فصلنامه خون
سال هفتم شماره 2 (پیاپی 27، تابستان 1389)

Background and Objectives Thrombocytopenia is a common complication in neonatal intensive care unit neonates. The objective of this study is to identify the frequency and risk factors for neonatal thrombocytopenia among newborns. Materials and Methods Via a cross sectional and descriptive-analytical study, 201 deliveries were evaluated. An initial comparison was done for the presence of thrombocytopenia. Following that, an analysis of data was made for various possible risk factors. The data were analyzed with Fisher, Chi-Square test by SPSS 15. Results Out of the total number, 79 neonates (39.3%) were thrombocytopenic. Thrombocytopenic infants had a significantly lower average gestational age at delivery (32 ± 3.7 versus 34 ± 3.4), and lower birth weight (1888 ± 932 g. versus 2366 ± 820 g.). In this group, hemorrhage was more frequently observed to be intraventricular (37%). Use of some drugs including Heparin and occurrence of some pregnancy complications like pre-eclampsia were effective factors too. Conclusions Because of high frequency of thrombocytopenia in neonates, we have to use cautiously anti thrombotic agents in infants and cast attention on occurrence of intraventriculatr hemorrhage in these high risk newborns.

Background and ObjectivesThere is a large number of couples who are considered potential carriers of alpha or beta-thalassemia. The exact determination of gene defect for thalassemia carriers is essential for premarital screening genetic counseling. In this study, we conducted a molecular study of those suspected of carrying alpha-thalassemia mutated genes in order to detect potential deletional and non-deletional mutations in the alpha globin gene cluster. Materials and Methods In this study, those suspected of having mutation in alpha-globin gene cluster with MCV < 80 fl, MCH < 27 pg, normal serum iron, and HbA2 were selected from those referred to Pasteur Institute of Iran. Four common deletional mutations and non-deletional mutations were studied using multiplex gap-PCR, ARMS-PCR, and direct sequencing. ResultsOne hundred and forty samples with above criteria entered the study with 126 (90%) cases showing at least one mutation. Study of 4 common deletional mutations using multiplex gap-PCR revealed at least one deletion in 99 (70.71%) cases. Non-deletional mutations were found using ARMS-PCR or direct sequencing in 27 (19.28%) cases. Nine different mutations were found in the samples with –α3.7 being the most common deletion in 100 (35.71%) alleles out of 280 studied chromosomes followed by -α5nt in 25 (8.93%) alleles as the next most common. ConclusionsWe used direct sequencing to characterize more suspected carriers of alpha thalassemia. However, using other methods like real-time PCR and multiplex ligation-dependent probe amplification (MLPA) for gene dosage study of alpha-globin gene cluster could help find other non-common deletions.

Background and Objectives Thalassemia is a hereditary anemia requiring lifelong transfusion treatment with thromboembolism as one of its side effects. In the present study, we tried to determine the activity of anticoagulant proteins in thalassemic patients. Materials and Methods This descriptive study was conducted in Adult Thalassemia Clinic in Tehran. Measurement of protein C, S, and anti thrombin III levels were done. At the same time, we did these tests on 110 normal people who were matched by age and sex as the control group. The data were analyzed with Chi-Square, Pearson and T-test by SPSS 16. Results Out of 127 patients with the mean age of 29.4 year (SD ± 10.6), 114 (89.8%) were spelenectomized. Seven patients (5.5%) had history of thrombosis with 2 of them being diagnosed as major and 5 intermedia; all of them were spelenectomized. In 61.1%, 67%, and 22.5% of thalassemic patients, the levels of protein C, protein S, and anti thrombin III decreased, respectively. We did not observe any correlation between these results and the type of thalassemia except antithrombin III (p= 0.001). Conclusions In our study, we observed significant decreased activity of protein C, S, and antithrombin III in comparison to the normal population which shows that thalassemic patients are prone to the thromboembolic events.

Background and ObjectivesHelicobacter pylori (H.pylori) has recently been postulated to play a role in the pathogenesis of autoimmune diseases including immune thrombocytopenic purpura (ITP). In the present study, we looked for an association between H.pylori infection and chronicity of ITP in adults with acute ITP. Materials and MethodsTwelve patients with positive and 12 patients with negative urea breath test (UBT) for H. pylori infection aged 14 years and older with acute ITP and a platelet count below 100×103/μL were enrolled in this cohort study. All patients with platelet counts < 30×103/μL were treated with corticosteroid. After two weeks, platelet count started to be monitored at the baseline every month for 1 year. After 6 months, ITP patients with a platelet count recovery of greater than 100×103/μL were defined as ITP responders. The data were analyzed by Chi-square test and independent sample T-test with SPSS 11.5. ResultsThere were no significant differences between the mean age and sex of the infected and non-infected patients. At the baseline, the mean platelet counts of the infected and non-infected patients were 7.3 ± 5.3×103/μL and 6.4 ± 3.3×103/μL, respectively. No significant difference was seen between the mean platelet count of the infected and non-infected patients after 6 months. ConclusionsAlthough there was a greater rate of chronicity in the infected patients, it was not statistically significant. Therefore, we found no evidence of any association between H.pylori infection and chronicity of ITP. Further studies on larger number of patients with longer follow-up are recommended.

Background and ObjectivesHuman immunodeficiency virus (HIV) is the retrovirus that causes the disease known as AIDS. In the current study, we examined the genetic diversity of HIV-1 strains circulating in Iran. Materials and MethodsSequencing and phylogenetic analysis of human immunodeficiency virus type 1 (HIV-1) pol gene is a useful method for subtyping strains of HIV-1. HIV-1 RNA was derived from plasma of 24 infected patients in Imam Khomeini Hospital in Tehran. RT Nested-PCR was developed and the product was extracted from agarose gel. The products were sequenced and phylogenetic analysis by Mega 4 software was constructed. ResultsThe developed PCR method successfully amplified the specific sequence. The sequence and phylogenetic analysis showed subtypes A1 and B in 14 and 10 patients, respectively. ConclusionsThe results indicated intravenous drug users infected with HIV-1 subtype A1, and hemophiliacs infected with HIV-1 subtype B. The results also showed that the subtype A1 is the most prevalent in HIV infected patients. Such information can be useful in intervention strategies and control programs.

Background and ObjectivesClinical use of blood components seems to be inappropriate in Iran. The aim of this study was to evaluate blood utilization patterns in Rasht. Materials and MethodsThis was a cross-sectional descriptive study. Data about blood orders issued at day 20 of each month during the year 2007 were obtained from 5 hospitals. Data were analyzed by T-test with SPSS 15 descriptively for 491 cases. For all hospitals and wards, crossmatch/transfusion ratio (C/T) was calculated and compared separately. ResultsC/T=1/9 compared to the expected ratio was normal but significant differences were observed among different centers. The highest and lowest orders were from surgery (42.8%) and internal wards (7.1%), respectively. In surgical ward, the mean hemoglobin concentration of recipients was 9.03 ± 2.19 g/dl. ConclusionsPacked cell utilization in Rasht was normal but not ideal. In this study, blood utilization model in obstetrics and gynecology ward was appropriate. This may suggest an active and effective blood transfusion committee in hospitals. It seems that the lack of hospital blood transfusion committees with no experienced doctors attending, lack of executive support and external audit of hospital blood bank agenda, the lack of software integration between blood centers and hospitals, the lack of type and screening for patients, and importantly free of charge blood dispensation are major obstacles in reforming blood utilization patterns, running haemovigilance system, and raising quality.

Background and ObjectivesWeibel-Palade bodies (WPBs) are specific storage granules of vascular endothelial cells to which they are unique and for whose physiology they might play an important role. Materials and MethodsWe have reviewed the most recent literature published regarding the function of WPBs and their related proteins. ResultsDiscrimination of WPBs from lysosomes drew scientist's attention to their biological importance. Furthermore, localization of the important proteins such as von Willebrand factor (vWF), P-selectin, and Osteoprotegerin has confirmed the critical role played by these organelles. The content of WPBs can be very quickly available in case of vascular injuries suggesting that they could act as regulators of rapid vascular responses. Since the biogenesis of these granules depends on the presence of vWF, it would be interesting to know how the absence of vWF and WPBs would affect the function of other stored proteins. This situation which is present in von Willebrand Disease needs to be evaluated. ConclusionsThis review contributes to a better knowledge of WPBs biology and function. At the same time, further investigations have yet to be done to reveal the roles these organelles can play.

Background and ObjectivesExpression of strong IgM type Anti H in Bombay phenotype cases can lead to acute intravascular hemolytic reactions if they are transfused with ABO group O negative blood. CaseOur case had been recognized as blood group O with Negative Rh. In his recent donation, during antibody screening a high titers of anti A, anti B, and anti H were detected. In addition, there was no agglutination when donor's RBC and anti H were mixed. There were no Kell and CDE in the sample and the Rh genotype was recognized as cde/cde (rr). ConclusionsAccording to Oh phenotype reports, the number of Bombay phenotype is 6 in Esfahan and 15 in Iran. It seems that antibody screening should be further considered in Esfahan.