Trend in Prevalence of Hepatitis C Virus Infection among ß-thalassemia Major Patients: 10 Years of Experience in Iran.

BACKGROUND: Hepatitis C virus (HCV) is the leading cause of transfusion transmitted infections (TTIs) among patients with ß-thalassemia major. A high prevalence of HCV was reported among these patients. The aim of this study is seeking the trend of the prevalence of HCV infection among the patients with b-thalassemia major in Guilan province, Northern Iran over a 10-year period. METHODS: A retrospective study was conducted on 1113 patients with b-thalassemia major in the Guilan province, northern Iran from 2002 to 2012, using multiple data sources. A blood sample was taken from each patient, and a questionnaire regarding demographic data and risk factors was completed for them. Enzyme-linked immunosorbent assay and recombinant immunoblot assay for HCV were performed in all cases. A stepwise forward logistic regression analysis was done. RESULTS: The prevalence of hepatitis C infection among ß-thalassemia major patients was 13.6%. The risk of hepatitis C among ß-thalassemia major patients was greater before screening program for HCV (odds ratio = 9.6, 95% confidence interval: 2.3-40.5). In addition, the prevalence of anti-HCV seropositivity was decreased dramatically among patients who have received transfusions after implementation of blood donor screening for HCV. There were no positive HCV cases in the patients younger than 10 years. CONCLUSIONS: The risk of TTIs including HCV can be reduced by implementing screening program for healthy blood.

Effect of hepatic iron concentration and viral factors in chronic hepatitis C-infected patients with thalassemia major, treated with interferon and ribavirin.

BACKGROUND: Beta thalassemia major patients are vulnerable to transfusion-transmitted infection, especially hepatitis C virus (HCV), and iron overload. These comorbidities lead to cirrhosis and hepatocellular carcinoma in these patients. In order to prevent these complications, treatment of HCV infection and regular iron chelating seems to be necessary. The aim of this study was to evaluate the effect of hepatic iron concentration (HIC) and viral factors on the sustained virological response (SVR) in chronic HCV-infected patients, with beta thalassemia major being treated with interferon and ribavirin. MATERIALS AND METHODS: We enrolled 30 patients with thalassemia major and chronic HCV who were referred to the Hematology Clinic of Guilan University of Medical Sciences, between December 2002 and April 2006. HIC was measured by atomic absorption spectroscopy before treatment. The viral factors (viral load, genotype) and HIC were compared between those who achieved a SVR and nonresponders. RESULTS: Mean age of the 30 thalassemic patients, was 22.56 ± 4.28 years (14-30 years). Most patients were male (56.7%). Genotype 1a was seen in 24 (80%) cases. SVR was achieved in 15 patients (50%). There were no significant correlations between HIC (P = 1.00), viral load (P = 0.414), HCV genotype (P = 0.068), and SVR. No difference was observed in viral load (P = 0.669) and HIC (P = 0.654) between responders and nonresponders. CONCLUSION: HIC, HCV viral load, and HCV genotype were not correlated with virological response, and it seems that there is no need to postpone antiviral treatment for more vigorous iron chelating therapy.

Alpha-thalassemia mutations in Gilan Province, North Iran.

One hundred and three patients from Gilan Province, Iran, presenting with hypochromic and microcytic anemia parameters without iron deficiency were included in this study. Using gap-polymerase chain reaction (gap-PCR), reverse hybridization StripAssay and DNA sequencing, we detected a total of 113 alpha-globin mutations in 94 (91.3%) of these patients. Most prevalent of the 16 different alpha-thalassemia (alpha-thal) alleles was -alpha(3.7) (42.5%), followed by the polyadenylation signal (poly A2) (AATAAA>AATGAA) (12.4%), Hb Constant Spring [Hb CS, alpha142, Term-->Gln (TAA>CAA in alpha2] (10.6%), --(MED) (8.8%), IVS-I donor site [GAG GTG AGG>GAG G-----, alpha(-5 nt) (-TGAGG)] (7.1%), -alpha(4.2) (4.4%) and poly A1 (AATAAA>AATAAG) (3.5%). An additional nine mutations were observed at frequencies below 2%. We also found two novel alpha1 gene mutations: alpha(-9) (HBA1: c.-9 G>C) and alpha(IVS-I-4) (HBA1: c.95+4 A>G). Our new findings will be valuable for improving targeted thalassemia screening and prevention strategies in this area.

Concomitant Hodgkin's lymphoma and atopic dermatitis in a child with Celiac disease.

Celiac disease is an autoimmune disease associated with increased risk of several diseases including a variety of malignancies. This is the first report of the concomitance of atopic dermatitis and Hodgkin's lymphoma in a child with celiac disease. Here, we report an 11-year-old boy with chronic diarrhea, glossitis, chronic dermatitis, and megaloblastic anemia who later developed Hodgkin's lymphoma.

Elucidating the spectrum of alpha-thalassemia mutations in Iran.

Alpha thalassemia (alpha-thal) is one of the most common hemoglobin (Hb) disorders in the world. Alpha-globin genes are located on chromosome 16. The majority of alpha-thal mutations are deletions but point mutations are found as well. Since the Iranian population is a mixture of different ethnic groups, frequency and distribution of alpha-globin mutations in various regions of the country need to be clarified. These findings can contribute to a wider understanding of this disorder.

Cutaneous adverse reactions to hydroxyurea in patients with intermediate thalassemia.

Although the cutaneous effects of hydroxyurea have been described for patients with sickle cell anemia, myeloproliferative disorders, and psoriasis, there are no reports of cutaneous adverse effects from hydroxyurea when used for patients with intermediate thalassemia. Therefore 43 patients with intermediate thalassemia treated with hydroxyurea were examined by a dermatologist, and pertinent cutaneous findings were recorded. These patients had received hydroxyurea for a mean of 15.5 months. Nineteen had cutaneous hyperpigmentation, eight had xerosis, and three were found to have one cafe au lait macule each. Eleven patients had nail abnormalities, including nail ridging, partial leukonychia, and longitudinal melanonychia. There were no cases of leg ulceration. It was concluded that the risk of developing leg ulcers and pigmentary disorders appears to be related to the underlying disease being treated, as well as to a patient's age, gender, and pigmentation.