فصلنامه خون
سال دهم شماره 4 (پیاپی 42، زمستان 1392)

The time for platelet concentrate storage is 3 to 5 days. One of the limitations for platelet storage was storage lesion in which they lose their viability and undergo apoptosis. Studies show that apoptosis occurs in some anucleated cells such as platelets. In this study، the caspase 3 inhibitor was employed in platelet concentrates to obtain platelets with higher survival. After preparation of platelet concentrates، caspase3 inhibitor (16 μM) was injected to the test bags. These bags along with control bags which lacked the inhibitor were stored at 22 in shaker incubator for 7 days. Samples from these bags were obtained and the activity of caspase3 and the survival levels of platelets were analyzed. In the presence of caspase3 inhibitor، the activity of caspase3 in platelets decreased compared to the control group. Additionally، this study showed that the survival of platelets decreased during storage but in the presence of caspase3 inhibitor the survival of platelet concentrate was significantly higher than that of the control group (p<0. 05). Injection of caspase3 inhibitor to the platelet concentrate can reduce apoptosis and therefore increase their survival.

Acute lymphoblastic leukemia (ALL) is the most common cancer among children. Multi drug resistance (MDR) is the major cause of treatment failure and relapse in ALL. ABC transporters mainly contribute to this disorder and MDR1 gene is the most popular member of this family. The aim of this study is to evaluate the gene expression profile of MDR1 at mRNA level and its prognostic value in ALL. Bone marrow and peripheral blood samples were obtained from 28 newly diagnosed ALL patients and 15 controls. MDR1 gene expression profile was evaluated by using real time PCR. The minimal residual disease (1-year MRD) was considered as the factor of response to therapy. Data were analyzed by using SPSS 20 and Graph Pad Prism 5 softwares. MDR1 gene expression level in patients with MRD+ was significantly higher than MRD- patients (2. 29 ± 1. 22 vs 0. 79 ± 0. 21) (mean ± SEM، p= 0. 049). Most of the patients with over expression of MDR1 gene were MRD+ (66. 6% vs 33. 3) (mean ± SEM، p= 0. 048). MDR1 gene expression profile is suggested to be an unfavorable prognostic factor in ALL. The evaluation of the mRNA expression profile of MDR1 in new case patients facilitates identification of the high risk patients and helps modifying the protocols applied for ALL treatment in order to improve the therapeutic outcome.

Since stimulated telomerase activity provides nearly all of human malignancies including acute promyelocytic leukemia (APL) with unlimited proliferative potential، targeting telomerase seems to be an effective approach in cancer treatment. In this regard، BIBR1532، a small-molecule inhibitor of telomerase، has been shown to increase the therapeutic window of current chemotherapeutic regimens. This study was aimed to investigate the effects of BIBR1532 on cell proliferation as well as transcriptional alteration of hTERT (the catalytic subunit of telomerase). NB4 leukemic cells were treated with various concentrations of BIBR1532 and succeeding trypan blue exclusion assay، BrdU cell proliferation assay، and quantitative real-time PCR were applied to investigate cell viability index، cell proliferation and time-dependent alteration of hTERT mRNA levels. BIBR1532 decreased cell viability index and exerted an antiproliferative effect against NB4 leukemic cells; we found that exposing cells with BIBR1532 at 30، 60 and 90 μM for 72 h inhibited DNA synthesis rate by 24، 45 and 70%، respectively. Furthermore، transcriptional suppression of hTERT was found upon NB4 treatment by BIBR1532 in a time- and dose-dependent manner. Based on the short telomere length and high terlomerase activity in APL as well as antiproliferative effect of BIBR1532 against NB4 cells، anti-telomerase-based therapy might be regarded as a successful strategy in APL therapy.

Selection of healthy blood donors is an important process to identify high risk volunteers. Monitoring and evaluation of the deferral procedure is of the highest importance to balance blood availability and safety. The aim of the current study was to compare the prevalence of HBV، HCV and HIV serologic markers between deferred donors and eligible blood donors to assess the value of the current donor selection process. Six hundred and nineteen voluntary blood donors in Ahvaz city were included. Serum samples from deferred and eligible donors were examined for serologic markers of HBV، HCV، and HIV using enzyme-linked immunosorbent assay. All positive samples were confirmed using confirmatory tests. The prevalence rates of HBV infection among deferred and eligible blood donors were estimated to be 0. 5% and 0. 2%، respectively. Anti-HCV reactivity was detected among 1. 3% of deferred blood donors. Moreover، the prevalence of anti-HIV among deferred and eligible blood donors was 0. 2% and 0. 05%، respectively. We found that the overall prevalence rates of HBsAg، anti-HCV، and anti-HIV in the deferred blood donors are significantly higher than those in the eligible blood donors. The results of this study indicate the high prevalence rate of blood borne infections. Moreover، it seems that the current donor selection process is effective to identify healthy blood donors. However، the effectiveness of some of the currently used deferral questions in reducing viral risks warrants further study.

Hematopoietic stem cells (HSCs) have become a standard protocol for the treatment of many hematologic malignancies and non-malignant disorders. Umbilical cord blood، as a source of HSCs، has many advantages compared to other sources. One major drawback in using this source in transplantation is the low HSC dose available. Ex vivo expansion of HSCs is a solution to overcome this limitation. In this study، we used TEPA، as a Cu chelator، and human bone marrow MSCs to investigate the expansion rate of UCB-HSCs. CB-HSCs were isolated using miniMACS magnetic separation system. We cultured the enriched CD34+ cells in various conditions. Culture condition A، supplemented only with recombinant cytokines; culture condition B، supplemented with BM-MSCs as a cell feeder layer and recombinant cytokines; culture condition C، supplemented with recombinant cytokines and TEPA; culture condition D، supplemented with recombinant cytokines، BM-MSCs as a cell feeder layer and TEPA. In order to evaluate the HSC expansion، we performed cell count، the analysis of CD34+ expression by flow cytometery، and CFU assay on day 10 after culture. The most fold increase rate in CD34+ cell، TNCs and CFU-C was observed in the culture condition D (110. 11 ± 15. 3، 118. 5 ± 21 and 172. 9 ± 44. 7، respectively) compared to other conditions. The results showed that co-culture of HSCs with BM-MSCs in the presence of copper chelating agent (TEPA) could dramatically increase the expansion rate of UCB-HSCs. Therefore، this strategy could be useful for HSC expansion.

Parvovirus B19 may cause a number of clinical illnesses including infectious erythem، arthropathy hydropes fetalis or congenital anemia، and transient aplastic crises. The virus is very infectious and is transmitted via respiratory tract، and blood transfusion. The aim of this study was to detect B19 virus in persons who suffer from blood disorders. Parvovirus B19 infection was compared in patients suffering from blood disorders with healthy people in Isfahan، Iran. Sera were collected from 35 and 30 patients with the history of chronic anemia and lukemia/lymphoma. Thirty persons were considered as control group without any blood complications. DNAs of all sera were extracted and tested for presence of B19 DNA using semi-nested PCR. B19 DNA was detected in 15 samples (23. 1%) out of 65 patient sera. The infection rate was 17. 1% and 30% among benign and malignant blood disorder groups، respectively. None of healthy people showed positive results. Results are consistent with previous studies indicating that parvovirus B19 infection in high-risk individuals is higher including patients with hematologic disorders. Blood donor screening for parvovirus B19 in high risk patients is necessary and can effectively reduce the incidence and prevalence of B19 infection.

Hemophilia is a hereditary bleeding disorder associated with multiple complications. Adolescents are a wide population of hemophilia patients. The appropriate management of chronic diseases requires an adequate knowledge about their complications. The aim of the present study is to assess the knowledge of hemophilia affected adolescents on the prevention of its complications. In this descriptive study، the adolescents affected with hemophilia were selected from 11 hemophilia centers during 9 - month period between 2011-2012 by the available sampling method. For data collection، a questionnaire developed by researchers as the self report method was used. Data were analyzed using SPSS (version 13). From 108 participating hemophilic adolescents، 35. 3% were not aware of hemophilia complications، 43. 7% of the preventive methods of hemophilia complications، and 47% of the causes of the complications. Out of the total number، 69. 5% had not received any information about the prevention of complications. In the assessment of the adolescents'' knowledge of hemophilia complications، 67. 6% were not aware of viral infections، 56. 1% of threatening bleeding، 60. 6% of the complications of dentistry procedures، 68. 2% of threatening drugs، and 22. 4% of threatening exercises. As far as the other aspects of the prevention of complications are concerned، the adolescents were either unaware or modestly aware. There is a need to promote the preventive knowledge of complications among hemophilia adolescents. Faced with the scarcity of resources، it is the education that plays an important role in organizing hemophilia services in developing countries.

Beta-thalassemia major is a chronic، hematological disorder with psychological adverse effects on patients and their families. Parents، in particular mothers who are mostly the primary caregiver، are the ones to deal with the stress of their kid thalassemics and use different coping styles either adaptive or maladaptive. The aim of this study is to assess the relationship of coping strategies of mothers with the mental health of their thalassemic children. In this cross-sectional study، out of the patients referring to the Thalassemic Clinic of Ahwaz Shafa Hospital، 36 patients with thalassemia major (12-16 y/o) and their mothers were selected by the convenient sampling method. The subjects were assessed using the demographic data questionnaire، the coping styles questionnaire (CSQ) (for mothers)، and SCL90-R questionnaire (for children). The data were finally analyzed by the spss software. Out of the total number of the children with thalassemia major، 91. 2% suffered from psychosocial problems. Among the mothers، 80. 55% more frequently used the adaptive coping styles; a significant positive correlation was found between mothers’ maladaptive coping mechanism and psychological problems of their children (p= 0. 33) (r= 0. 356). Educational planning aimed at increasing the coping skills in mothers with thalassemic children can be useful in decreasing psychological problems of children.

The severest form of thalassemia is beta thalassemia with regular need for blood transfusion and medical care. Treatments such as desferrioxamine injection and regular blood transfusion cause several complications in patients'' vital organs including eyes. The vision is considerably more sensitive than other senses in perceptual-motor responses and it is also sensitive to details، depth، and color. As a result، it has a decisive role in controlling movement. This study has been conducted to draw a comparison of depth perception in healthy adolescents and patients with thalassemia major. This method is semi-experimental and the sample was 18 thalassemic patients and 18 healthy subjects within the age range of 13-17 years (15. 33 ± 1. 37) who were selected through sampling. These subjects have been evaluated three times by the depth perception device made by the researchers; it has the capability to assess the depth perception with the smallest error (one ten thousandth cm). The average of these three time tests was used as depth perception score. Independent T-test results showed significant difference between healthy adolescents and patients with thalassemia major (t=-18. 52، p≤ 0. 0001، df = 34). The average error in the healthy adolescents was significantly lower (MD = 14. 01). The probable reasons that affect the loss of depth perception in thalassemia major were anemia، chronic hypoxia، inactivity or increased iron deposition in tissues of the eye. It requires a solution in this regard due to the importance of depth perception in human motor behavior.

For people with Bombay phenotype due to the expression of strong IgM Anti H in their sera، transfusion with ABO group O blood will cause acute hemolytic reactions. Therefore، it is important to detect them. Case: Following O blood type transfusion to a blood group O Rh positive patient with multiple trauma، acute hemolytic transfusion reaction happened. The reaction was recognized by the trained nurse in the haemovigilance system. In serologic studies، a high level of anti-H، anti-A، and anti-B was detected in the patient blood. This case increased the number of Bombay phenotypes in Iran to 16. Therefore، using antibody screening test and existence of trained nurses and physicians with haemovigilance system in order exact management of transfusion reactions should be considered necessary.

Using suitable laboratory methods for the diagnosis of hemoglobinopathies in laboratories not equipped with automatic systems is essential. The correct diagnosis of hemoglobin Q-Iran (α75Asp → His) is important. Case: A 33-year-old woman was referred to the national reference globin chain biosynthesis laboratory. The patient''s globin chains chromatogram indicated an unknown peak after α globin chain peak. Direct conventional sequencing revealed single G to C missense mutation in the -globin gene. The genetic analysis led to the identification of a rare hemoglobin variant. Medical diagnosis laboratories not equipped with modern automatic systems must run solubility test and hemoglobin electrophoresis on citrate agar and cellulose acetate for definite detection of Hb Q-Iran; otherwise، it can be regarded as a cause of misdiagnosis with hemoglobin S.

Clinical use of blood components seems to be inappropriate in Iran. The application of excessive blood in elective surgeries is the common problem leading to escalating costs and increased workload in blood banks. The aim of this study was to evaluate blood utilization patterns in Birjand Imam Reza Hospital. This was a cross-sectional descriptive study done on hospitalized patients. In this study، the amount of blood required for storage، the number of cross-matched units، and the number of transfusion units were evaluated. The data were analyzed by SPSS software. Then، crossmatch/transfusion ratio (C/T) and transfusion probability (T%) for all patients and wards were calculated and compared. The mean age of patients was 23. 4 ± 40. 3. In this study، 857 patients were evaluated. The total cross-match to transfusion ratio (C/T ratio) in our study was 4. 2 and transfusion probability (T%) was 20. In this study، the blood utilization model in ICU and burn wards was evaluated to be appropriate. But the high total C/T ratio 4. 2 and very low utilization percentage of cross-matched units (only 23. 4%) showed that the change in blood ordering pattern in this hospital is necessary.