Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 17 1 2023 01 16 4 8 Farzad Mollahoseini Foomani Blood Transfusion Organization Research Center, Iranian Blood Transfusion Organization, Mashhad, Iran Mahammad Hadi Sadeghain Cancer Molecular Pathology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Saeede Bagheri Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran Zahra Badiee Pediatrics Department, Doctor Sheikh Hospital, Mashhad University of Medical Sciences, Mashhad, Iran Reihane Bazargani Blood Transfusion Organization Research Center, Iranian Blood Transfusion Organization, Mashhad, Iran Zahra Aryanpour Blood Transfusion Organization Research Center, Iranian Blood Transfusion Organization, Mashhad, Iran Saeid Hallajian Blood Transfusion Organization Research Center, Iranian Blood Transfusion Organization, Mashhad, Iran Seyyede Fatemeh Shams Blood Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran 2020 10 31 2021 05 01 Background:  Thalassemia is an inherited disease with anemia and hemolysis. Blood transfusion is a routine treatment for thalassemia patients; alloimmunization is one of the complications of blood transfusion, which is very serious for these patients, especially girls and young women.    Materials and Methods:  Four hundred forty-six thalassemia patients were examined in this cross-sectional study. Demographic information of patients was extracted and recorded. The phenotype of ABO, Rh, and Kell antigens (tube method) with antisera from IMMUNDIANOSTICA Company (Germany) and the frequency of alloantibodies were determined. Results: 55.8% of the studied individuals were male and 44.2% were female. The mean age of the studied patients was 19.94±10.63.  The alloantibodies were detected in 7.5% of pack cell receivers. The most prevalent phenotype of the ABO system was the O blood group (37.4%), and the most abundant antigen of the Rh group was ‘e’, which was found in 99.8% of the studied population. The most frequently detected alloantibody was Anti K (38.2%); concerning kell phenotype, (K_k+) and (K+k+) were found in 99.3% and 0.7% of patients, respectively. The frequency of Anti-D, Anti-C, Anti-c, and Anti-E was 23.5%, 14.7%, 2.9%, and 14.7%, respectively. Conclusion: According to the results of this paper, finding the compatible pack cells in terms of Kell and Rh systems antigens in addition to the ABO blood group is recommended to decrease the rate of alloantibodies in thalassemia patients. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1453 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1453/957

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 17 1 2023 01 16 1 3 Ali Akbar Pourfathollah Department of Immunology, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran Karim Shamsasenjan Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran Mahmoud Hadipour Dehshal Mehregan Pharmacy, Tehran, Iran 2022 08 22 2023 01 07 No Abstracts  No Abstracts  No Abstracts  https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1899 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1899/956

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 17 1 2023 01 16 56 62 Rachel Hicks Brookdale University Hospital Medical Center Mohammed Alsabri Brookdale University Hospital Medical Center Mario Peichev Brookdale University Hospital Medical Center Viswanathan Kusum 2019 11 04 2020 09 22 Abstract Congenital factor XIII (FXIII) deficiency is an extremely rare bleeding disorder (RBD) with estimated prevalence of one per 2 million in the general population. The disorder causes different clinical manifestations such as intracranial hemorrhage (ICH), recurrent miscarriage, umbilical cord bleeding, etc. High incidence of the disorder might be due to founder effect. To assess founder effect, haplotype analysis is an important step. For this purpose, suitable and reliable genetic markers such as microsatellites (Hum FXIII01 and HumFXIIIA02) and single nucleotide polymorphisms (SNP) are suggested. In the present study we tried to describe evaluation of founder effect in patients with congenital FXIII deficiency via haplotype analysis using suitable genetic markers. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1206 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1206/868

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 226 231 Rony Benson Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram 695011, India Sreejith Nair Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram 695011, India Geetha Narayanan Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram 695011, India 2019 10 25 2020 05 04 Background: The half-life of free light chain is short and can be used as an early marker for tumor response in patients with multiple myeloma [MM]. This prospective study is aimed at evaluating whether early light chain response can predict response to treatment in patients with MM. Materials and Methods: Thirty six patients with a diagnosis of MM and with an abnormal to normal light chain ratio of > 10 were included in this study. Results: The median age at presentation was 56 years. Fourteen patients had lambda light chain disease, whereas 22 patients had kappa light chain disease. Twenty-four patients [66.6%] had reduction of abnormal to normal light chain ratio to < 10 after 2 cycles, of whom 15 [62.5%] achieved a CR or VGPR after 6 cycles. Among 12 patients who did not have reduction of abnormal to normal light chain ratio to < 10, only 1 patient achieved CR while 11 patients [91.6%] achieved a PR or less[Fishers exact p=0.004]. Median follow-up was 13 months. Median progression-free survival for the entire cohort was 15 months. One-year Progression-Free Survival was 77% vs 57.1%, [p= 0.008], respectively for patients with early normalization and those who did not show early normalization. Conclusion: Early light chain response after 2 cycles of chemotherapy is a good predictor for treatment response in patients with MM treated with bortezomib based chemotherapy. Treatment intensification based on early light chain response merits further evaluation in a prospective trial https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1199 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1199/863

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 274 288 Lucio Henrique Sousa Pinheiro Department of Pharmacy, Laboratory of Hematology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil Louise Trindade Department of Pharmacy, Laboratory of Hematology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil Fernandes de Oliveira Amanda Costa Department of Medicine, University of São Paulo, Ribeirão Preto, São Paulo, Brazil Nathanielly de Lima Silva Department of Pharmacy, Laboratory of Hematology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil Alex Freire Sandes Department of Medicine, Hematology Course, Federal University of São Paulo, São Paulo, São Paulo, Brazil Marco Antônio Prado Nunes Department of Medicine, Federal University of Sergipe, Aracaju, Sergipe, Brazil Cristiane Bani Correa Department of Morphology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil Carlos Arthur Cardoso Almeida Nursing and Pharmacy School, Federal University of Alagoas, Maceió, Alagoas, Brazil Geydson Silveira da Cruz Hematologist, University Hospital, Federal University of Sergipe, Aracaju, Sergipe, Brazil Divaldo Pereira de Lyra Júnior Department of Pharmacy, Laboratory of Hematology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil Dulce Marta Shimieguel Department of Pharmacy, Laboratory of Hematology, Federal University of Sergipe, São Cristóvão, Sergipe, Brazil 2019 10 14 2020 03 01 Background: The aim of this review was to evaluate the influence of aberrant phenotypes in prognosis and survival in acute myeloid leukemia (AML) patients by multiparametric flow cytometry. Materials and Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a review of PubMed, Scopus, Science Direct and Web of Science was carried out through 1998 to 2016, conducted by two reviewers independently, evaluating titles, abstracts and full-texts of the selected studies. Results: Ten studies were included on this review, in which the aberrant phenotype expression of 17 markers were detected in AML patients. From these, 11 aberrant phenotypes were associated with prognosis, which eight had shown negative impact on prognosis: CD7, CD56, CD15, CD2, CD3, CD90low, CD123high, CD117high, and three others were associated with good prognosis: CD19, CD98high and CD117+/CD15+. Meta-analysis showed that aberrant expression of CD56 as a poor prognostic marker with unfavorable outcomes is implicated in decreased overall survival in AML patients in 28 months (95% CI: 0.62 to 0.92). Conclusion: This was observed when there was association between CD56 expression and other prognostic factors, influencing on patients’ management care and treatment. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1194 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1194/869

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 232 236 EN Rafet Eren University of Health Sciences, Okmeydanı Training and Research Hospital, Department of Hematology, Istanbul, Turkey Bahar Sevinçoğlu University of Health Sciences, Okmeydanı Training and Research Hospital, Department of Internal Medicine, Istanbul, Turkey Esra Doğan University of Health Sciences, Okmeydanı Training and Research Hospital, Department of Hematology, Istanbul, Turkey Demet Aydın University of Health Sciences, Okmeydanı Training and Research Hospital, Department of Hematology, Istanbul, Turkey Nihan Nizam İstanbul University, İstanbul Faculty of Medicine, Department of Internal Medicine, Istanbul, Turkey Naciye Demirel University of Health Sciences, Okmeydanı Training and Research Hospital, Department of Hematology, Istanbul, Turkey 2019 10 23 2020 03 03 Background: We evaluated the frequency of subnormal erythropoietin levels, JAK2V617F positivity and polycythemia vera (PV) in patients who did not meet WHO 2008 criterion for hemoglobin levels but were suggested to be investigated for PV in 2016 revision. Materials and Methods: We assessed the data of 92 patients, who were further evaluated with JAK2V617F mutation and serum erythropoietin (EPO) levels and bone marrow biopsy, if necessary. We also compared this patient group with 20 patients whose Hgb>18.5 g/dL for men and >16.5 g/dL for women. Results: Nine patients (45%) in the higher hemoglobin group were JAK2V617F positive, while 4 patients (4.3%) in the lower hemoglobin group were JAK2V617F positive (p<0.001). The number of patients with serum EPO levels <4.3 mIU/mL was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=7, 7.6%) (p<0.001). Finally, the number of patients who received a diagnosis of PV was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=9, 9.8%) (p<0.001). Conclusion: We found a substantial increase in patients who were candidates for testing for PV with the introduction of WHO 2016 criteria; these patients were diagnosed with PV with a rate (9.8%) that cannot be underestimated. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1198 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1198/864

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 237 247 Mehrnaz Ahmadi Department of Nursing, Student Research Committee, School of Nursing and Midwifery, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Mahin Gheibizadeh Nursing Care Research Center in Chronic Diseases, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Maryam Rassouli School of Nursing and Midwifery, Shahid Beheshti University of Medical Sciences, Tehran, Iran Abbas Ebadi 1) Behavioral Sciences Research Center, Life Style Institute, Baqiyatallah University of Medical Sciences, Tehran, Iran 2) Nursing Faculty, Baqiyatallah University of Medical Sciences, Tehran, Iran Marziyeh Asadizaker Nursing Care Research Center in Chronic Diseases, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Mojtaba Jahanifar Shahid Chamran University of Ahvaz, Ahvaz, Iran 2019 12 06 2020 03 01 Background: Uncertainty leads to a stressful situation in patients with thalassemia major that can dramatically affect their psychosocial coping ability, treatment process and disease outcomes, and reduce patients' quality of life. As one of the important factors affecting the health of thalassemia patients, understanding the concept of uncertainty is of major importance to health care providers especially nurses as the first line of exposure to these patients. The present study aimed to explore the experiences of uncertainty in patients with thalassemia major. Materials and Methods: The present qualitative study was conducted through in-depth face-to-face semi-structured interviews held with 18 patients with major thalassemia selected through purposive sampling. Interviews continued until saturation of data. All interviews were recorded, transcribed and analyzed with conventional content analysis method of Landman and Graneheim using MAXQDA10 software. Results: Two main themes, including 'living in the shadow of anxiety' and 'coping with uncertainty' emerged from patients’ experiences of illness uncertainty of thalassemia. 'Living in the shadow of anxiety' included four categories of 'fear of complications', 'contradictory views on treatment', 'unknown future' and 'stigma'. 'Coping with uncertainty' included three categories of 'spiritual coping', 'psychosocial coping' and 'knowledge acquisition'. Conclusion: According to the results of this study, uncertainty is a major psychological stress in patients with thalassemia major. Healthcare providers should therefore consider the challenges and concerns faced by patients and, through utilizing appropriate training and communicational practices, plan interventions and strategies to empower patients for coping with uncertainty.   https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1219 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1219/865

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 248 256 EN Aysun Senturk Yikilmaz Department of Hematology, Yıldırım Beyazıt University, Ankara 06010, Turkey Sema Akinci Department of Hematology, Ataturk Training And Research Hospital, Ankara 06010, Turkey Sule Bakanay Department of Hematology, Yıldırım Beyazıt University, Ankara 06010, Turkey İmdat Dilek Department of Hematology, Yıldırım Beyazıt University, Ankara 06010, Turkey 2019 12 01 2020 02 25 Background: Complete response (CR) and very good partial response (VGPR) are targeted with pre-ASCT induction regimens in patients by diagnosed multiple myeloma (MM), who are candidates for ASCT. In this study, it was aimed to compare the response and survival evaluations of cases who underwent induction treatment by vincristine-doxorubicin-dexamethasone (VAD) protocol versus bortezomib containing regimens. Materials and Methods: The data of 96 ASCT eligible patients, retrospectively analyzed. P value> 0.05 was considered statistically significant. Results: While 66 cases had received bortezomib containing regimens as induction regimen, 30 cases had received VAD protocol. The total survival was 91.3 (st.s 6) months and 43 (st.s 7.9) months, respectively, when we compared the cases without ASCT and with ASCT (p = 0.001). The OS of patients who underwent ASCT after reaching at least VGPR was longer than the underwent ASCT without reaching VGPR (p=0.019).  Post-ASCT PFS (p=0.717) and OS (p = 0.126) analyzes were performed in 74 cases undergoing ASCT treatment, there was no significant statistical difference when patients with treated by VAD protochol and treated by bortezomib containing regimens as pre-ASCT induction regimens was compared to each other. Conclusion: Whatever the type of induction regimen is, the level of response achieved before ASCT is important. The survival of the myeloma patients are much more influenced with HDT-ASCT as well as post-transplantation strategies to keep the patients in remission. Even though it is outdated, we think that the VAD protocol may be an option in patients who are not responding with the new generation of agents in the following days. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1216 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1216/866

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 4 2020 10 07 257 264 Amirhosein Maali 1) Infection Diseases and Tropical Medicine Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran 2) Cellular and Molecular Biology Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran 3) Student Research Committee, Babol University of Medical Sciences, Babol, Iran