ALBERT

Description: INTRODUCTION: Life expectancy of thalassemia patients has markedly increased over the last few decades. Nevertheless patients suffer from many complications of this congenital chronic disease.The presence of a highincidence of thrombotic events has led to the identification of a hypercoagulable state in these patients.The thrombotic risk is higher in β-thalassemia intermedia and splenectomized patients. The mechanisms responsible for the increased thrombotic risk are still unclear. Several factors that contribute to the hypercoagulable state in patients with thalassemia have been identified: chronic platelet activation, abnormal red blood cells, microparticles,iron overload, endothelial damage, splenectomy, decreased levels of anticoagulant factors and presence of prothrombotic mutations. We aimed to assess hypercoagulability in children with β-thalassemia. DESIGN AND METHODS: Sixty eight thalassemia major and 42 thalassemia intermedia patients included our study. The control group consisted of 41 age and sex matched healthy children. Demographic data of patients were recorded from their medical records. None of the thalassemic patients have thrombosis before. To evaluate the relative role of microparticles, blood cells and plasma: coagulation tests (prothrombin time, activated prothrombin time, fibrinogen and d-dimer), serum coagulation factor levels (factor II, V, VII, VIII, IX, X, von willebrand factor, protein C, protein S, antithrombin III), procoagulant phospholipid activity and thrombin generation assay were studied from plasma, thromboelastography from whole blood. The main component of microparticles are negative anionic phospholipids.Procoagulant phospholipid activity is a functional analyse to detect microparticles.Thromboelastography measures indices of the viscoelastic properties of whole blood after activation of coagulation and the thrombin generation assay measures the actual thrombin concentrations before and after the clot is formed. RESULTS: The median age is 144 months ( 11-236 months)in thalassemia major and 142 months (72-202 months) in thalassemia intermedia patients. Plasma factor II, factor V, factor IX, factor X and protein C levels were significantly lower in thalassemia major and intermedia patients than control subjects. Plasma phospholipid activity and whole blood thromboelastography parameters were all consistent with hypercoagulability in thalassemic patients, especially in splenectomized patients. Endogenous thrombin potential (area under the curve in thrombin generation assay) was significantly lower in thalassemic patients than control subjects and in non-splenectomized patients than splenectomized patients contrary to expectations. CONCLUSIONS: The hypercoagulability in thalassemic patients especially in splenectomized patients can be determined with procoagulant microparticle activity and whole blood thromboelastography but not with thrombin generation assay in platelet poor plasma. These findings showthat blood cells and/or platelets may be more important determinants of thrombotic risk rather than plasma abnormalities in thalassemic patients. Disclosures No relevant conflicts of interest to declare.

Description: A recent study showed that expression of Toll-like receptor and interferon-gamma associated genes is significantly increased in patients with chronic ITP. Interferon-gamma is an important protein which takes place in immunoregulation. +874A/T polymorphism in the first introne of interferon gamma gene is found to be associated with the development and clinical phenotype of some autoimmune diseases such as diabetes mellitus, thyroiditis, multiple sclerosis, and SLE. The aim of our study was to investigate whether interferon gamma +874A/T polymorphism is a risk factor for the development of ITP and whether it affects the clinical course and response to the treatment. Thirty five children with acute ITP and 40 children with chronic ITP who were followed for at least 6 months were included. Control group consisted 90 healthy children. Two millilitres of blood sample was taken into sterile tubes containing 0.1% EDTA from each child and all blood samples were stored at −20 until analysis. DNA was isolated from blood samples and interferon gamma +874A/T polymorphism was studied with real-time PCR and LightCycler TM. Twenty one patients had AA, 35 patients had AT, and 19 patients had TT genotype. In the control group, 47 children had AA, 36 children had AT, and 7 children had TT genotype. There was a statistical difference between ITP and control group regarding the genotype (p=0.001). The frequency of A and T alleles in ITP group was 52% and 48%, respectively. The frequency of A and T alleles in control group was 72.7% and 27.8%, respectively. The frequency of allele distribution was statistically different between the ITP and control groups (p

Description: Thalassemia Major (TM) represent one of the largest groups requiring regular transfusions lifelong. A total of 3830 patients are currently registered with homozygous b-Thalassemia in Turkey. This study is designated to reveal the seropositivity status of thalassemia patients on chronic transfusion treatment for blood-borne infections. A questionnaire inquiring the demographics and medical history of the patients as well as serological status for hepatitis B, C and HIV, was sent to 12 Thalassemia Centers which are generally settled down in the University or big government hospitals with high capacity blood banks where the packed red cells were provided. The collected data of 999 patients who have regular transfusions of 1–2 Unit red cells, every 3–4 weeks beginning from the first years of life and screened for transfusion transmitted viruses (TTV) 6 monthly was analyzed. The patients were aged between 0–53 years (median 16 years). Overall HBsAg and Anti-HCV positivity were found 2.5% and 12.6% respectively. Confirmed Anti-HIV positivity was 0.2% in the study group and consistent with the low frequency of the disease among blood donors in Turkey. Age distribution of sero-positivity within the patients was as follows: 0.7% in 0–5 years, 17% in 6–10 years, 48.3% in 11–20 years and 34% over 21 years, indicating the strong relation between the sero-prevalence of TTV and transfused blood volume and the gradually increased safety of blood in Turkey. The highest prevalence rate was seen in the age of 11–20 years which also represents highest number of patients. Donated blood screening for HBsAg, anti-HIV 1/2 (since 1985), anti-HCV (since1996), and RPR for syphilis is obliged by law in Turkey. Although, widespread use of HBV vaccination in transfusion dependent patients has been started since 1990s, the data confirmed that this measurement is insufficient alone for eradicating the HBV transmission in this group. Vaccination against HBV has been scheduled routine vaccination program of infancy by the health authority since 1999. However, the positive reflection of this policy in blood safety would be seen in the next generations. In that point of view, vaccination against hepatitis B can be practised in blood donors as a preventive measure in Turkey which is considered as in the middle endemic region regarding hepatitis B infection. Although, Anti-HCV screening in blood donors caused significant decrease in HCV infection, it still represents the main cause of morbidity following cardiomyopathy in this group of patients. This study clearly shows that the threat of TTV infections among multi-transfused thalassemia patients and underlines the importance of strengthen blood donor selection and implementing the sensitive screening assays for TTV at blood centers.