Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Bone Mineral Density, Lean Body Mass and Bone Biomarkers Following Physical Exercise in Children with Acute Lymphoblastic Leukemia Undergoing Chemotherapy 69 75 EN Intsar Waked Kamal Albenasy Background: This study was aimed to evaluate the effects of physical exercise on bone mineral density (BMD), lean body mass (LBM) and bone biomarkers in children with acute lymphoblastic leukemia on chemotherapy. Methods: forty-six leukemic children were randomly assigned to two groups; exercise group (E) undergoing supervised mixed exercise program consisting of aerobic, resistance and flexibility training and control group (C) who did not receive any kind of exercise. BMD and LBM were measured by Densitometry (DEXA) and bone biomarkers were assessed through blood sample analysis at baseline, and then 6 and 12 months later. Results: There was marked increase in BMD, LBM and bone biomarkers in response to exercise with the time compared with control group (P<0.001). At the end of the study, the results of the study showed that the percentage of patients with normal lumbar BMD (LBMD) and total BMD (TBMD) in exercise group were 13% and 17.4%, respectively compared to 0% in control group. In addition, the percentage of osteoporotic patients at LBMD & TBMD in exercise group were 21.7% and 17.4%, respectively compared to 78.3% and 52.18% in control group, respectively (P<0.05). Conclusion: It can be concluded that physical exercise could be effective in increasing BMD, LBM and bone biomarkers in children with ALL. 

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Correlation between Mean Serum Ferritin Levels and Atrial Electrocardiographic Markers in Beta-Thalassemia Major 76 81 EN Yazdan Ghandi Aziz Eghbali Fatemeh Rafie Background: Atrial fibrillation (AF) is the most common arrhythmia in patients with β-thalassemia major (β-TM) due to cardiac iron overload. AF may be predicted by changes in P wave parameters. The aim of this study was to evaluate the correlation between P-wave parameters and atrial markers with mean serum ferritin levels measured yearly. Methods: Fifty patients with β-TM and 50 healthy subjects as control group were enrolled in the study. Both groups underwent electrocardiography (ECG) and echocardiography. P-wave dispersion (PWd), maximum and minimum P-wave duration (P-max and P-min) were measured. Echocardiography was performed by pulse wave Doppler of the lateral mitral valve. Annual mean serum ferritin level was calculated through monthly sampling. Results: There were 22 men and 28 women with β-TM (mean age: 21.4±8.3 years). Mean serum ferritin levels were 1476.8±919.2 ng/ml. Systolic and diastolic blood pressure were significantly different between thalassemia and control groups (P<0.002 and P<0.004, respectively). There were significant differences between BMI, PWd, P-max and P-min (P<0.0001, respectively). A cut off value of 40 msec for PWd had a sensitivity of 100% and a specificity of 90.7% in identifying mean serum ferritin levels higher than 1078 ng/ml. Mean serum ferritin level of 1024 or more was associated with sensitivity of 100% and specificity of 90.1% for inter-atrial block > 110 msec. There were correlations between mean serum ferritin level and age of the patients along with PWd, P-min and P-max (P<0.0001, respectively). Conclusion: In β-TM, atrial parameters in ECG were correlated with both mean serum ferritin levels measured yearly and patients’ age. We recommend that in patients with thalassemia major with mean serum ferritin levels higher than 1000 ng/ml, P wave parameters should be carefully evaluated and closely followed.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Analysis of Survival in Patients with β-thalassemia Major in Guilan, Northern Iran 82 86 EN Zahra Mohtasham-Amiri Guilan University of Medical Sciences Korosh Khanaki Guilan University of Medical Sciences Ali Davoudi-Kiakalayeh Guilan Blood Transfusion Organization Seyed Mahmood Rezvani Guilan University of Medical Sciences Abbas Jafari-Shakib Guilan Blood Transfusion Organization Reza Jafari-Shakib Guilan University of Medical Sciences Background: Advances in treatment of thalassemia major (TM) have improved life expectancy and survival of the patients. This study was conducted to assess survival rate of these patients in Guilan province, Northern Iran. Methods: In this cross-sectional study, records of 1243 patients with TM from 2001 to March 2016 were evaluated in Guilan province. Sources of data were health centers of the province and territories, blood transfusion organization, general hospitals and private clinics. Data were analyzed using Kaplan–Meier method.  Results: 958 (77%) patients were born before 1997, the year that screening program of β-thalassemia in Iran was started. No case of β-TM was born during the last 5 years of the study. The 5, 10, and 15-year survival rate for all patients were 100%, 100%, and 99.6%, respectively. Hepatitis C infection was associated with decreased survival rate. Conclusion: Survival in patients with TM has dramatically improved over the recent years and future studies about causes of death in these patients are highly recommended.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Efficacy of Rituximab in the Treatment of Plasma Exchange Refractory Thrombotic Thrombocytopenic Purpura 87 91 EN Alireza Sadeghi Assistant Professor, Department of Hematology-Oncology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Farzaneh Ashrafi Assistant Professor, Department of Hematology-Oncology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Arman Sourani Student Research Committee, School of medicine, Isfahan University of Medical sciences, Isfahan, Iran Background: Rituximab can induce a durable remission in plasma exchange refractory thrombotic thrombocytopenic purpura (TTP). Timing of Rituximab infusion in combination with plasma exchange (PE) and long term follow-up for probable side effects of such treatment is still lacking. Methods: This study was conducted among 10 patients with plasma exchange refractory TTP. According to the study designation, first PE was performed within 36-48 hours after first dose of rituximab. Results: Eight of ten (80%) patients received 1 course of rituximab. Two of ten (20%) patients received another course of rituximab due to relapse. Repsonse rate (RR) to rituximab in combination with plasma exchange, was 90%. Overall Survival of the patients was 90% and 1 and 5-year relapse free survival rate (RFS) was 90% and 83%, respectively. One of the patients expired due to Systemic Lupus Erythematosus flare up. Conclusion: According to this study, treatment of refractory TTP with rituximab in combination with PE could be effective. Iranian Registry of Clinical Trials: IRCT2017012232125N1

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Adrenocortical Carcinoma Mimicking Pheochromocytoma: A case Report and Review of Literature 92 95 EN Mazaher Ramezani Molecular Pathology Research Center, Emam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran Mahdokht Azizi Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran Masoud Sadeghi Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Adrenocortical carcinoma (ACC) is an uncommon malignancy originating from cortex of adrenal gland. The most common pitfall in diagnosis of ACC is to distinguish it from pheochromocytoma. Here we report a 62-year-old hypertensive man with presentation of dyspnea. The laboratory data showed an increase in urine cortisol and renin with a mild increase in aldosterone, but decrease in adrenocorticotropic hormone. Computerized tomography (CT) scanning revealed heterogeneous mass with enhancement in the adrenal region. The patient underwent surgery with an empirical diagnosis of pheochromocytoma, whereas ACC with high mitotic rate of about 50 mitoses/50 high-power field with vascular invasion was reported on histopathology. Immunohistochemistry was positive for Vimentin, Inhibin-α, Melan-A, and Synaptophysin. Ki-67 was positive in 25% of tumor cells. The results confirmed the diagnosis. ACC should be considered in differential diagnosis of adrenal masses especially in cases of hypertension and Cushing syndrome.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 Congenital Hypofibrinogenemia Detected during Preoperative Workup for Major Cardiac Surgery 96 98 EN K.B Chetak Vilas Hiremath K R Sujay U S Dinesh Kumar K Jagadish kumar Afibrinogenemia or hypofibrinogenemia is a quantitative defect in fibrinogen. It is a rare disease with estimated frequency of 1 in 1,000,000 population. Routine preoperative screening tests of coagulation disorders are insufficient to detect subtle disorders of fibrinogen. Herein, we report a child who was supposed to undergo open cardiac surgery for a large atrial septal defect. Preoperative coagulation screening tests revealed minimal prolongation of prothrombin time which made us do further work-up. Finally, she was diagnosed with hypofibrinogenemia who was suggested to be congenital.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 10 3 2018 9 1 CMV Pneumonitis with Bilateral Pleural and Pericardial Effusion in A Child with Non Hodgkin Lymphoma 99 100 FA Mohammad Ali Fallahzadeh Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mitra Khalili Department of Pediatric Radiology, Shahid Beheshti University of Medical Sciences, Tehran, Iran