---

Introduction: Thalassemia is common in Iran. Appropriate therapy for this disease includes a regular blood transfusion and chelation therapy. However, patients will inevitably confront with side effects, particularly iron overloads in critical organ including heart, ductless glands and liver. This study tries to determine the prevalence of diabetes mellitus in transfusion dependent β thalassemia major.

Patients & Methods: This is a cross sectional study and included all 437 patients suffering Thalassemia major that were referred to medical centers linked to the Iranian blood transfusion organization since Jan 2004 up to Jan 2005. All patients tested for CBC, FBS, 2hr BS, HbAlC, liver and renal function, and endocrine disease. Initially, reports of adenoidal experiments as well as other associate parameters were provided via medical folders.

Results: Four hundreds and thirty-seven patients enrolled in this study, the diabetes found in 28 patients (5.4%). There was no relationship between sex and the kind of Thalassemia. In patients with Thalassemia intermedia, the mean of age was 34.7 ± 1.4 years and the mean of ferritin was 893.2 ± 122.4 Mg/dl. and patients with Thalassemia major, were 27.1 ± 0.3 years and 1678.8 ± 87.5. Mg/dl respectively the mean of age and ferritin in two groups of Thalassemia intermedia and Thalassemia major had significant difference while the mean of ferritin in diabetic and non-diabetic groups had no significant difference.

Conclusion: We can conclude According to previous studies considering endocrine abnormalities in thalassemic patients, that our data is just like others.

---

Haemovigilance is a system with standard program to cover the entire transfusion chain, monitor, evaluate and analyse the data to improve patients’ safety. We report the implemented haemovigilance system in hospitals and transfusion reactions (TR) in Iran. Methods and Materials: This was a prospective descriptive study. The national reporting system for transfusion incidents was introduced in January 2009. In the period 2009- 2011, 47 hospitals in a mandatory manner reported transfusion incidents among patients to the blood bank in a mandatory manner. All incidents were anonymously recorded in a standardized report form and registered in 10 categories. Results: A total of 377 transfusion incidents were reported and categorized as: incorrect blood component transfused (n = 4), Febrile non hemolytic transfusion reaction (FNHTR) (n=84), Immune hemolytic transfusion reactions (n =12), nonimmune hemolytic transfusion reactions (n = 7), allergic reactions (n = 247), transfusion-related acute lung injury (n = 2). There were no reports in the categories of infections, post-transfusion purpura, transfusion-acquired viral infection, and transfusion-related graft versus host disease. Conclusion: Haemovigilance is a tool for quality improvement and better surveillance on patient safety. The safety and quality of blood transfusions can be improved if we follow the transfusion chain, prevent and treat transfusion reactions and report adverse reactions to change the protocols. Key Words: Adverse reaction, Blood Safety, Haemovgilance, Risk

---

Background: The advances in treatment regimes for thalassemic patients have increased the survival among them therefore osteoporosis has emerged as an important cause of morbidity. The aim of this study was to determine the prevalence of osteoporosis and osteopenia in patients with thalassemia from Zafar Adult Thalassemia Clinic, Tehran, Iran. Patients and Methods: In this cross sectional investigation, we studied 239 patients with β thalassemia major and 87 patients with thalassemia intermedia with a mean age of 29±8 years. All demographic data including age, weight, height, sex, age at diagnosis, age at blood transfusion initiation, chelating agent therapy and serum ferritin level were obtained from patients’ history. Bone mineral density of the lumbar spine (L1-L4) and femoral neck was determined using dual-energy X-ray absorptiometry (DEXA). Results: The prevalence of osteoporosis was 65.6% (214 out of 326 patients). Osteoporosis was present in 10.7%, 11% and 43.9% of patients in the lumbar spine alone (L1-L4), femoral neck alone and both places, respectively. In the rest of patients 18.7% showed osteopenia and only 15.7% were normal. Osteoporosis was more prevalent in patients with thalassemia intermedia compared to thalassemia major (p<0.001). Also higher age of patients, longer duration of transfusion and longer intervals between transfusions had a positive correlation with osteoporosis. Conclusion: The prevalence of osteoporosis among Iranian thalassemia patients is similar to prevalence reported elsewhere. Bone Mineral density is a good index of bone status in patients with thalassemia and recommended to be done for thalassemic patients annually. Key words: Thalassemia, bone mineral density, osteoporosis, DEXA.

---

Background: In thalassemia major as a chronic disease patients need to require information about the disease processes and therapeutic interventions. The aim of the present study was to evaluate the knowledge, attitude, and practice behavior of thalassemic patients. Patients and Methods: This was a cross-sectional descriptive knowledge, attitude and practice study conducted in Zafar adult thalassemia clinic, Tehran, Iran, with a simple random sampling. The questionnaire was designed by a research team including an expert physician involved in counseling and education of thalassemic patients for many years. Results: One hundred and ninety thalassemic patients entered the study. Ninety nine patients (52.1%) were female, 91(47.9%) were male, with age range of 14 to 48 years (mean 26.79 ±5.96). Results of the knowledge part of the questionnaire indicated that 39 % of patients had poor information about thalassemia 32.5% had little information and 28.5% had satisfying information. Fifty eight percent of patients had a positive attitude towards their appearance, quality of life and social relations. Positive attitude was significantly correlated with good knowledge (p=0.009). Low knowledge about the disease had caused treatment to be irregular and improper. Based on our questionnaire 144 patients (75.8%) were depressed which was significantly higher among females (P=0.002). Conclusion: Increasing knowledge, attitude and practice awareness among thalassemic patients considering their disease and treatment methods has a positive influence in their quality of life. This will improve their performance to deal with their lifelong disease and its challenges during the course of treatment.

---

Background: Deferoxamine mesylate is still the conventional and well-known iron chelator for patients with thalassemia major. However, due to some marketing issues the well-known original brand, Desferal®, produced by Novartis Pharmaceuticals Company is not as available as before. The generic brands of Deferoxamine have been introduced in many parts of the world including Iran; however, they are not well accepted by the physicians and patients yet. This triple-blind randomized controlled trial was designed to compare the efficacy and safety of a new Iranian generic and the original brand product of Deferoxamine mesylate in Iranian patients. 
Methods: The present Randomized triple-blind controlled trial research was carried on  in nine centers throughout Iran. They were randomly divided into two similar groups and a Cross-over study was designed. 24-hour urine was collected after subcutaneous infusion of either drugs and urinary iron excretion was measured via atomic absorption spectrophotometer device. Acute adverse events during and after drug infusion were recorded. Mack Nara test and p-pair test were applied to compare two cross over interventions. 
Results: 154 patients from 9 centers were enrolled in this study. There were 95 women and 59 men aged 6-34 years (mean age of 21.1years). Mean urinary iron concentration for Desferal (intervention A) vs. Desfonak (intervention B) groups was 22.5±22.6 vs. 21.5±16.9 mg/m2 , respectively. Mean urinary iron excretion/Kg body weight for Desferal (intervention A) vs. Desfonak (intervention B) groups was 0.48±0.48 vs. 0.47±0.40 mg/m2, respectively. 
Conclusion: According to the results of this study, there was similarity between efficacy and safety of the original and generic brands of deferoxamine (desferal vs. desfonac).