Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Deferasirox in Chelation Naive Children with Transfusional Iron Overload in Basra, Iraq: A Two-Year Single Center Study 115 122 EN Basim Abdul-Hassan Center for Hereditary Blood Diseases, Basra Maternity and Children Hospital, Basra, Iraq Mea`ad Kadhum Hassan Department of Pediatrics, College of Medicine, University of Basra Rawshan Zuhair Jaber Center for Hereditary Blood Diseases, Basra Maternity and Children Hospital, Basra, Iraq Background: Effective management of iron overload in patients receiving long-term blood transfusion requires assessment and monitoring of both severity of iron overload and excessive iron chelation. We aimed to evaluate the efficacy and safety of Deferasirox (DFX) in chelation naive patients with transfusion dependent thalassemia and sickle cell disease. Methods: Chelation naive patients with transfusion dependent thalassemia (TDT) and sickle cell disease (SCD), aged 2-5 years, who had received DFX for at least 2 years were enrolled. Safety of DFX was assessed based on alanine aminotransferase (ALT) and serum creatinine levels, while efficacy was assessed based on serum ferritin levels. Results: The study included 93 chelation naive patients; 64 (68.82%) with TDT and 29 (31.18%) with SCD. Mean SF levels declined significantly from 2297.40±1037.46 ng/ml at baseline to 1700.65±1038.7 ng/ml at the end of the study. The efficacy of DFX was increased with increasing DFX dose to ≥30 mg/kg/day. The most commonly observed adverse effects were abdominal pain in 20 (21.50%), nausea in 8 (8.60%), and vomiting in 4 (4.30%) patients, which were transient and mild to moderate in severity. A minor, although significant change in the mean serum creatinine was reported after 24 months of treatment with DFX compared to the baseline (75.72±7.87 vs. 71.59±11.14 mmol/L) (P<0.05). The mean ALT (17.69±1.44 vs. 21.75±3.37 U/L) and median height-SDS at the end of the study did not show significant changes compared to the baseline levels. Conclusion: Although Deferasirox was found to be a safe, tolerable, and effective drug for reducing iron overload, monitoring safety markers and serum ferritin to ensure appropriate drug dosing can improve its efficacy.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Assessment of Association between Duffy Blood Group Phenotypes and Susceptibility to Systemic Lupus Erythematosus and its Severity 123 126 EN Alireza Rezvani Assistant professor of hematology and oncology, Shiraz Hematology Research Center, Shiraz, Fars, Iran Reza Fakhar MD graduate of Shiraz University of Medical Sciences Ehsan Taherifard Medical student at Shiraz University of Medical Sciences, Shiraz, Fars, Iran Zahra Habibagahi Assistant Professor of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Iran Erfan Taherifard Medical student at Shiraz University of Medical Sciences, Shiraz, Fars, Iran Background: Considering the role of Duffy antigenic system expression on RBCs as chemokines for inflammatory cytokines, expression of Duffy antigens as different phenotypes was studied in a group of patients with SLE. The association between different phenotypes of Duffy antigens and occurrence of SLE and its severity was assessed.  Methods: This cross-sectional study was carried out on 100 patients diagnosed as SLE using the “Systemic Lupus International Collaborating Clinics 2012 (SLICC) classification” criteria and 100 age-matched healthy subjects as control from April to June 2017. Duffy blood group status was determined in both groups. The patients were categorized into three groups of mild, moderate and severe according to “Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)” scores. Results: The results of this study showed no significant association between the three different phenotypes of Duffy antigen system and increased risk of SLE or its severity. Conclusion: The results of the current study did not approve of any association between three different Duffy phenotypes and increased risk of SLE or its severity. 

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Diagnosis of Iron Deficiency and Iron Deficiency Anemia with Reticulocyte Hemoglobin Content among Children Aged 6-18 Years 127 132 EN Murti Andriastuti Department of Child Health, Faculty of Medicine, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta, Indonesia Melita Adiwidjaja Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia Hindra Irawan Satari Department of Child Health, Faculty of Medicine, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta, Indonesia Background: Iron deficiency (ID) is the most common micronutrient deficiency in the world. If left untreated, ID will lead to iron deficiency anemia (IDA) and other irreversible consequences. The American Academy of Pediatrics recommended reticulocyte hemoglobin content (Ret-He) as an alternative laboratory examination to screen and detect ID. We aimed to compare Ret-He with other laboratory parameters to screen for iron status in healthy children.  Methods: This is a cross-sectional study comprising 207 children aged 6-18 years in Jakarta, Indonesia. Children were divided according to their iron status. Ret-He was compared with hemoglobin (Hb), mean corpuscular volume (MCV), ferritin, and transferrin saturation (TS) to assess iron status in children. Receiver operating characteristic (ROC) curve was performed to determine the optimal cut-off value for Ret-He using SPSS software.  Results: Ret-He had a positive correlation with MCV (r=0.690, n=207, P<0.001), Hb (r=0.491, n=207, P<0.01), and ferritin (r=0.336, n= 207, P<0.001). Ret-He can not be used  to detect iron depletion with the cut-off value of 30.3 pg with 100% sensitivity, 19.7% specificity, 100% negative predictive value (NPV), and 5.4% positive predictive value (PPV). A Ret-He cut-off value of 28.9 pg was established as optimal to identify ID (78.9% sensitivity, 56.2% specificity, 92.2% NPV, and 28.9% PPV) and 27 pg to detect IDA (75% sensitivity, 80% specificity, 98.1% NPV, and 18.7% PPV). Conclusion: Ret-He can be used as an alternative screening parameter to detect ID and IDA in children aged 6-18 years. Screening for IDA with Ret-He has to be done with other parameters, such as Hb examination.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Evaluation of Changes in Blood Products Transfusion Indices Following Three Years of Implementation of Iranian Blood Transfusion Organization Standards 133 138 EN Fereshteh Karbasian Pediatric Congenital Hematologic Disorders, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Ali Zafari Student Research Committee, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Kourosh Goudarzipour Pediatric Congenital Hematologic Disorders, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Department of Allergy and Immunology, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Siavash Shariatzadeh Student Research Committee, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Peyman Eshghi Pediatric Congenital Hematologic Disorders, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Background: Increasing demand for blood and blood products mostly due to increased individual access to health care services rises the concerns over lack of adequate amounts of blood products. It also can impose additional costs to blood transfusion centers and may increase transfusion-related complications. We aimed to investigate the impact of the Iranian Blood Transfusion Organization (IBTO) standards after three years of blood-bank committee activities on optimizing the consumption of the blood products.  Methods: All request forms of blood and blood products of Mofid Children’s Hospital were reviewed according to the department and type of the product in 2013 and indexes of “cross-matched to transfusion ratio” (C/T ratio), “transfusion index” (TI) and “transfusion probability” (%T) were calculated. Following 3 years of training by the Blood Bank Committee of the hospital and holding different meetings and workshops using IBTO standards, the same parameters were reassessed in 2017.  Results: In 2013, 13653 units of blood and blood products were ordered of which 10472 units (77%) were transfused and 3181 units (23%) were returned. C/T Ratio was equal to 1.43. After about 3 years of intervention, 17946 units of blood and blood products had been cross-matched of which 14775 units (83%) were transfused and 3171 units (17%) were returned. C/T Ratio after 3 years of intervention was equal to 1.33 (P<0.0001). T% was 69% in 2017 compared to 62% in 2013. Also, TI was 0.97 in 2017 compared to 0.83 in 2013. The blood bank committee’s activities in surgical departments improved C/T ratio of 2.83 to 2.13.  Conclusion: This study showed that holding scientific sessions and blood bank academic activities during more than 3 years was successful to optimize the use of blood and blood products in our hospital, particularly in surgical departments.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Pneumothorax and Acute Kidney Injury in the Early Phase of Acute Lymphoblastic Leukemia Induction Therapy due to Aspergillus Fumigatus and Pneumocystis Jirovecii Co-Infection: A Case Report 139 142 EN Parinaz Sedighi Student research committee, Hamadan University of Medical Sciences Hossein Esfahani Hamadan University of Medical Sciences Leukemia is the most common malignancy in children which leads to immunosuppression and predisposes patients to opportunistic infections. We report a 12-year-old girl with acute lymphoblastic leukemia (ALL) who developed simultaneous infection with pneumocystis Jirovecii pneumonia and aspergillosis in the induction phase of chemotherapy. The patient developed pulmonary cavitation and pneumothorax which was treated with trimethoprim-sulfamethoxasole in addition to intravenous liposomal Amphotrecin B. Pediatric oncologists should consider concurrent co-infection of opportunistic infections such as aspergillus fumigatus and Pneumocystis Jirovecii in immunocompromised patients especially if pneumothorax develops in early stages of disease or immunosuppressive therapy.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Maxillofacial Malignancy in Pregnancy; Report of Two Cases 143 147 EN Majid Sanatkhani Associate Professor of Oral and Maxillofacial Medicine, Oral and Maxillofacial Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Javid Rasekhi Specialist of Oral and Maxillofacial Medicine, Mashhad, Iran Zohreh Dalirsani Oral and Maxillofacial Medicine, Oral and Maxillofacial Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Narges Ghazi Assistant Professor of Oral and Maxillofacial Pathology, Oral and Maxillofacial Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Pregnancy is a physiological period in a woman’s life. It is well known that some pathological changes have been reported to be more common in pregnancy than other stages of the life. It seems that primary tumors have a tendency to progress more rapidly in pregnancy due to hormonal alterations or increased expression of some growth factors. The exact relationship between pregnancy and malignancy has not been established. There are different kinds of malignancies occurring during pregnancy. Herein, we report malignant tumors of the jaws in two pregnant patients. Histopathological diagnosis was compatible with chondroblastic variant of osteosarcoma and squamous cell carcinoma, respectively.  The clinical practitioners and dentists must be able to differentiate between the inflammatory and neoplastic conditions, especially during pregnancy to avoid unnecessary treatments which could lead to harmful effects on pregnant woman or her fetus. 

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 Massive Spontaneous Hemothorax in a Child with Hemophilia 148 149 EN Peyman Eshghi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Zahra Khafafpour Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Fatemeh Malek Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Behnaz Habibpanah Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 11 4 2019 12 1 CMV-induced Duodenal Papillitis in a Child with Acute Lymphoblastic Leukemia 150 152 FA Parastou Molai Tavana Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Childern's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mitra Khalili Pediatric Radiology Department, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Samin Alavi Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Childern's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran