Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 The Correlation between Serum Ferritin, Serum Troponin T, cardiac T2* MRI and Echocardiographic Findings in Patients with Thalassemia Major 1 5 EN Aziz Eghbali Associate Professor, Department of Pediatrics, School of Medicine, Aliasghar clinical research development center, Iran University of medical sciences, Tehran, Iran. Sarvenaz Mehrabi Department of emergency, School of Medicine, Arak University of Medical Sciences, Arak, Iran Yazdan Ghandi Associate professor, Department of Pediatrics, School of Medicine, Arak University of Medical Sciences, Arak, Iran. Aygin Eghbali Students Research Committee, school of medicine, Iran university of Medical Sciences, Tehran, Iran Mona Dabiri Department of emergency, School of Medicine, Arak University of Medical Sciences, Arak, Iran Morteza Mousavi_Hasanzadeh Students Research Committee, School of Medicine, Arak University of Medical Sciences, Arak, Iran. Background: Frequent blood transfusions lead to various complications in patients with thalassemia major. Heart problems caused by iron deposition in the myocardium are the major cause of death in patients with thalassemia major. Early diagnosis of cardiac dysfunction in patients with thalassemia major undergoing frequent blood transfusion is very important. We aimed to investigate the correlation between serum Ferritin level, serum Troponin T (TropT), cardiac T2* MRI and echocardiographic findings in patients with thalassemia major. Methods: This cross-sectional study was conducted on all children >5 years old with thalassemia major admitted to Amirkabir Hospital, Arak, Iran, during 2016-2017. serum Ferritin level and Troponin T, echocardiography, and cardiac T2*MRI were analyzed in all participants. Results: We found no correlation between serum ferritin and Trop T levels. However, there was a significant correlation between serum Trop T and serum ferritin with cardiac T2*MRI and also between serum Trop T and echocardiographic parameters (positive correlation with E/A ratio and functional shortening); and a negative correlation with left ventricular ejection fraction (LVEF). Moreover, there was no correlation between cardiac T2* MRI and echocardiographic findings. Conclusion: Our results showed a positive correlation between serum troponin T with E/A size and FS and a negative correlation with LVEF and it was an important prognostic factor in early stage of cardiac damage in patients with thalassemia major. Moreover, there was a negative correlation between results of T2* MRI and serum ferritin and troponin T.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Expression of Proviral Integration of Moloney Virus-2 (PIM2) Gene in Patients with Acute Myeloid Leukemia and its Clinical Significance 6 11 EN Dina El Dahshan Clinical Pathology Department, Faculty of Medicine, Beni-Suef University Amira Hammam Clinical Pathology Department, Faculty of Medicine, Beni-Suef University Aya Ahmed Clinical Pathology Department, Faculty of Medicine, Beni-Suef University Mohamed El Samra Clinical Oncology Department, National Cancer Institute, Cairo University ackground: Despite extensive research in leukemia, intracellular events leading to prolongation of cell cycle and resistance to pro-apoptotic factors are still not clearly defined. In recent years, the search for such events led to focusing on an anti-apoptotic factor, PIM-2 (Proviral integration of Moloney virus-2). The aim of the present study was to assess the expression of PIM2 gene in patients with acute myeloid leukemia (AML) through quantitative real time polymerase chain reaction (QRT-PCR) and to correlate the results with clinical and laboratory findings of the patients as well as their response to the treatment. Methods: 80 patients with AML and control group were enrolled in the present study. QRT-PCR was used to study PIM2 gene expression. Results: The mean expression level of PIM2 gene was significantly higher in AML patients (3.5941±7.7736) compared with the control group (0.5303±0.4014) (P=0.034). Its expression level was not different in terms of achieving remission. A positive correlation was observed between PIM2 gene expression and total leucocytic count (R=0.059, P=0.719), while there was a negative correlation between the gene expression and platelet count (R=-0.118, P=0.470). No significant correlation was found between PIM2 gene and patients’ response to treatment as (P=0.883) although it’s level was higher in patients who did not achieve complete remission (6.1±11.9) than patients who achieved complete remission (3.1±5.3). Conclusion: The present study showed higher PIM2 expression level in AML patients than normal population. Also, its level was higher in patients who did not achieve complete remission.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Nutritional Status of Pediatric Patients with Cancer in Iran: A Single Center Study 12 17 EN Leila Khajavi Department of Nutrition, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Hamid Farhangi Associate Professor in Pediatric Hematology & Oncology, Faculty of Medicine Mashhad University of Medical Sciences, Mashhad, Iran Sara Movahhed Department of Nutrition, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Fatemeh Nejati Salehkhani Department of Nutrition, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Abdolreza Norouzy Department of Nutrition, Medical school, Mashhad University of Medical Sciences, Iran Background: As children have a higher metabolic rate and higher calorie needs for growth, they are more susceptible to nutritional depletion than adults. We aimed to assess the nutritional status of children with cancer who were referred to the outpatient clinic of hematology and oncology clinic at Sheikh Children Hospital, Mashhad, Iran. Methods: The nutritional status of the children with cancer was assessed by anthropometric indices and nutritional risk screening tool. We used BMI-for-age (BMIFA), height-for-age (HFA) and weight-for-height (WFH) to define malnutrition. The anthropometric indices (BMIFA, HFA, and WFH) were categorized according to z-scores. STRONGkids tool was applied for screening risk of malnutrition. Results: 61 pediatric patients with cancer were assessed. Of the studied patients, 77% were boys (47 patients) with a mean age of 8.4 years (6 months to18 years). Acute lymphoblastic leukemia (ALL) was the most prevalent cancer (64%). 26.2% of the patients had malnutrition according to BMI for age z-score index (underweight), 24.5% according to weight for height (WFH) index (wasting) and 21.3% according to HFA index (stunting). The STRONGkids classified 34.4% of patients as high risk, and 65.6% as moderate risk of malnutrition. Conclusions: Malnutrition was prevalent among pediatric cancer patients in this study, so appropriate nutritional screening and management should be implemented for improving the nutritional status of children with cancer.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Thalassemia Gene Mutations in Kohgiluyeh and Boyer-Ahmad Province 18 23 EN Jalal Pouranfard Departments of Paramedical Sciences, Yasuj University of Medical Sciences, Yasuj, Iran Farzad Vafaei Departments of Pediatrician, School of Medicine and clinical research center, Emam Sajad Hospital, Yasuj University of Medical Sciences, Yasuj, Iran Sajad Afrouz Cellular and Molecular Research Center, Yasuj University of Medical Sciences, Yasuj, Iran Mohsen Rezaeian Department of health research center, Yasuj University of Medical Sciences, Yasuj, Iran Background: Thalassemia is the most common hereditary anemia which has a relatively high prevalence in Iran. In most cases, more than 300 mutations have been identified, which affect genes of alpha and beta globin chains and lead to lack of production or reduction of chains. Iran’s population is composed of different ethnic groups, thus, determining the frequency and distribution of these mutations is essential in different parts of the country. We aimed to assess Thalassemia gene mutations in Kohgiluyeh and Boyer-Ahmad province. Methods: In this cross-sectional study, 656 couples were selected and their Genomic DNA was extracted by DNA extraction kit method and tested using multiplex gap-polymerase chain reaction (gap-PCR), amplification refractory mutation system-PCR (ARMS-PCR), and DNA sequencing. Finally all data were analyzed using the SPSS version 17 software. Results: More than 13 mutations were found on α-globin genes. Based on gene frequency, the most common mutant allele was –α3.7/αα (rightward) (71.3%) followed by the two gene deletion −α3.7/−α3.7 (2.5%). Other common mutations were polyA2 (2.1%), αcodon 19α/αα (1.7%), –α3.7/αα/–α3.7/αα (1.5%), – (α) 20.5 (0.6%), α−5 nt/αα (0.5%), and other mutations. In this study, more than 21 mutations were identified on beta thalassemia gene. The most common mutation was CD36- /37 (-T) (19.8%). Other common reported mutations included IVSII-1 (G>A) (9.5%), IVS I-110 (G>A) (4.7%), IVSII-745 (C>G) (4.4%), codon 82/83(-G) (3.7%), FSC 8/9 (+G) (1.7%), Codon19(1.5%), 25 bp deletion (beta0) (1.5%), IVS-I-116 (T>G) (1.4%), IVSI-6 (G>C) (1.1%), codon 5 (-CT) (0.9%), codon 88 (-C) (0.5%), and IVSI-1(G>A) (0.3%). Conclusion: The frequencies of these mutations were different in various parts of the country. Therefore, defining thalassemia mutations is necessary to establish prenatal diagnosis programs leading to lower medical cost in Kohgiluyeh and Boyerahmad province.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Association of MicroRNA-146a rs2910164 Polymorphism and Lung Cancer in Iranian Population: A Case-Control Study 24 28 EN Ali Dideban uman Genetics Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran Ensiyeh Seyedrezazadeh Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, Iran Akbar Sharifi Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, Iran Bahareh Abd-Nikfarjam Department of Immunology, School of Medicine, Qazvin University of Medical Sciences, Qazvin, Iran Morteza Sadeghi uman Genetics Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran Background: Lung cancer is the first cause of cancer deaths worldwide. Polymorphisms in microRNAs genes affect their structure and their attachment to target genes. The purpose of the present study was to investigate the association of miR-146a rs2910164 polymorphism with the risk of non-small cell lung cancer (NSCLC) in Iranian patients.  Methods: This case-control study was performed among 103 patients with lung cancer and 100 healthy controls. The genotyping of miR-146a rs2910164 polymorphism was assayed by polymerase chain reaction–restriction fragment length polymorphism (PCR–RFLP) and a number of samples were sequenced for final approval.  Results: A significant association was found between rs2910164 polymorphism CG genotype and risk of lung cancer progression in dominant models (CG vs. GG: OR=1.855, CI=1.09-3.33, P=0.03). There was no association between the C allele and frequency of rs2910164C>G polymorphism and risk of lung cancer (C vs. G: OR=1.54, CI=0.97-2.44, P=0.08).  Conclusion: There was a significant association between miR-146a rs2910164 polymorphism and lung cancer in Iranian population. This polymorphism can be considered as a risk factor of non-small cell lung cancer.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Multiple Cerebral Juvenile Xanthogranuloma; a Case Report 29 33 EN Sima Fallah-Arzpeima Assistant Professor of Radiology, Department of Radiology, School of Medicine, Alzahra Hospital, Poursina Hospital, Guilan University of Medical Sciences, Rasht, Iran Bahram Darbandi Associated Professor of pediatric hematology and oncology, Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran Afagh Hassanzadeh Rad PhD of Linguistics, Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran Mohamad Haghani Dogahe Student of Medicine, Guilan University of Medical sciences, Rasht, Iran Parham Niyasti Student of Medicine, Guilan University of Medical sciences, Rasht, Iran Adel Baghersalimi Assistant Professor of pediatric hematology and oncology, Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran Juvenile xanthogranuloma (JXG), is the most common form of non-Langerhans cell histiocytosis. It is a rare and usually benign, monoclonal proliferative disorder of histiocytic cells. The occurrence of multiple brain lesions is extremely rare. Due to the rarity of CNS disease, the preferred treatment and overall prognosis of these patients remain unclear. In this case report, authors present their experience about a patient with isolated multiple cerebral JXG which yields considerable outcomes. The patient was an eleven-year-old boy with anorexia and recurrent vomiting. Brain MRI demonstrated multiple lesions involving right lateral ventricle wall and periventricular white matter, the right side of splenium, mammillary body and tuber cinereum, fourth ventricle floor and roof, and cerebellar tonsil. After initial response to chemotherapy, the lesions recurred and radiotherapy was done. Consequent to good response after radiotherapy and despite the occurrence of a seizure attack, long term follow-up showed the marked resolution of the lesions and a good general status. 

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Solitary Fibrous Tumor of Testis: A rare Case with Review of Literature 34 37 EN Parham Nejati Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Farhad Amirian Molecular Pathology Research Center, Imam Reza hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran Masoud Sadeghi Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Kimia Karami Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran Mazaher Ramezani Molecular Pathology Research Center, Imam Reza hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran Solitary fibrous tumors (SFTs) more commonly arise in the pleura but recently, they have been reported in several extrapleural organs. Urogenital localization is rare, and only small numbers of cases of paratesticular SFT have been reported. An 81-year-old male with a history of colon carcinoma and complaint of testis swelling was referred for evaluation of a right paratesticular mass. Physical examination revealed a 2 cm oval-shaped paratesticular mass and herniation of intestinal loops in the right inguinal region after cough and Valsalva maneuver. An ultrasound examination was found in the upper pole of testis a well-defined hypoechoic mass in favor of testicular mass. It also revealed moderate to severe bilateral hydrocele and calcified wall in favor of benign lesion. In conclusion, SFT should be considered in the differential diagnosis of paratesticular masses and needs to be confirmed by IHC. CD34 and CD99 biomarkers are useful for confirmation of SFT.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 1 2020 3 1 Pancytopenia: A Rare Presentation of Late Onset Isovaleric Acidemia 38 40 EN Hossein Karami Thalassemia Research Center, Department of Pediatrics Hematology and Oncology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran Daniel Zamanfar Diabetes Research Center, Department of Pediatrics, Bou Ali Sina Hospital, Mazandaran University of Medical Sciences, Sari, IR Iran Mohammad Reza Navaifar Pediatric Infectious Diseases Research Center, Mazandaran University of Medical Sciences, Sari, IR Iran Parastoo Jamallivani Student Research Committe, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran Kimia Rasoli Student Research Committe, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran Mohammad Naderisorki Thalassemia Research Center, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, IR Iran