THE SCIENTIFIC JOURNAL OF IRANIAN BLOOD TRANSFUSION ORGANIZATION (KHOON)
Year:2011 | Volume:8 | Issue:1 (30)|Papers Count:10

Background and Objectives: Antithrombin (AT), protein C (PC) and protein S (PS) deficiencies plus activated protein Cresistance (APC-R) are among the most common hereditary causes of VTE. No comprehensive study on the prevalence of natural anticoagulant protein deficiencies in thrombophilic patients had been done before in Iran so we aimed to analyze these deficiencies in Iranian VTE patients.Materials and Methods: This descriptive-retrospective analysis was done on 734 patients presented with the symptoms of VTE who were referred to Iranian Blood Transfusion Organization for thrombophilia screening tests in 2004-2005. After exclusion of patients with a history of an acquired thrombophilic condition, sampling of AT, PC, PS and APC-R activities were performed consecutively on 404 patients. Z-test was used to compare the present results with those of other population studies.Results: Out of the total number of 404 patients, 369 (91.3%) were presented with DVT, 63 (15.6%) with PE, and 9 (2.2%) with TUS; there were also 39 patients (9.6%) with coexistence of two kinds of thrombosis. The deficiency of PS in 92 patients (22.8%), APC-R in 44 (10.9%), PC deficiency in 27 (6.7%), AT deficiency in 21 (5.2%) and compound deficiencies in 36 (8.9%) were observed. Conclusions: PS deficiency and APC-R were the most and the next most prevalent hereditary defects in our patients, respectively. The findings of this report are more compatible with the results of Asian countries than those of European-American.

Background and Objectives: Survivin (SVV) is an inhibitor of apoptosis. Its expression rises in most cancer types and is associated with resistance to chemotherapy, increased recurrence, and decreased patient survival. In this study, the expression of SVV gene was analyzed in APL(Acute Promyelocytic Leukemia) patients.Materials and Methods: In this case-control study, the blood samples of 50 patients were collected in three groups; diagnosis, remission, and recurrence. Then, SVV gene expression was studied using absolute quantitative real time PCR. The data were analysed with SPSS version 17, Kruskal-Wallis and Tukey tests.Results: For the first time, this study demonstrated that SVV overexpressed significantly in APL patients compared with the control (Mean ± SD; 910.5 ± 699) (p <0.01). This overexpression was seen both in diagnosis (4981.4 ± 4112.2) and recurrence groups (4584.2 ± 5133.6) (both p<0.01). After arsenic trioxide therapy (ATO) the SVV expression declined significantly as compared to the diagnosis group (p<0.01).Conclusions: Findings indicate that SVV may have a role in survival of APL cells and induction of apoptosis by decreasing SVV expression can be a probable mechanism of ATO. This study indicates that the SVV may be used as a biomarker in APL patients during the course of the disease.

Background and Objectives: The molecular basis for thalassemia intermedia (TI) is determined in most thalasemia affected countries, but in Iran there has been no perfect investigation so far. This report is the results of the first step of a comprehensive analysis on molecular basis of TI in Iran. Two most important factors influencing the phenotype of TI, i.e. beta globin gene mutations, Gg XmnI polymorphism, and genotype/phenotype correlation in these patients were analysed.Materials and Methods: In an experimental pilot study, 42 TI patients who referred to Ali-Asghar Hospital were selected and genomic DNA was extracted by salting out method. The ARMS-PCR technique was performed to detect the most prevalent b-thalassemia mutations in Iran: IVSII-1 (G>A). Direct DNA sequencing was performed on the samples which had at least one unidentified allele. Simultaneously, GgXmnI polymorphism was determined using PCR-RFLP procedure. Afterwards, the association of this polymorphism with TI was analyzed. Results: Among 76 chromosomes, IVSII-1 was the most frequent mutation detected with 42 alleles (55.26%). Totally, 66 b globin alleles (86.84%) were b0 and 7 (9.21%) b+. In addition, 61 chromosomes (80.26%) were positive for XmnI polymorphism. This polymorphism was in strong linkage to b0 mutations, mainly IVSII-1. No cases with IVSII-1 mutation were XmnI-/-. Conclusions: It seems that the presence of XmnI polymorphism may play an important role in reducing the clinical severity of thalassemia in patients with severe b0 alleles. However, other genetic factors should be also investigated.

Background and Objectives: Thalassemia is the most common hereditary anemia in Iran. Considering the recent treatment protocols and comprehensive data about clinical status of thalassemic patients, their survival rate has improved. The aim of this study was to investigate the clinical condition of patients in Adult Thalassemia Clinic.Materials and Methods: This cross sectional analysis was done on 695 patients having referred to Adult Thalassemia Center in 2008. We reviewed the medical files for some demographic and lab data about serum ferittin, hepatitis B, C, and HIV. The results were analyzed with Chi Square and Pearson correlation tests by SPSS version16.Results: We had 695 patients: 358 (51.5%) male and 337 (48.5%) female with mean age of 26.26 years. Out of the total unmber of patients, 501 (72%) were major thalassemia and 170 (24.5%) intermedia. The mean ferritin level was 1755.16 ng/ml. Moreover, 423 (59.2%) of patients were spelenectomized. Out of our major and intermedia patients 24.5% were HCVAb positive out of which 66% were HCV PCR positive. For HBV, 565 (81.2%) of our patients were HBsAb positive after vaccination; 90 (12.9%) were immune to HBV from past infections (HBcAb+). Totally, 655 (94.2%) were immune to HBV. We didn’t have any HIV positive patient. Conclusions: In our study, the quality of treatment is evaluated to be good as compared with other centers. We advise regular periodic control of patients for early dignosis and treatment to decrease mortality and morbidity rate of patients.

Background and Objectives: Viral RNA is the first blood marker which appears in HIV-1 infection. RT-PCR and NASBA are the commonly used techniques for amplification of RNA. NASBA technique provides more advantages over RT-PCR. In this study, an NASBA assay combined with an easy, sensitive enzyme-linked immunosorbent assay (ELISA) was developed for detection of HIV-1 RNA.Materials and Methods: 11-dig-UTP was added to an NASBA reaction mixture and the RNA amplicons were labeled. Then, the dig-labeled NASBA products hybridized to a biotinylated specific probe in hybridization solution buffer and the hybrids were transferred to a streptoavidin-coated plate. After several washing processes and emission of nonspecific products, the final detection of the captured RNA was done by addition of anti-dig antibody-enzyme conjugate and the substrate.Results: The results demonstrated that, NASBA is an efficient method for amplification of the target genome. Detection of NASBA products by agarose gel electrophoresis showed a unique 176 bp band corresponding to the specific target. For the detection of NASBA products, an ELISA assay was performed; using 0.01 PM probe, the OD of 1.049 ± 0.11 was obtained. Conclusions: In this study, by using suitable primers and probe a highly sensitive and specific NASBAELISA method for detection of HIV-1 developed.

Background and Objectives: There is a loss of iron with each donation and reports have shown iron deficiency in regular blood donors. This study aimed to determine the prevalence of iron deficiency in regular male blood donors and study the related factors.Materials and Methods: In this cross-sectional study, a total of 253 regular volunteer male blood donors during 2007, were sent for blood tests (complete blood count, serum iron, and ferritin) within 20 days either at pre-donation or post-donation periods. Data were analyzed with Pearson Correlation test by using SPSS 15.Results: Thirty two (12.6%) had iron deficiency anemia (hemoglobin less than 12.5 g/dl), 67 (26.6%) had low serum iron (less than 50 micg/dl), 135 (54.9%) had low ferritin levels (less than 17 ng/ml) and 13 (5.1%) had hematocrit lower than 38%. The number of donations per year had a significant negative correlation with hemoglobin, hematocrit, serum iron, and ferritin levels (p< 0.001, p< 0.005, p< 0.009, p< 0.001, respectively); the total number of donations also showed a significant negative correlation with hemoglobin, hematocrit, serum iron, and ferritin levels (p< 0.001, p< 0.004, p<0.001 and p< 0.023, respectively). MCV also lowered as the number of donations per year increased (p< 0.01). Conclusions: Iron stores in regular male blood donors were low which showed a strong correlation with the number of donations per year and total number of donations. Since recruiting regular blood donors is essential, preventing iron deficiency by early diagnosis will be beneficial for unreturned rate of regular donors to decrease.

Background and Objectives: Platelets are the smallest blood cells which play a major role in hemostasis. The aim of this article is to investigate the most effective cell adhesions and the most important signaling pathways to activate or deactivate platelets at the site of vessel injury.Materials and Methods: The present manuscript, using 52 recent published articles, introduces the latest information regarding the platelet molecular interactions and signaling pathways.Results: Platelets at the site of vessel injury bind to the subendothelial extracellular matrix through their surface receptors. These interactions lead to activation of some intracellular pathways contributing to the formation of blood clots. The molecules involved in these interactions are the extracellular matrix molecules such as laminin, fibronectin, collagen, vWF and the platelet surface receptors such as GPVI, GPIb-V-IX, aIIbb3 and a2b1 integrins. Platelet functions in hemostasis, thrombosis and inflammation are directly attributed to the platelet-cell interactions, cell junctions and the signaling pathways regulated through these interactions.Conclusions: Studying this field and the related mechanisms provides a better understanding of coagulation and platelet dependent hematologic diseases, and may offer new perspectives to immunogenic disorders.

Background and Objectives: Multiple studies in Iran reported different prevalence rates for HCV infection in thalassemics but most studies were based on serologic tests not on molecular tests. The present study was carried out to determine the prevalence of HCV in thalassemics based on serologic and molecular tests.Materials and Methods: A retrospective cross-sectional study was conducted on 91 thalassemic patients. Demographic data were obtained from patient files and serum specimens were tested by ELISA for anti- HCV antibody and by RT-PCR for HCV RNA. Serum aminotransferase (ALT&AST) were also tested.Results: Out of 91 patients, 41 (45%) were male and 50 (55%) female, with mean age range of 13.2 ± 5.7 years. The overall prevalence rate of anti-HCV was 21% (19.91, CI 95%=17.77-25.82). Nine of anti-HCV positive patients (9.19, 47%) were HCV RNA positive and 98% had high serum level of ALT and AST. Anti HCV- positive patients were significantly older than anti HCV-negative ones (p< 0.005).Conclusions: Because all anti HCV- positive patients do not have active HCV infection, it is suggested that before any decision to be made in case of seropositve thalassemic patients HCV infection be confirmed by RT- PCR and other precise tests.