Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Investigation of Leptin, Leukemia Inhibitory Factor (LIF), and IL-6 Serum Levels in Myeloid Leukemia 0 0 EN Background: Leptin has been implicated in the differentiation and proliferation of hematopoietic cells. Leukemia inhibitory factor (LIF) may play an important role, along with Interleukin-6 (IL-6) and granulocyte colony stimulating factor (G- CSF), in the regulation of early hematopoietic stem cells. The aim of the study was to evaluate serum level of leptin, LIF, and IL-6 in myeloid leukemia patients. Materials and Methods: We investigated serum level of leptin, LIF, and IL-6 levels, body mass index, hemoglobin, and hematocrit in 30 myeloid leukemia patients (15 patients with acute and 15 with chronic myeloid leukemia) before chemotherapy, and compared the results with 15 healthy controls. Results: Leptin, LIF and IL-6 serum levels, and lipid profile in myeloied leukemic patient was significantly different from the control group (p<0.05). The relationship between leptin and BMI was statistically significant in control group, whereas in the patient group, there were no significant relationship between leptin and BMI. Conclusion: Lipid profile and leptin, LIF, and IL-6 serum levels of leukemic patients were significantly different from normal population.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Frequency of Human Platelet Antigens (HPA-2/3/5) Polymorphism in Iranians Evaluated by RFLP-PCR 0 0 EN ackground: Knowing the human platelet antigens (HPA) and genes frequency in different populations is important not only for population studies but also for clinical transfusion practice. HPA genes frequency in Iran is not evaluated, so we investigated the HPA-2, HPA-3, and HPA-5 frequency and their polymorphism. Materials and Methods: DNA from 120 Iranians (99 Iranian blood donors and 21 patients with platelet refractoriness) was isolated from peripheral blood mononuclear leucocytes and tested by restricted fragment length polymorphism analysis (RFLP). Results: The frequency of HPA phenotypes was determined as follows: (in donors) HPA-2a/2a:75.4%, HPA-2a/2b:24.8%, HPA-3a/3a:8.1%, HPA-3a/3b:70.7%, HPA-3b/3b:21.2%, HPA-5a/5a:99 % and HPA-5a/5b:1% (in patients) HPA-2a/2a:66.7%, HPA-2a/2b:33.3%, HPA-3a/3a:23.8%, HPA-3a/3b: 47.6%, HPA-3b/3b:28.6%, HPA-5a/5a:90.5%, and HPA-5a/5b: 9.5%. Frequency of genotypes evaluated by RFLP were as below: HPA-2a=0.87, HPA-2b=0.13, HPA-3a =0.28, HPA-3b=0.72, HPA-5a=0.99, and HPA-5b=0.01 in both donors and patients. Conclusion: HPA gene frequencies observed in Iranians were in the range obtained in previous studies but not exactly equal to other populations. It might be due to their relations with other populations. There was not any significant difference between two groups in this study.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Iron Stores in Male Blood Donors in Tehran Regional BloodTransfusion Center 0 0 EN Background: Iron deficiency and its related anemia, which is the world’s most widespread nutritional deficiency, can be one of the possible consequences of regular blood donation. This research was carried out to compare iron stores in regular blood donors and first-time blood donors in Tehran Regional Blood Transfusion Center. Materials and Methods: This study was carried out on 2,149 male blood donors, who were divided into two groups of regular and first-time blood donors. Blood samples were analyzed for red blood cell parameters, serum iron and ferritin, and total iron binding capacity. Results: The results showed that iron depletion was more common in regular blood donors than in first-time donors. Eleven percent of regular blood donors and 0.8% of first-time blood donors had iron deficiency. Of regular donors, 4.2% suffered from iron deficiency and 2.5% suffered from iron depletion anemia while there was no case of iron deficiency or anemia in first-time donors. Difference between two groups was statistically significant (p<0.01). According to the results, 8.9% of regular donors donated more than 4 times during last two years suffered from iron deficiency. Conclusion: Regular blood donation seems to have an impact on the iron stores of blood donors. However, the prevalence of iron deficiency in Iranian blood donors is lower than donors in other countries. We recommend an annual investigation of iron stores be carried out for blood donors who have given blood more than twice a year.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Neonatal Screening for G6PD Deficiency in Mazandaran Province, Iran 2007-2010 0 0 EN Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common disease of the hexose monophosphate pathway existing in more than 400 million people worldwide. The aim of this study was to identify neonates with G6PD deficiency following national program for screening and education of affected newborns’ parents started since June 2007 in Mazandaran, a northern Province of Iran. Materials and Methods: Blood sampling was performed via a heel prick prepared for screening of congenital hypothyroidism, Phenylketonuria, and G6PD deficiency. Fluorescent spot test kit set by Kimia Pajohan Company (Iran) was used. A confirmatory test with a venous blood sample was done within 4th month of age according to the protocol. The second enzyme activity test was a quantitative photometric method using a commercially available kit (Randox, U.K.) with sensitivity of 154 IU and normal range of 6.97- 20.5 U/gHb. Data were analyzed using SPSS software version 16 and descriptive methods. Results: During 36 months, 115622 newborns (51.4 % male) were studied. G6PD enzyme deficiency was found in 6.1% of the newborns (CI95%= 5.92-6.28%). As expected, male/female ratio of affected newborns was 6.19:1. Of confirmed affected infants in second enzyme activity test, 86.1% were male and 13.9% were female. Conclusion: The reported rate of G6PD enzyme deficiency was less than expected (12-13%). We strongly recommend continuing screening and education of parents and also mass media education against consuming “Fava bean” and “Naphtalen”.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Neonatal Screening for Sickle Cell Disease in South West Iran: a Pilot Study 0 0 EN Background: Children affected with sickle cell disease (SCD) are at increased risk for severe morbidity and mortality, especially during the first 3-5 years of life. It is suggested that early treatment can improve the condition. The aim of this pilot study was to estimate the incidence of hemoglobin S (HbS) by umbilical cord blood screening in Khorramshahr and Abadan cities in southwest of Iran. Materials and Methods: Cord blood sample (10 ml) was collected and stored in ethylenediaminetetraacetic acid (EDTA) precoated tubes and transported to the reference laboratory in Ahwaz. Samples were analyzed for presence of HbS by hemoglobin electrophoresis. Positive specimens were confirmed by sickle preparation test. Results: Four patients (1.3%) with sickle cell disease were identified among 308 screened newborns, all with Arabian ethnic origin. Conclusion: We concluded that HbS is a common disease in Khuzestan Province.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Seroprevalence of Transfusion-transmitted Infections Among Multi-transfused Patients in Southwest Iran, Khuzestan Province 0 0 EN Background: Transfusion-transmi!ed infec"ons (TTIs) con"nue to be a problem in many parts of the world and mul"-transfused pa"ents are at a par"cularly increased risk for TTIs. The aim of this study was to determine the prevalence of Hepa""s C, Hepa""s B, and human immunodeficiency virus (HIV) infec"ons among mul"-transfused pa"ents in Khuzestan Province. Materials and Methods: A cross-sec"onal study was conducted on 349 mul"-transfusion pa"ents referred to the Hemoglobinopathy and Thalassemia Research Center from January 2007 to February 2008. Results: Out of 349 pa"ents, 205 (58.7%) and 144 (41.3%) were men and women, respec"vely. Mean ( ±SD) age of the par"cipants was 18.70±9.18 years. Of these 349 mul"-transfused pa"ents, 206 (59%) had thalassemia, 87 (24%) hemophilia, and 56 (16%) sickle cell anemia. The overall prevalence of HBsAg, an"-HCV, and an"-HIV were 0.6% (95% CI: 0-1.56), 28.4% (95% CI: 27.4-29.3), and 0.9% (95% CI: 0-1.86), respec"vely. The data indicate that an"-HCV posi"vity was significantly associated with an older age (P<0.001), longer dura"on of transfusion (P<0.001), and frequency of transfusion (P<0.001). Conclusion: Although it seems more sensi"ve screening tests and stringent donor selec"on procedures has reduced HCV infec"on, TTIs in mul"-transfusion pa"ents are s"ll a serious risk for these pa"ents. More stringent policy for blood product usage and con"nuous awareness programs for medical staff, general popula"on, and pa"ents are required to reduce the incidence of TTIs.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Retinoic Acid Syndrome: A Case Report and Review 0 0 EN Background: The treatment of acute promyelocy!c leukemia with all-trans-re!noic acid (ATRA) some!mes results in a syndrome characterized by fever, respiratory distress, weight gain, pleural or pericardial effusion, and pulmonary infiltrates. We report the major clinical and radiologic features of ATRA syndrome. Materials and Methods: In the past, occasional case reports and literature pertaining to ATRA syndrome. The purpose of our report and the literature review is to heighten physicians’ awareness of this syndrome, which o$en manifests as nonspecific clinical and radiographic findings. Results: The e!opathogenesis of the syndrome remain unclear. The Incidence of the syndrome has varied in reports from 5% to 27% and the mortality from 5%-29%. The !me of !me onset of ATRAS varies. The reported median !me to the occurrence of ATRA syndrome is 7-12 days. Chest radiographs show increased cardiothoracic ra!o in 64% of the pa!ents, increased vascular pedicle width in 76%, increased pulmonary blood volume in 82%, ground-glass opacity in 57%, consolida!on in 60%, nodules in 60% of the pa!ents. Pleural effusion is noted in 75% of the pa!ents either unilateral or bilateral effusions. Pulmonary hemorrhage is developed in about 20% of the pa!ents during course of ATRAS. Conclusions: Diagnosis of ATRAS manifesta!ons and immediately star!ng cor!costeroids a$er the diagnosis of ATRAS may be improve the pa!ents’ outcome. Because the radiologic features of ATRAS are nonspecific, it would be impossible to differen!ate one from the other based solely on these features. . Prompt administra!on of steroids is cri!cal, not only when the diagnosis is defini!vely established, but also at the first sign of unexplained dyspnea, fever, weight gain, or pulmonary infiltrates.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Imerslund-Grasbeck Syndrome: A Case Report 0 0 EN Introduction: Megaloblasc anemia is an uncommon problem in childhood most frequently associated with vitamin deficiency or gastrointesnal disease. The common causes of megaloblasc anemia are vitamin B12 (cobalamin) deficiency and folic acid deficiency. Familial selecve malabsorpon of vitamin B12 associated with proteinuria firstly was described by Imerslund (1960) and Grasbeck et al (1960) (Imerslund-Grasbeck syndrome).1,2 About 300 paents have been published worldwide, with new paents mostly appearing in eastern Mediterranean countries. However, lots of paents may be misdiagnosed.3 Imerslund-Grasbeck syndrome (IGS), an autosomal recessive disease, is associated with megaloblasc anemia and proteinuria. The diagnosis should be considered when three typical features (macrocyc anemia, decreased serum B12 level, and proteinuria) are present. Symptoms may appear from the 4th month of age (not immediately a!er birth as in transcobalamin deficiency) up to several years later. The cause is a defect in the receptor of the vitamin B12-intrinsic factor complex on the ileal enterocytes. In most cases, the molecular basis of the selecve malabsorpon and proteinuria involves a mutaon in one of two genes, cubilin (CUBN) on chromosome 10 or amnionless (AMN) on chromosome 14. Both proteins are components of the intesnal receptor for the vitamin B12- intrinsic factor complex and the receptor mediang the tubular reabsorpon of protein from intraglomerular filtrate. Management includes life-long vitamin B12 injecons, resulng in a long healthy life. Nevertheless, proteinuria persists. To diagnose the disease, it is important to be aware that cobalamin deficiency affects enterocyte funcon therefore, all tests suggesng general and cobalamin malabsorpon should be repeated a!er resolving the deficiency.3

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 2 4 2010 10 1 Age-specific cutoff in discriminating Iron deficiency anemia from beta-thalassemia traits 197 197 EN