Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Thromboembolic Events in Severe Thalassemia Syndromes 41 46 EN Mozhgan Hashemieh Imam Hossein Medical Center, Shahod Beheshti University of Medical Sciences, Tehran, Iran. In the last few decades, the prognosis of patients with β-thalassemia has improved dramatically. However these patients suffer from many serious complications. One of the most important morbidities in patients with thalassemia are thromboembolic events that are mainly observed in thalassemia intermedia. Profound hemostatic changes have been observed in patients with thalassemia intermedia and numerous factors have been attributed to the pathogenesis of hypercoagulable state among patients with thalassemia intermedia. In this review the different mechanisms resulting in hypercoagulability in patients with thalassemia, the clinical importance, prevention and treatment of thromboembolic episodes have been discussed.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Elevation of D-Dimer, But Not PT and aPTT, Reflects the Progression of COVID-19 Toward an Unfavorable Outcome: A Meta-Analysis 47 53 EN Davood Bashash Department of Hematology and Blood Banking, School of Allied Medical Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran Hassan Abolghasemi Pediatric Congenital Hematologic Disorders Research Center, Mofid Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Sina Salari Department of Medical Oncology, Hematology and Bone Marrow Transplantation, Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Meysam Olfatifar Gastroenterology and Liver Diseases Research Center, Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Peyman Eshghi Pediatric Congenital Hematologic Disorders Research Center, Mofid Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Mohammad Esmaeil Akbari Cancer Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Background: Coronavirus disease 2019 (abbreviated as COVID-19) is a mysterious respiratory syndrome symptomatically spanning from healthy carriers to patients with life-threatening complications, in some cases, leading to a mournful death. For the time being, the contributory role of hematologists is much more recognized in the management of COVID-19, since the emergence of coagulopathy has recently been the focus of many studies in SARS-CoV-2 infection.  Methods: To provide a well-conceptualized viewpoint demonstrating the prognostic value of coagulation-related laboratory tests, we planned to perform a meta-analysis of pertinent literature representing information on PT, aPTT, and D-dimer tests in patients with COVID-19.  Results: Albeit the estimated pooled means of PT and aPTT were higher in severe cases, their mean values were not significantly higher as compared with patients in a non-severe condition. On the other hand, the mean value of D-dimer in severe patients was significantly higher than non-severe cases (X2=6.34, P=0.01), highlighting that the elevation of this parameter may be associated with the progression of the disease toward an unfavorable clinical outcome.  Conclusion: Even though at the time of writing this article the lack of adequate and appropriate studies denotes a major limitation to the current study, planning for the future research to determine the prognostic value of laboratory tests reflecting SARS-CoV-2-induced coagulopathy, mainly D-dimer, will definitively cast a flash of light on the significance of therapeutic anticoagulation at least for those with no absolute contraindication.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Detection of FLT3 Gene Mutations In Patients With Acute Myeloid Leukemia In Surabaya, Indonesia: A Single-Center Study 54 57 EN Paulus Budiono Notopuro Doctoral Student, Faculty of Medicine, Airlangga University, Indonesia Nugraha Jusak Departement of Clinical Pathology, Faculty of Medicine Airlangga University, Surabaya, Indonesia Notopuro Harianto Departement of Biochemistry, Faculty of Medicine Airlangga University, Surabaya, Indonesia Background:  FLT3 gene mutation contributes worse prognosis in patients with acute myeloid leukemia (AML). Almost 67% of patients with AML with FLT3 gene mutation cannot reach complete remission after induction therapy, and  they are also at high risk of  relapse. We aimed to investigate the FLT3-ITD, -TKD (D835) gene mutation prevalence in patients with AML at Surabaya and their association with leukocyte and bone marrow blast cell count. Methods: 20 de novo patients with AML were recruited during February–July 2018. They were investigated through routine AML check-up and detection for FLT3-ITD, -TKD (D835) gene mutation.  Results:  Four patients with de novo AML (20%) had FLT3-ITD gene mutation, and one  patient had  FLT3-TKD (D835) gene mutation. Median leukocyte count in patients with AML with FLT3-ITD mutation was higher than wild type patients ( 146.3×103/ µL vs 16.4 x 103 / µL, P=0,002). The mean bone marrow blast cell count was higher in patients with AML with FLT3-ITD mutation than wild type patients ( 86.5% vs 57.9%, P=0,047).  The difference in leukocyte and bone marrow blast cell count in patients with FLT3-TKD (D835) mutation could not be analyzed since there was only one patient with this mutation.  Conclusion: The prevalence of FLT3-ITD gene mutation in patients in AML in Surabaya was 20% and FLT3-TKD (D835) was 5%. Patients with FLT3-ITD gene mutation was associated with leukocyte and bone marrow blast cell count. 

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Spectrum of Mutations of Thalassemia among Couples from Izeh City, Khuzestan Province, Iran 58 63 EN Azam Khedri Department of Clinical Biochemistry, Faculty of Medicine, Ahvaz Jundishapur University of Medical Science, Ahvaz, Iran Fatemeh Asadi Department of Molecular Genetics, Science and Research Branch, Islamic Azad University, Fars, Iran Seyedeh Moloud Rasouli Ghahfarokhi School of Nursing and Midwifery, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Narges Obeidi Department of Hematology, School of Para Medicine, Bushehr University of Medical Sciences, Bushehr, Iran Frough Talebi Blood Transfusion Organization, Bushehr, Iran Sahar Moghbelinejad Department of Genetics, Qazvin University of Medical Sciences, Qazvin, Iran Background: Hemoglobinopathies are inherited blood disorders with an autosomal recessive pattern. We aimed to evaluate the frequency of mutations of thalassemia and hemoglobinopathies among couples referred to health centers of Izeh in Khuzestan Province, Iran. Methods: This cross-sectional study was performed on 150 couples referred to Izeh Health Centers in 2015-2018. DNA was isolated from peripheral venous blood samples and then the HBB gene was analyzed by using Sanger sequencing. For molecular analysis of α-globin gene, multiplex Gap-PCR and ARMS-PCR was performed to identify mutations of α-thalassemia.  Results: DNA analysis revealed 13 different mutations for beta thalassemia in studied couples. Three mutations including 36/37 (- T), IVS-II-1 (G>A) and IVS-I-110 (G>A) accounted for 20.7, 19.3 and 13.3% of beta thalassemia mutations, respectively. For alpha thalassemia; α3.7 (49.5%), -- MED (19.1 %) and -α4.2 (3.1%) were identified as the most common mutations.  Conclusion: Considering common alpha and beta mutations of this geographic area of Iran could be useful concerning genetic counselling in of the population where the rate of consanguineous marriage is high.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Papillary Adenocarcinoma of the Lung in a Young Man: A Case Report 64 66 EN Mazaher Ramezani Molecular Pathology Research Center, Imam Reza hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran Ruhollah Heydari Students Research Committee, 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 Lung cancer is the leading cause of cancer mortality worldwide. Adenocarcinoma is the most frequent pulmonary malignancy in most parts of the world. The papillary predominant subtype is applied when more than 75% of the tumor has the papillary architecture in histopathology. This subtype consists of about one-tenth of pulmonary adenocarcinomas. A 27-year-old man was referred with the history of dyspnea for the past three months. The patient had a diagnosis of right pulmonary empyema and was on medical treatment. After non-response he underwent surgery. The pathologist reported papillary adenocarcinoma of the lung, moderately differentiated with pleural involvement. Immunohistochemistry confirmed the diagnosis. Correct diagnosis of this subtype in histopathology is necessary for the determination of prognosis, cytogenetic changes, and selecting best treatment modalities.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Massive Ascites, Protein Losing Enteropathy and Pseudo-Obstruction as Rare Manifestations of Systemic Lupus Erythematosus in a known case of Immune Thrombocytopenic Purpura 67 69 EN Reza Jafari Department of Radiology, Baqiyatallah University of Medical Sciences, Tehran, Iran and Chemical Injuries Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran Hassan Abolghasemi Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran Mohammad Javanbakht Nephrology and Urology Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran Zahra Najjari Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran Fatemeh Beiraghdar Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran Zohreh Kavehmanesh Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease with diverse clinical manifestations involving multiple organ systems. Peritoneal involvement as a sign of gastrointestinal disease in SLE is a rare condition. Massive ascites as the initial manifestation of SLE is an extremely rare situation. Here, we report a 13-year-old female with bile-stained vomiting, ascites and signs of pseudo-obstructions with a past medical history of immune thrombocytopenic purpura (ITP) who was later diagnosed to have SLE.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 All That Glitters is Not Gold: A Pregnant Woman with a Rare Inherited Bleeding Disorder 70 71 EN Reza Aminnejad Department of Anesthesiology & Critical Care, Qom University of Medical Sciences, Qom, Iran.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 12 2 2020 6 1 Imatinib Mesylate-Induced Periorbital Edema in a Child with Chronic Myeloid Leukemia 72 73 FA Samin Alavi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Azin Ashnagar Ophthalmologist, Zanjan University of Medical Sciences, Zanjan, Iran