Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Relation between Estrogen and Progesterone Receptor Status with p53, Ki67 and Her-2 Markers in Patients with Breast Cancer 93 97 EN Robab Sheikhpour Hematology and Oncology Research Center, Shahid Sadoughi University of Medical Science, Yazd, Iran Fatemeh Poorhosseini Department of Immunology, Kerman University of Medical Science, Kerman, Iran Background: Breast cancer is the most common cancer in women, containing approximately one third of all illnesses in women. Assessment of molecular markers is valuable in predicting the outcome of disease and decision making for optimal treatment. The purpose of this study was to determine the relationship between estrogen and progesterone receptors with Her-2, Ki67, P53, and clinicopathological factors in breast carcinoma. Methods: 184 patients with breast cancer were chosen and immunohistochemistry was used for expression of p53 protein, Her-2, Estrogen receptor, Progesterone receptor and Ki67 in breast tissues. For statistical analysis, Pearson’s Chi-square tes and Spearman’s rho were used. Results: Positive staining of estrogen receptor, progesterone receptor, Her-2, Ki67 and p53 was found in 63%, 53.8%, 54.6%, 56.2% and 42%  respectively. Also there was reverse relation between estrogen receptor, progesterone receptor with Her-2 (P<0.05), but there was no relation between estrogen receptor and progesterone receptor with p53 and Ki67 (P>0.05). Also over-expression estrogen receptor was significantly associated with decreased lymph node metastasis and malignancy grade (P<0.05). Also over-expression of progesterone receptor was significantly associated with decreased malignancy grade (P<0.05). Conclusion: Breast cancer progression is often associated with alterations in expressions of estrogen receptor, progesterone receptor, HER-2/neu, p53, and Ki67 and reverse association between hormones receptors and HER2 leads to lower or absent hormone receptors in women with HER2 positive breast cancers. Also positive estrogen receptor status can be associated with better survival in these patients.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Clinicopathological Features of Patients with Non-small-cell Lung Cancer in West of Iran 98 102 EN Mehrdad Payandeh Department of Hematology and Medical Oncology, Kermanshah University of Medical Sciences, Kermanshah, Iran Masoud Sadeghi Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Edris Sadeghi Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Background: Lung cancer is the most common cause of cancer death worldwide with an annual mortality rate of more than 1.3 million worldwide. We aimed to analyze the clinicopathological features of patients with non-small-cell lung cancer (NSCLC) in west of Iran. Methods: 64 patients with NSCLC who referred to our clinic were analyzed. Sex, age, histopathology, location of the tumor, treatment, sites of metastasis and overall survival of the patients were studied. EGFR mutations were analyzed using th DxS kit with Multiplex allele specific real-time PCR×29 primers (ARMS Method), DxS Scorpion and ARMS assay. Results: Mean age of the patients at diagnosis was 60 years. 51 (79.7%) patients were men. 34.3% were in their sixth to seventh decade of life (50-69 years). 40 (68.75%) patients had distant metastasis to bone and liver as the most common sites. Adenocarcinoma, squamous cell carcinoma and large cell carcinoma were observed in 68.8%, 23.3%, and 7.9% of the patients, respectively. 3-year survival rate was 25% and the mean overall survival (OS) was 16 months for all patients. Conclusion: The mean age of Iranian patients with lung cancer in a western province of Iran was between 55-65 years in our study. Metastasis to bone was more common than other studies. Also, the OS in this study was higher than the other studies.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Efficacy of Deferasirox (Exjade®) versus Osveral® in Treatment of Iron Overload in Patients with Beta- thalassemia Major in Iran; A Non-randomized Controlled Trial 103 107 EN Neda Ashayeri Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Elham Sadeghi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sara Sadeghi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Peyman Eshghi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Samin Alavi Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Background: Iron chelators are an important part of management of patients with thalassemia. It is prudent to compare efficacy of different iron chelators in treatment of iron overload in these patients who receive regular blood transfusion. We aimed to compare the efficacy and safety of available oral iron chelator; Deferasirox (Exjade®) with Deferasirox (Osveral®) in reducing iron overload in patients with β-thalassemia major. Methods: Children suffering from β-thalassemia major referring to Mofid Children Hospital were enrolled in this non-randomized clinical trial. The patients were divided into two groups receiving Deferasirox (Exjade®) versus Deferasirox (Osveral®) and their response to either treatment was assessed by measurement of serum ferritin levels and estimation of cardiac and liver iron by MRI T2* of heart and liver. Efficacy of either medication was compared before and after 12 months of treatment. Serum ferritin levels were measured every three months. Mean serum ferritin at baseline was compared with post-treatment values. MRI T2* of heart and liver was performed before and after treatment. Results: Out of 69 patients with a mean age of 13.6±7.4 years, 42 (60.9%) were male. 30 patients were assigned to take Deferasirox (Exjade®) and 39 patients to take Deferasirox (Osveral®). The groups were not different regarding the age and the gender (P=0.18 and 0.621, respectively). There was no statistically significant difference in post-treatment serum ferritin level measurements between the two groups. In patients who received Osveral®, decrease in liver iron overload was significant (0.99 ms in Exjade® group vs 1.16 ms in Osveral® group, p=0.007). In the group of patients who received Exjade®, decrease in cardiac iron overload was significant (4.52 in Exjade group vs. 1.71 in Osveral group, P<0.001). Conclusion: Deferasirox (Osveral®), the iron chelator manufactured in Iran, was as efficient as Deferasirox (Exjade®) in iron removal and could be a substitute for Deferasirox (Exjade®).

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Health-Related Quality of Life in β Thalassemia Major Children in North of Iran 108 111 EN Abbas Jafari-Shakib Guilan Blood Transfusion Organization, Rasht, Iran Ali Davoudi- Kiakalaye Guilan Blood Transfusion Organization, Rasht, Iran AA Pour-Fathollah Iranian Blood Transfusion Organization, Tehran, Iran Reza Jafari-Shakib Department of Immunology, Guilan University of Medical Sciences, Rasht, Iran Zahra Mohtasham-Amiri Department of Community and Preventive Medicine, Guilan University of Medical Sciences, Rasht, Iran Background: Advances in treatment of thalassemia major have improved the life expectancy of the patients and therefore their quality of life as other chronic diseases. This study was conducted to assess health- related quality of life in these patients in Guilan province. Methods: In a cross-sectional study, thirty-one children, aged 8-12 years, with β-thalassemia major were interviewed in Guilan, northern Iran, from January to March 2016. Source of data were health centers of the province and its satellite centers, blood transfusion organizations, general hospitals and private clinics. Health-related quality of life was assessed by using PedsQL questionnaire. The Questionnaire was completed at baseline by all patients and their parents. T and Chi-square tests were used as appropriated.  Results: Of the 31 children, 58.1% were girls. Total summary score in children was 75.9±20.1. Physical, Emotional, social, school and psychosocial functioning scores were 70.6±24, 73.3±22.9, 85.9±21, 74.1±21.5, 77.7±19.7, respectively. None of the children underwent splenectomy. Sex, Serum ferritin and hemoglobin levels did not show any association with quality of life in this study. Conclusion: Although quality of life in these patients was acceptable, HRQOL showed lower scores in comparison to the healthy population. It seems more social and familial support for increasing the quality of life of these children is surely needed.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Frequency of Blood Components Wastage and Associated Factors in Yazd Healthcare Centers 112 116 EN Hayedeh Javadzadeh Shahshahani Associate professor, Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran Naghi Taghvaee Director, Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Yazd, Iran, High Institute for Research and Education in Transfusion Medicine, Yazd, Iran Fatemeh Akhavan Tafti Head of quality assurance, Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Yazd, Iran, High Institute for Research and Education in Transfusion Medicine, Yazd, Iran Background: Excessive ordering of blood components imposes heavy costs to blood transfusion centers and increases the amount of blood product wastage due to lack of their use. In recent years, attempts have been made against unnecessary blood and blood product requests in our country. We aimed to investigate the frequency of blood and blood product wasting in healthcare centers of Yazd city in Iran. Methods: In this cross-sectional study data were obtained from questionnaires, software system of hospital blood banks and data extracted from medical records. PPS (probability proportional to size) was applied as sampling method. Required samples were collected randomly from each hospital. Data were analyzed using descriptive statistics. Results: In total, 171, 147, 465 request forms for fresh frozen plasma (FFP), platelets, and RBC were evaluated. Approximately, 11%, 6%, and 21.5% of FFP, platelets and RBC units were discarded in the hospitals, respectively. The highest rate of blood component wastage belonged to burns (23%), ICU (22%), surgery (20%) and Thalassemia (20%) departments. Expiring which caused the products to be discarded was the most common cause of blood wastage (82%). “cross match to transfusion ratio” which is a standard quotient in transfusion medicine was 2.9-8 in these medical centers. Conclusion: The most common causes of blood wasting were lack of definite indications to transfuse blood products, long term storage of blood products without their consumption or discharging the patients not receiving any transfusion. Therefore, it is possible to reduce blood and blood products wastage through proper management of requests and their rational use.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Diagnostic Utility of Bone Marrow Sampling and Profile of Hematological Abnormalities in Indian HIV-infected Individuals 117 122 EN Sushma N Ramraje Associate Professor, Department of Pathology, Grant Govt. Medical College, Mumbai-400008, India Samir AH Ansari Assistant Professor, Department of Pathology, Grant Govt. Medical College, Mumbai-400008, India Snehal Kosale Department of Pathology, Grant Govt. Medical College, Mumbai-400008, India Background: Hematological abnormalities are a common complication of HIV infection and occur in all stages of the infection. These abnormalities increase as the disease advances. We aimed to evaluate the diagnostic utility of bone marrow sampling in HIV-positive patients. Methods: 40 HIV-infected individuals were screened for hematological abnormalities. Investigations such as iron studies, hematological work-up, bone marrow evaluation and coagulation profile were performed. Results: The most common single hematological abnormality was anemia, seen in 8 (20%) patients. However, anemia was seen as a subset of pancytopenia in 21 (52.5%) patients. Microcytic hypochromic anemia was present in 12 (30%) cases while anemia of chronic disease (normocytic normochromic anemia) occurred in 4 (10%) cases. Macrocytic anemia was observed in 32.5% (n=13) cases. Leucopenia and thrombocytopenia was seen in 21 (52.5%) patients as a subset of pancytopenia. However, they were not present as a single hematological abnormality. 45% of the patients showed hypercellular marrow whereas normocellular marrow was seen in 35% (n=14) and hypocellular marrow in 15% (n=6) of the patients. Myelodysplasia was found in 10 (25%) patients. It was commonest in erythroid (12.5%) followed by myeloid series (10%) and megakaryocytic series (2.5%).  Tuberculosis was seen in 10% of the cases and gelatinous transformation was seen in 1 (2.5%) case.  Conclusion: Bone marrow sampling has diagnostic utility in HIV-infected patients. Morphological examination in HIV-positive patients plays a distinctive role in ruling out the presence of opportunistic infections.

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Simultaneous Neuroblastoma and Acute Lymphoblastic Leukemia in an Infant 123 124 EN Mohammad Naderisorki Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Ahmad Mohammadi Ashiani Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Zahra Khaffafpour Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Iranian Pediatric Hematology and Oncology Society Iranian Journal of Blood and Cancer 2008-4595 8 4 2016 12 1 Kaposiform Hemangioendothelioma in a Newborn with Kasabach-Merritt Syndrome 125 126 EN Shahnaz Armin Pediatric Infections Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Roxana Azma Pediatric Radiology Department, Shahid Beheshti University of Medical Sciences, Tehran, Iran Maryam Kazemi Aghdam Pediatric Pathology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran