Background: Irradiation leads to increased storage lesions that may have harmful effects if transfused. Various storage lesions research has been carried out, and only very few articles are available on the impact of gamma irradiation on RBC storage lesions. Since there has been no study about finding the best time for irradiation, we decided to investigate the effect of irradiation on Red blood cells at different storage times after blood collection

Materials and Methods: A total of 40 units of red blood cells divided into two groups: irradiated and non-irradiated. Irradiated RBCs were divided into three groups, each containing ten units. The remaining ten units were considered as non-irradiated controls. Sampling from these irradiated and non-irradiated blood units was performed weekly to evaluate biochemical parameters and free plasma hemoglobin/Hemolysis index levels.

Results: A significant increase in the mean values of plasma potassium, plasma Hb/Hemolysis index, and LDH, as well as a significant reduction in the mean value of 2,3 DPG and plasma sodium, were observed in both groups. Although the reduction of 2,3 DPG is extremely remarkable, it is compensated 24-48 hours after transfusion. Hence, the clinical result of 2,3-DPG-depleted RBC transfusion is known to be negligible. The irradiation group alteration was more notable than the non-irradiated one and the changes in the parameters were most significant in the group that was stored for a longer period after irradiation.

Conclusion: The investigation on the impact of gamma irradiation on RBCs makes it possible to suggest a storage time up to 28 days after irradiation and the best time for irradiation after blood collection is up to 14 days. It is pointed out that the blood unit should be transfused as soon as possible after the irradiation

Background: Although the precise pathogenesis of acute lymphoblastic leukemia (ALL) remains unclear, studying gene-regulating mechanisms during ALL pathogeneses may shed light on the underlying mechanisms driving malignant behavior. There is some evidence showing the promoter hypermethylation and silencing of RASSF1A tumor suppressor gene in ALL cells; however, there is a lack of evidence for whether the gene indeed alters during different phases of ALL or in response to therapy. Thus, the current study aimed to clarify this issue using groups of adult ALL patients who have been scarcely investigated regarding expression levels and promoter methylation status.

Materials and Methods: In this case/control study, the expression levels and methylation status of the gene promoter was evaluated using quantitative real-time PCR and methylation-specific PCR (MSP), respectively in adults with ALL. The study included peripheral blood of patients with newly diagnosed ALL (n=10), complete remission (CR) (n=10), or relapse (n=10), and 10 control samples from healthy individuals.

Results: MSP results revealed an unmethylated status for almost all patients and control samples, except a case with relapsing ALL, which showed a hemimethylated pattern. RASSF1A also showed no difference in terms of gene expression in the patients compared with the control group (p>0.05).

Conclusion: The results revealed an up-regulation of RASSF1A tumor suppressor in adult ALL patients experiencing CR, suggesting this to be a marker of therapy response. However, further investigations using more sensitive methylation detecting tools with larger sample sizes may better clarify the involvement of the promoter methylation of RASSF1A in these patients. 

Background: A number of mutations have been reported to occur in patients with acute myeloid leukemia (AML), of which NPM1 and FLT3 gene mutations are the commonest and have important diagnostic and therapeutic implications, respectively.

Material and Methods: Molecular testing for NPM1 and FLT3 genes was performed in 92 de-novo AML patients. The frequency and characteristics of NPM1 and FLT3 mutations were analyzed.

Results: Nucleophosmin 1(NPM1) and FMS-like tyrosine kinase 3 (FLT3) mutations were seen in 22.8% and 16.3% of patients, respectively. Amongst FLT3 mutations, FLT3-ITD mutation was seen in 8.7% cases, FLT3-TKD in 5.4%, and FLT3-ITD+TKD in 2.2% cases. Certain associations between the gene mutations and clinical characteristics were found, including in NPM1 mutated group- female preponderance, the higher incidence in M4/M5 categories and decreased expression of CD34 and HLA-DR; and in FLT3-ITD mutated group- higher age of presentation, higher total leucocyte count and blast percentage.

Conclusion- AML patients with NPM1 and FLT3 mutations have differences in clinical and hematological features, which might represent their different molecular mechanisms in leukemogenesis. The frequency of NPM1 and FLT3 mutations in this study was comparable to reports from Asian countries but lower than that reported from western countries. However, as the number of patients in the study was less, a larger number of patients need to be studied to corroborate these findings

Background: Thalassemia is a chronic disease requiring lifelong treatment. The adherence to regular iron chelation therapy is important to ensure complication-free survival and good quality of life.

The study aim to assess the adherence to iron chelation therapy (ICT) in patients with transfusion-dependent thalassemia (TDT), evaluate various causes of non-adherence and study the impact of non-adherence on the prevalence of complications secondary to iron overload.

Materials and Methods: Patients with TDT on ICT for > 6 months were enrolled in the study. Hospital records were reviewed for demographic details, iron overload status, treatment details, and the prevalence of complications. A study questionnaire was used to collect information on adherence to ICT, knowledge of patients, and the possible reasons for non-adherence.  

Results:  A total of 215 patients with a mean age of 15.07+7.68 years and an M: F ratio of 2.2:1 were included in the study. Non-adherence to ICT was found in 10.7% of patients. Serum ferritin levels were significantly higher in the non-adherent group (3129.8+1573.2 µg/l) than the adherent population (2013.1+1277.1 µg/l). Cardiac as well as severe liver iron overload was higher in the non-adherent patients. No correlation was found between disease knowledge and adherence to ICT. Difficulties in drug administration and many medicines to be taken daily were statistically significant reasons for non-adherence. There was no difference in the co-morbidities arising due to the iron overload in the two groups.

Conclusion: Nearly 11% of patients with TDT were non-adherent to ICT. Non-adherence results in higher iron overload. 

Background: Acute lymphoblastic leukemia is a disease of the hematopoietic system and chemotherapy is recommended as the primary treatment.  As many chemotherapeutic agents have severe adverse effects, patients require to be supported by their family to deal with chemotherapy-related symptoms. This study attempted to investigate the effect of a family-centered supportive program on chemotherapy symptom control in patients with acute lymphoblastic leukemia.

Materials and Methods: Sixty-six patients with acute lymphoblastic leukemia undergoing chemotherapy along with their caregivers participated in this nonrandomized clinical trial. Patients in Shariati and Taleghani Hospital were assigned to the intervention (n=33) and control group (n=33), respectively. A survey of the family-centered supportive program was conducted via in-person and telephone up to 6 cycles of chemotherapy. The chemotherapy symptom assessment scale was administered to record the data during six cycles of chemotherapy treatment. The control group only received routine interventions. Data were analyzed using Chi-square and Mann–Whitney U tests.

Results: The results of the study indicated that there was a statistically significant difference in terms of the frequency of 9 chemotherapy-induced symptoms including nausea, shortness of breath, problems with the skin and nails, a sore/sensitive mouth or throat, anorexia, weight gain or loss, headache and sore/scratchy/dry eyes between the control and intervention group. There was also a statistically significant difference in the severity and level of discomfort of 19 chemotherapy-induced symptoms between the control and intervention group.

Conclusion: Family-centered supportive program can be considered as an approach to decrease the frequency, severity and discomfort level of chemotherapy-induced symptoms.

Background: Finding a suitable donor at the optimal time is one of the most challenging issues in many transplant centers. We evaluated the clinical outcomes of 248 patients with acute leukemia and without matched sibling donors (MSD) who underwent alternative transplantation, including haploidentical (n=118), 10/10 matched unrelated (MUD, n=91), 9/10 mismatched unrelated (MMUD, n=21), and 9/10 mismatched related (MMRD, n=18) between January 2010 and November 2019 in our center.

Materials and Methods: The myeloablative conditioning regimen was used in most of the patients. Both post-transplant cyclophosphamide (40mg/kg at +3, +4) and pre-transplant ATG were used in most of Haploidentical transplantations. Patients with unrelated donors received ATG as a part of the conditioning regimen.

Results: The median follow-up was 31.83 months. No significant difference in probability of 3-year leukemia- free survival (LFS) and overall survival (OS) as well as 3-year relapse incidence (RI) were noted between donor sources.

A significant difference was found in the 3-year cumulative incidence (CI) of non-relapse mortality (NRM) among the donor sources: 37.89%, 24.20%, 24.30%, and 11.48%, for Haplo, 9/10 MMUD, 10/10 MUD, and 9/10 MMRD (p=0.02). Using the multivariable Cox model, the advanced age of patients and Major-ABO mismatched, were two risk factors independently associated with lower OS and DFS as well as higher NRM, whereas male donor and AML disease compared to ALL were associated with a better OS and DFS.

Conclusion: Given that no significant differences were observed in the overall outcome of Haplo with other alternative transplantations, suggesting that Haploidentical transplantation is a suitable, accessible, and inexpensive option.

Background: Transplant success largely depends on the number of hematopoietic stem cells. The release of catecholamines following exercise can, as a treatment in addition to medication, affect the mobilization of stem cells from the bone marrow into the peripheral blood. The aim of the present study is to compare two types of aerobic exercise on stem cell mobilization before autologous transplantation.

Materials and Methods: In a quasi-experimental applied study, 60 patients in the age range of 22-69 years referred to Taleghani Hospital were randomly selected and assigned into 3 groups of 20 members (continuous aerobic, discontinuous aerobic and control group). Aerobic exercise program was performed for 7 consecutive days of mobilization period including walking on a treadmill (according to the patient's ability) continuously and discontinuously for 30 minutes in the morning and afternoon. Blood samples were taken the morning before and after mobilization and the CD34 and MNC levels were counted as absolute. Chi-square test, paired t-test, analysis of covariance (ANCOA) and multiple comparison test were used for statistical analysis. All analyses were considered significant at p ≤ 0.

Results: Moderate-intensity continuous and discontinuous aerobic activity increases the number of CD34 and MNC cells. A comparison between continuous and discontinuous aerobic activity showed an increase in the amount of these cells. The continuous aerobic activity group was found to have a statistically significant increase compared to the discontinuous group (P≤0.05).

Conclusion: Moderate intensity continuous and discontinuous aerobic exercise significantly increased hematopoietic stem cells. However, this increase was greater as a result of continuous aerobic exercise than discontinuous exercise. Regarding the potential role of these cells in transplantation, they could possibly help the transplant process.

Endothelial injury by toxins, drugs, immune complexes leads to activation of coagulation cascade and thrombosis, which result in platelet consumption and red blood cell injury. These thrombotic microangiopathies can potentially injure numerous organs and result in organ dysfunction. In this case, we present the fourth reported patient with thrombotic thrombocytopenic purpura associated with COVID-19.