Excess cardiac iron deposition leads to congestive cardiac failure and accounts for more than 70% of deaths in thalassemia major patients. In three separate studies involving 145 thalassemia patients, serum ferritin and magnetic resonance imaging (MRI) relaxation times T2 and T2* have been compared for assessing iron load levels during chelation treatment. In two studies, variable levels of cardiac iron load have been detected by T2 and T2* in patients treated with deferoxamine (DFO), which, however, were unrelated to serum ferritin. In most cases, similar range levels from normal to severe cardiac iron load could be identified by both the T2 and T2* methods. However, in a few cases there were substantial differences in the levels detected between the two methods. In the third study, the ferrikinetics of the normalization of the iron stores during the International Committee on Chelation (ICOC) deferiprone (L1)/DFO combination protocol was followed up using T2 and T2* and serum ferritin. Iron deposits were found not to be proportionally distributed between the liver and the heart or uniformly distributed within each organ. Iron mobilization in each patient varied and iron deposits in each organ were cleared at different rates. Despite some limitations, the application of the MRI relaxation times T2 and T2* offers the best diagnostic methods for iron overload estimations in most organs and especially the heart. These MRI methods and serum ferritin could also be used for the ferrikinetics of iron mobilization and removal during chelation therapy and the normalization of the iron stores during the ICOC L1/DFO combination protocol. There is a need to standardize the two MRI relaxation times T2 and T2* methods and identify the factors causing the differences between them.

These data indicate that less frequent ANC monitoring and continuation of deferiprone therapy during neutropenia are not associated with prolonged neutropenia or with progression to agranulocytosis.

996 The incidence of agranulocytosis during deferiprone (Ferriprox®) use has been reported in clinical trials where patients' neutrophil counts were generally monitored weekly and deferiprone was discontinued at the first sign of neutropenia (neutrophil count < 1.5 × 10 9/L), but the incidence in a less rigorously monitored environment is unknown. This observational, open label, prospective, multi-centre, non-interventional drug surveillance program was designed to assess how the safety of deferiprone therapy is monitored in clinical practice. All patients in the 15 participating treatment sites (Egypt, Oman, Saudi Arabia and Turkey) who initiated therapy with deferiprone during the observation period, were enrolled in the program. There were no exclusion criteria. The program was approved by local ethics boards and informed consent was obtained. 294 patients (53.4% males) were enrolled and the results of the first year of treatment are reported herein. Mean (SD) age of all patients was 12.2 ± 10.1 years (range 1 to 52 years old). 224 (76%) were children (93 of which were 1–5 years; 83 were 6–11 years and 48 were 12–17 years old). The majority of the patients had a diagnosis of Thalassemia Major (N= 261). The other patients had Thalassemia Intermedia (N=17), Sickle Cell Disease (N=9), Spherocytosis (N=3), Red Cell Aplasia/Diamond Blackfan Anemia (N=2), Immune Hemolytic Anemia (N=1) or Sideroblastic Anemia (N=1). Deferiprone was initiated as monotherapy (20 to 100 mg/kg/day) in 247 patients or added to deferoxamine therapy in 41 patients or to deferasirox in 6 patients. Serum ferritin was the common measure of iron overload in all 294 patients. Measurement of cardiac (CIC) and liver iron content (LIC) were conducted within 1 year prior to enrollment in 16 patients. Frequency of those measurements and the technique for those assessments varied among participating centers. CIC and LIC were assessed in 14 patients during the first year of follow up. At completion of 1 year of observation, the mean serum ferritin of the 282 patients who had a baseline and at least one follow up assessment had declined from 2858 ± 2481 to 2454 ± 2074 μg/L (p<0.0001). Monitoring of the neutrophil count was conducted at an average interval of 5 ± 4 weeks (1 week to 6 months). One patient (0.3%) experienced agranulocytosis, which resolved with G-CSF 8 days after discontinuation of deferiprone. Ten patients (3.4%) experienced neutropenia (neutrophil count <1.5 × 109/L but not <0.5 × 109/L). One neutropenia was related to acute myeloid leukemia (AML). Deferiprone therapy was interrupted at diagnosis of AML, considered not related to DFP; and the patient was withdrawn from the program. Two patients had two episodes of neutropenia. All neutropenias, except the one associated with AML, resolved within 51 ± 48 days (range 8 to 167 days). Deferiprone therapy was continued in 7 episodes of neutropenia (time for resolution was 28 ± 24 days (range 8 to 67 days)). Deferiprone was interrupted in the remaining 4 episodes (time for resolution was 93 ± 55 days (range 35 to 167 days). None of the neutropenias progressed to agranulocytosis. The data from this observational study indicate that less frequent monitoring of the neutrophil count and continued deferiprone therapy during neutropenia was not associated with prolonged duration of the neutropenia or progression to agranulocytosis. Further evaluation of this observation is warranted. Disclosures: Elalfy: ApoPharma Inc.: Research Funding. Off Label Use: In USA, FERRIPROX® (deferiprone) is an iron chelator indicated for the treatment of patients with transfusional iron overload due to thalassemia syndromes when current chelation therapy is inadequate. Shebl:ApoPharma Inc.: Research Funding. Badr:ApoPharma Inc.: Research Funding. Elsafy:ApoPharma Inc.: Research Funding. Salama:ApoPharma Inc.: Research Funding. Al-Tonbary:ApoPharma Inc.: Research Funding. Abdel Rahman:ApoPharma Inc.: Research Funding. Qari:ApoPharma Inc.: Research Funding. Al Damnhouri:ApoPharma Inc.: Research Funding. Al Hawsawi:ApoPharma Inc.: Research Funding. Wali:ApoPharma Inc.: Research Funding. Yesilipek:ApoPharma Inc.: Research Funding. Kilinc:ApoPharma Inc.: Research Funding. Yazman:ApoPharma Inc.: Research Funding. Karakas:ApoPharma Inc.: Research Funding. Tricta:ApoPharma Inc.: Employment.

Background and Objectives: Beta-thalassemia (BT) has a high prevalence in Mediterranean, Southeast Asian, and African countries. Studies stated that thalassemia is an endemic disease that causes significant health problems in Cyprus. This study aimed to measure the contact angle between the implant and blood samples from BT major patients and healthy individuals to compare the contact angles and wettability of Grade 5 titanium implant surfaces. Materials and Methods: Grade 5 titanium discs that were 10 mm in diameter were used since they mimic the surface of dental implants. Following receiving informed consent, blood samples were taken from the patients’ index fingers in each group with lancet needles and a photo of the contact angle between the blood samples and the titanium surface was taken; the collected blood was transferred to a titanium disc with a medical pipette. ImageJ software with a specific contact angle plugin was used for the contact angle measurements. Results: Theta-mean, theta-circular, and theta-ellipse values were compared between all groups, and no significant difference was found (p > 0.05). Conclusions: In this study, it was hypothesized that the patients’ rheological property of decreased deformability would affect the wettability of implant surfaces in vitro; however, no such finding was reached in this study. Since in-depth studies associated with dental implant success in BTM patients are absent in the literature and Cyprus is one of the Mediterranean countries with a high prevalence of BTM, this study was conducted to enrich the literature. While some systemic diseases may affect the contact angle between the implant surface and blood, it can be concluded that this condition was not present for BTM patients in our study. Last but not least, we emphasize that this experiment was done on a single surface type and the results can be totally different when using other surface types.

Anaemia causes lower oxygen transport to tissues, interfering with normal physical development and may interfere with exercise and sports in thalassemia and Sickle-Cell-Disease (SCD) l. Moreover, in SCD, the abnormal haemoglobin alters the erythrocyte shape and leads to pulmonary parenchymal damage, impaired vascular function and micro vascular complications. However, we now accept that, with regular blood transfusions and efficient oral or parenteral chelation modalities, many patients lead a normal development and life with little or no change from the lifestyle of their unaffected friends, although there are cognitive and emotional factors leading behaviour for participation in the social life and exercise, as studied by many scientists in Italy, United Kingdom, Greece and India.贫血导致组织供氧不足，妨碍身体的正常发育，并可能干扰地中海贫血和镰状细胞病（SCD）患者的体育运动。更严重的是，在镰状细胞病患者体内，异常血红蛋白能改变红细胞形态，造成肺薄壁组织损害、血管功能损伤，并引起微血管并发症。 然而，意大利、英国、希腊和印度的多位科学家的研究表明：尽管认知因子和情感因子是参加社会生活和运动的主导行为，但只要患者定期输血和采用高效的口服或非口服螯合疗法，很多人都可以正常发育和生活，与未受感染的人群并无大的区别。