Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Table of contents 0 0 EN

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Members Information Pack 0 0 EN

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Relation between the Short Term Health Related Quality of Life and Various Types of Medical Treatment in Patients with Idiopathic Chronic Thrombocytopenia 45 49 EN M Faranoush KH Arjmandi Rafsanjani Z Sadr AA Hedayatiasl A Mehrvar Background:Chronic Idiopathic Thrombocytopenic Purpura (ITP) is an autoimmune disorder characterized by a low platelet count due to reduced production and higher destruction of the platelets. Therapy is aimed at increasing the patient’s platelet count to safe levels and includes a wide variety of treatments, which has a large impact on the patient’s quality of life. Health Related Quality of Life (HRQoL) is one of the most important factors to evaluate the efficacy of the treatment. This study was conducted to compare the effect of the various types of treatment on HRQoL in ITP patients. Materials and Methods:Ninety under 25 year-old patients were included in this cross-sectional study. All patients had active disease with platelet count of less than 120,000/mm3. The patients were divided into 3 treatment groups: 1- Intravenous immunoglobulin (IVIg) treatment, 2- various corticosteroids treatments and 3- Immunosuppressive treatments, and filled-out a validated “quality of life” questionnaire.The HRQoL was calculated according to the collected information such as well-physical activity, gynecologic problems, bleeding disorders, response to treatment, side effects and social interactions. Results:Ninety patients were enrolled, consisting of 38 male and 52 females. The mean age of the patients was 19.73±4.02 years. The mean total score of HRQoL was 21.8±9.1. We did not find any significant difference in HRQoL total score among treatment groups (p=0.129). Conclusion: HLQoL did not differ among treatment protocols and it seems that other factors are more important in influencing quality of life among ITP patients. Keywords: Purpura, thrombocytopenic, idiopathic, quality of life, immunoglobulins, corticosteroids, immunosuppressive agent

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Side Effects of Hydroxyurea in Patients with Sickle Cell Anemia 51 53 EN A Ghasemi B Keikhaei SJ Sayedi Background: Hemoglobin S arises is the result of a point mutation (A-T) in the sixth codon on the -globin gene on chromosome 11 causing sickle cell anemia. The presence of fetal hemoglobin in infancy plays a relatively protective role for vaso-occlusive symptoms that are the major contributor for the morbidity and mortality among patients with sickle cell anemia. hydroxyurea, an s-phase-specific and non-DNA-hypomethylating chemotherapeutic agent is capable of inducing HbF synthesis. Materials and Methods: We reviewed the records of 28 sickle cell anemia patients, aged 4-52 years, treated with hydroxyurea to study the drug’s side effects. Results: In our study, the most common adverse effect was dermatologic complication which occurred in 15 patients (53.5%). The gastrointestinal side effects were nausea, vomiting, abdominal pain and anorexia occurring in 3 patients 10.7%. The neurologic adverse effects were uncommon and occurred in 4 patients (14.3%). Conclusion: Side effects of hydroxyurea were common but mild to moderate, benign and transient. Starting a low dose of hydroxyurea (10 mg/kg per day) and increasing the dose slowly in pediatric and adult patients with sickle cell anemia can be tolerated well, without serious side effects. Keywords: Side effect, hydroxyurea, sickle cell, anemia.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Allele Frequencies of HLA-A, B and DRB1 among People of Fars Ethnicity Living in Tehran 55 59 EN M Shaiegan F Yari H Abolghasemi N Bagheri M Paridar A Heidari Sh Kaskani F Sabaghi F Dikloo M Zaman-Vaziri Background: Human leukocyte antigens (HLA) are polymorphic cell surface proteins. Distribution of HLA alleles vary among different racial and ethnic populations in unrelated stem cell registries. Determination of HLA allele frequencies in different ethnic groups is useful for population genetic analyses. Materials and Methods: Based on data available from the Iranian Stem Cell Donor Registry, HLA-A, B, DRB1 allele frequencies were evaluated from 244 individuals who were recruited as unrelated volunteer donors by PCR-SSP method in people of Fars ethnicity living in Tehran, Iran. Results: The most frequent alleles found were HLA-A*02(19.8%), HLA-A*03(13%), HLA-A*11 and -24 (12.5%), HLA-B*35(17.7%) HLA-B*51(13.2%), HLA-DRB1*11(20.8%), whereas HLA-A*34 and HLA-A*44 (0.2%), HLA-B*47, B*54, B*56, B*73(0.2%), and HLA-DRB1*09 (0.4%) were the least frequent alleles. Conclusion: Identifying HLA allele frequencies in different ethnic groups, helps in designing a better plan for development of donor centers in different provinces of a country, and a more precise prediction of donor size in the registry, in addition to finding suitable donors for patients in need of hematopoietic stem cell transplantation. Keywords: HLA, unrelated donors, ethnic groups, Iran

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 3 4 2011 8 1 Assessment of Mean Corpuscular Volume as a Surrogate Marker for Detecting Early Iron Deficiency among School Children 61 68 EN SM Abdelsalam ME Awad AE Eldesouky Background: The aim of the present study was to assess the mean corpuscular volume (MCV) as a surrogate marker for detecting early iron deficiency prior to definitive investigation and treatment. Materials and Methods: This study was done on one hundred students in Swada Preparatory School – Sharkia Governorate from 2010 to 2011. They were subdivided into three groups: Group (A): comprised of 31 anemic children. Group (B): comprised of 19 microcytic children. Group (C): comprised the control group of 50 apparently healthy children with normal CBC. All subjects were subjected to: full history taking, thorough clinical examination and iron studies including: serum iron , total iron binding capacity, serum ferritin and transferrin saturation. Results: There was a significant difference among studied subjects in height, presence of anorexia and pallor. Moreover, there was a significant difference among studied subjects in Hb, RBCs count, MCH, RDW (p < 0.001) and WBCs count (p= 0.004). We noticed a significant positive correlation between MCV (fl) and serum iron (mg/dl) in anemic subjects. Also there was a significant positive correlation between MCV (fl) and TIBC (μg/dl) in anemic subjects as well as a significant positive correlation between MCV (fl) and serum ferritin (μg/dl). In microcytic subjects, we found a significant positive correlation between MCV (fl) and iron (mg/dl) and a significant positive correlation between MCV (fl) and ferritin (μg/dl). Conclusion: Every child with clinical manifestations of anemia proved by CBC who have microcytosis and low MCV should undergo iron studies ( serum iron, serum ferritin, total iron binding capacity and transferrin saturation ). Keywords: Iron deficiency , anemia, children.