patients showed pulmonary hypertension (PH) and 29 (10.4%) demonstrated arrythmias, the majority of which were atrial fibrillation. 3 patients had suffered embolic stroke because of atrial fibrillation. In 111 patients on subsequent review the mean pre‐transfusion Hb level was 105 g/L with a range of 100‐110 g/L (n = 111). The mean ferritin was 100 (range 100‐1500)(n = 111). Mean LIC was 1.0 mg/g dry weight with a range of 0.9‐4.5 (n = 111). Cardiac T2∗ showed 54% acceptable, 45 % mild to moderate and 1% severe iron load. in patients with PH there was an improvement in 4 to normal range and stabilization in 1.

Summary/Conclusion:

This project has proven valuable in providing service to patients who would have difficult access to major centres and appropriate subspecialists for their care. In some case, patients had unidentified complications that were either life threatening or at least likely to cause serious debilitating conditions. The patients reviewed twice showed improved iron status and in some the pulmonary hypertension showed improvement. This project will be ongoing in order to access more patients and also more subsequent reviews.