1 2008-4595 Iranian Pediatric Hematology and Oncology Society 173 Pediatric Hematology & Oncology Molecular Markers in Neuroblastoma 1 2 2010 2 2 49 60 12 03 2011 Neuroblastoma, one of the common malignant childhood tumors, arises from neuroblast cells derived from theneural crest and destined for the adrenal medulla and the sympathetic nervous system and shows remarkable biologicalheterogeneity, resulting in favorable or unfavorable outcomes. Some tumors make rapid progress with a fataloutcome. In other instances, the tumors regress spontaneously in infants or to differentiate into a benign ganglioneuromain older patients. This heterogeneity within neuroblastoma depends on the molecular characteristics of tumorcells. Several distinct genomic alterations have been found in neuroblastoma, including MYCN amplification, DNAploidy, deletion of the short arm of chromosome 1, gain of chromosome 17q, and deletion of 11q. The difference ofexpression was also found in genes related to cellular growth, differentiation, and apoptosis of neural network includingsignaling by NTRK1 or ALK receptor tyrosine kinases, and telomerase activity. And this presentation discussesdiagnostic and prognostic molecular makers for extensive heterogeneity of neuroblastoma. This should lead to morerisk-adapted therapies according to the genetic markers by which individual neuroblastomas are biologically characterized.
174 Pediatric Hematology & Oncology Effects of Recombinant Urate Oxidase (Rasburicase) and Allopurinol for Prophylaxis and Treatment of Hyperuricemia in Patients with Leukemia and Lymphoma 1 2 2010 2 2 61 66 12 03 2011 Background: Efficacy of rasburicase in pediatric patients with leukemia and lymphoma is proved. This study aims toweigh efficacy and safety of rasburicase versus more conventional therapy, allopurinol, and to compare their safetyand properties in tumor lysis syndrome (TLS) of leukemia and lymphoma patients.Materials and Methods: The study was done with a retrospective cohort design. Patients were selected from ourhematology ward admitted from 2005 through 2008. Patients were put into two groups based on their blood levelsof uric acid, before initiation of chemotherapy treatment group (the Uric Acid level of 6.5 mg/dl or more) and theprophylaxis group (the uric acid level below 6.5 mg/dl). Evaluation of effectiveness of therapy was performed after24, 48, 72-hour, and longer periods.Results: Of 184 patients 69% had leukemia, and 31% lymphoma. Twenty patients were treated with rasburicaseand 164 with allopurinol. Mean age of patients was 7.93± 4.247 years old. 60.8% were male and 39.2% were female.According to Chi-square test results, there was no significant difference between two agents regarding prophylaxis(chi-square = 4.247, p-value = 0.193) and treatment (chi-square = 0.780, p-value = 0.677). Most of the response toeach agent was seen in the first 24 hours after drug administration. Mean level of uric acid reduced from 7.4 to 3.4mg/dl in rasburicase, and from 5.4 to 3.9 mg/dl in allopurinol group. Mean duration of treatment for rasburicase was2 days, and for allopurinol 6 days. Adverse effects were minimal in both groups (in rasburicase 1.6% and in allopurinol5.4%).Conclusion: Rasburicase seems to be highly efficient in both prophylaxis and treatment of Hyperuricemia. Due tohigh costs in our practice, it was only administered to 20 patients with high levels of blood uric acid or leukocytosis.It prepares patients for chemotherapy faster and decreases cost of hospital stay indirectly by lowering cost oftreatment. Allopurinol, alternatively showed equal efficiency and comparable results. Thus, it can be used safely andeffectively until rasburicase becomes more widely available and more cost-effective. 175 Pediatric Hematology & Oncology Invasive Aspergillosis in Pediatric Hematology Oncology Ward 1 2 2010 2 2 67 70 12 03 2011 Background: Patients with prolonged neutropenia and/or severe underlying immunosuppression are at thegreatest risk for disseminated aspergillosis. This study was undertaken to determine the incidence of invasiveaspergillosis by Platelia Aspergillus enzyme-linked immunosorbent assay ELISA kit in high risk children admitted tothe hematology ward of Dr. Faghihi hospital, Shiraz University of Medical Sciences, Iran.Materials and Methods: From Oct. 2006 to Jun. 2008, 62 patients with hematologic malignancies were followedand evaluated for invasive aspergillosis in Shiraz. All clinical samples were cultured and a direct microscopic examinationwas performed. Blood samples were cultured by bedside inoculation to BACTEC medium.Blood samples were collected prospectively once a week and stored at –20 ˚C until examination. All the collectedblood samples were assayed for galactomannan antigen using Platelia Aspergillus ELISA kit. Patients were classifiedaccording to the diagnostic criteria set by the European Organization for Research and Treatment of Cancer-MycosisStudy Group.Results: The female-to-male ratio was 22:40, and mean age of the patients was 9.3 years. The sensitivity, specificity,negative and positive predictive values of the ELISA method were 91% , 90%, 83.3%, and 94.7%, respectively. Galactomannanantigen test was positive in 1 proven, 8 probable, and 2 possible cases. The incidence rate of invasiveaspergillosis was found to be 16.7%.Conclusion: Considering the incidence of invasive aspergillosis and the corresponding morbidity and mortality ratesin patients with hematologic disorders, it seems that more efficient methods are in demand for early diagnosis andthereby promoting the patients’ survival. 176 Pediatric Hematology & Oncology Current Indications of Bone Marrow Transplantation (BMT) in Pediatric Malignant Conditions a Review 1 2 2010 2 2 71 76 12 03 2011 Hematopoietic stem cell transplantation (HSCT) has been practiced for more than 30 years. Hematological malignanciesare the main indications for this treatment. However, its indications in adults are different from children.Advances in chemotherapy and target therapy have improved treatment outcome of some of the very high riskcancers, and changed indications of HSCT in children. Multi-center clinical trials evaluating outcome of childhoodcancers using different therapeutic protocols are needed to precisely define the role of HSCT. A review on the currentliterature about HSCT indications in pediatric cancers is presented in this manuscript. 177 Pediatric Hematology & Oncology Current Treatment Strategy in Langerhans Cell Histiocytosis 1 2 2010 2 2 77 80 12 03 2011 Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granulomaof bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into singlesystem LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostlytreated with local therapy. Multisystem LCH is subdivided into low risk and high risk groups. A 6-week course ofPRED/VBL is recommended for all patients with MS-LCH. Further therapy depends on the response to the initialcourse, and risk group of the patient. 178 Pediatric Hematology & Oncology Features of Bone Sarcomas at MAHAK Children Hospital, Tehran, Iran during 2007-2009 1 2 2010 2 2 81 86 12 03 2011 Background: Bone sarcomas are the most common malignancies of bone tissues in children, and are classified intotwo groups as osteosarcoma and Ewing’s Sarcoma. Treatment and prognosis depend on the subtype and grade ofthe tumor. The goal of this study was to evaluate the features bone sarcoma in patients referred to MAHAK childrenhospital since 2007 to 2009.Materials and Methods: This was a retrospective study of patients with bone sarcoma referred to MAHAKchildren hospital for continuing their treatment or as a new case of cancer. All of the patients had local pain, localswelling, and decreased range of motion and pathologic fractures as the presenting symptom. All patients underwentbone scan, chest computed tomography (CT) scan, chest X- ray, echocardiography, hearing tests, and bonemarrow aspiration/biopsy. All of the data were analyzed by SAS software.Results: In this study, 36 patients with bone sarcoma were included with the age range of 3 to 20 years old (55% maleand 45% female). Analysis showed that local pain and local swelling were the most frequent local manifestations inpatients. The most common primary tumor site was distal femur (30%). Treatment for the patients at MAHAKchildren hospital is performed according to German protocol. Out of 36 patients, 75% were followed until 2008 and25% until 2009.Conclusion: According to the analysis we can conclude that these data are similar to other studies. To achieve thebest results, oncologists have to modify the treatment of patients completely. 179 Pediatric Hematology & Oncology Case Series of Hepatoblastoma 1 2 2010 2 2 87 90 12 03 2011 Background: In the past 20 years, a dramatic improvement in the prognosis of patients with hepatoblastoma (HB)has been achieved by combining surgery and chemotherapy in several national and international trials.Materials and Methods: Four children ( 3 girl and 1 boy) aged 34 days, 4 months, 13 months, and 1 year with meanage of 6.53 (±5.15) months were presented to our center from 2002 to 2009 with abdominal mass and abdominaldistention. Their abdominal ultrasonography revealed liver mass, and pathological diagnosis was hepatoblasma.They were treated with a combination of surgery and chemotherapy.Results: Patients with non-metastatic HB were treated at Shahid Sadoughi Hospital of Yazd. A complete resection ofprimary tumor was achieved in all of them. The median follow- up of the patients was 38.25 (± 29.02) month and allof them (100%) remained alive with complete remission.Conclusion: Complete resection with chemotherapy can improve prognosis of hepatoblastoma patients. 180 Pediatric Hematology & Oncology ALK- negative Anaplastic Large Cell Lymphoma of Bone 1 2 2010 2 2 91 96 12 03 2011