Iranian Journal of Blood and Cancer
Volume:7 Issue: 3, Spring 2015

Treatment of severe Immune Thrombocytopenic purpura (ITP) accompanied by life-threatening bleeding events is challenging and a combination of treatment measures should be undertaken to rapidly increase the platelet count. Herein, we report a case of severe ITP in a seven-year-old boy who suffered from massive bleeding which was totally unresponsive to conventional therapeutic interventions. Since the patient was still symptomatic with severe thrombocytopenia after splenectomy, the patient underwent plasma exchange (PE) which was successful. To best of our knowledge this is the first case of severe ITP in children managed with plasma exchange due to unresponsiveness to standard treatments and splenectomy.

Beta thalassemia gene mutations are among common mutations in southwest Iran. However, Hemoglobin E (Hb E) and Hb E/β⁰ thalassemia account for a small number of hemoglobinopathies in Iran. This is the first study to directly address the existence of Hb E and consequently Hb E/β⁰ thalassemia in southwest Iran. This retrospective study discovered seven cases of Hb E/β⁰ thalassemia among 700 patients with hemoglobinopathies referring to Health Institute and Research Center for Thalassemia and Hemoglobinopathy in southwest Iran. EDTA and clot blood samples were obtained and analyzed for complete blood counts, hemoglobin electrophoresis, LDH, bilirubin, ferritin and amplification refractory mutation system (ARMS) technique by polymerase chain reaction (PCR) and DNA sequencing. Out of 700 cases, seven patients with Hb E/β⁰ thalassemia were detected (1%). Four patients were classified into non-transfused dependent Hb E/β⁰ thalassemia and three cases were classified into transfusion dependent Hb E/β⁰ thalassemia group. Alpha thalassemia (deletional and non-deletional) and XmnI gene polymorphism were not found in either of cases. Hb E/β Thalassemia is not a common hemoglobin disorder in southwest Iran. Phenotype heterogeneity is common in Iranian patients from a mild asymptomatic anemia to severe anemia that can be presented in the early years of life. This was the first report of Hb E/β⁰ thalassemia from Iran.

Preparations of platelet concentrates (PCs) that are stored under blood bank conditions and used for transfusion purposes, appear to be enriched in platelet derived-microparticles (PMPs) with high coagulant activity that may change platelet efficacy and safety issues. High shear stress could cause shedding of PMPs from the platelet plasma membrane, platelet aggregation, and activation of the coagulation cascade by increasing the catalytic phospholipid surface. These stresses may be prompted by processing and storage of blood and platelet rich plasma through various variables that has been fully described in this review. Depending on different rates of shear stress during processing and storage of PC, different quantities of MPs might be shed from platelets. On the other hand, the therapeutic effect of high levels of PMPs in PC has been reported for some patients. By using more sensitive and standardized methods for PMP measurement and change of platelet preparation process, further studies are required to monitor PMP generation during blood collection, processing and storage of PC to improve quality of PC and also in recipient’s reactions to transfusion.

Acute lymphoblastic leukemia (ALL) is the most common form of childhood cancer leading to cancer-related death in children. Most infants with ALL harbor recurring structural chromosomal rearrangements that are important initiating events in leukemogenesis but are insufficient to explain the biology and heterogeneity of the disease. Mixed-lineage leukemia-rearrangement (MLL-rearrangement) at 11q23 occurs in at least two-thirds of infants with ALL. The most common MLL rearrangements are t(4;11)(q21;q23)/MLL-AFF1 (AF4) found in approximately 50% of patients. Forty children with ALL were enrolled in our study. 11q23 rearrangement and its association with other prognostic factors such as age, sex, initial WBC, organomegaly, immunophenotype, and therapeutic response on the seventh day of induction were studied. Four patients including three (11.5%) boys and one (7.1%) girl were positive for 11q23 translocation. There was no association between 11q23 rearrangement and sex, age, and initial WBC counts. None of the patients with 11q23 translocation showed blast count less than 5% in the bone marrow on the seventh day of induction (P=0.002). There was a significant correlation between 11q23 translocation with lack of initial response to chemotherapy.

Retinoblastoma is the most common embryonic tumor of retina of children. This tumor include 3-4% of all children malignancies during the age range 0-14 years. In this study, we reported the incidence, therapeutic protocols, and treatment outcome of children with RB in South west Iran. We retrospectively investigated the medical reports clinical and pathological features of RB cases at referral Shafa Hospital of Ahvaz city located in Southwest Iran, between 1994 and 2014. Our results showed leukocoria, white discoloration in the pupil, as the most common primary symptom in retinoblastoma patients. Forty-two percent of our patients had bilateral neoplasms. Based on histological investigations, stage IV was most common stage in our patients (79.1%). In conclusion, our study showed that leukocoria, stage IV and Choroid involvement as most common symptom, stage and organ involvement, respectively and inherited cases is rare in our population. The results of our study can be as a base for further researches in future.

Hemophilia is a chronic bleeding disease and can interfere with daily performance of children. These children require continuous training to prevent bleeding. Since children nurses play an important role in the education of sick children and their self-efficacy and the increase use of educational computer games, the use of educational games for teaching self-efficacy to children with hemophilia can be effective. Therefore, we aimed to explore the impact of computer-based learning games on the self-efficacy of 8-12 year-old children with hemophilia. The present study is an experimental interventional study on 40 children with hemophilia aged 8-12 years old during 2014. Data collection tools were a standard self-efficacy questionnaire and patients’ demographic forms. Data were analyzed using SPSS software version 16. Based on the findings of this study, the mean self-efficacy scores of the samples before, after and one month after intervention were 53.25, 60.95, 60.90 in the case group, and 50.45 and 55.2 in the control group before and one month after the intervention, respectively. The findings revealed that self-efficacy scores after and before the intervention differed significantly. Educational hemo-action games increased self-efficacy in 8-12 year-old children with hemophilia.

Mesenchymal stem cells (MSCs) are ideal cells for cell and gene therapy. However, the low survival of MSCs after transplantation has limited their applications. We aimed to evaluate the expression of cytoprotective genes including NQO1, TXNRD1, HO-1, GCLC following the over expression of Nrf2 in umbilical cord-derived MSCs (UC-MSCs). 3-5 passages of UC-MSCs were cultured. Recombinant vector containing Nrf2 (pcDNA3.1-Nrf2) and empty vector were transfected into MSCs using FuGENE HD. After exposing the cells to UV light stress, RNA extraction and cDNA generation were performed. Using Primer3, software specific primers were designed for Nrf2, NQO1, TXNRD1, HO-1, and GCLC genes and the expression of these genes was evaluated by RT-PCR. The results were semi-quantified and analyzed statistically utilizing Image J2x software and ANOVA test. The expression of Nrf2 was up-regulated in UC-MSCs after transfection with pcDNA3.1-Nrf2 (P<0.01). Over expression of TXNRD1 and GCLC were also confirmed in these transfected cells (P<0.05 and P<0.01, respectively). However, expression of NQO1 and HO-1 did not alter in the transfected cells with pcDNA3.1-Nrf2 compared with those transfected with empty vector (P>0.05). Over expression of Nrf2 resulted in the over expression of TXNRD1 and GCLC in MSCs and might be explained by the fact that a known part of Nrf2 cytoprotective mechanisms is controlled by the expression of these genes.