Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 93 98 EN Pardis Nematollahi Department of Pathology, Cancer Prevention Research Center, Isfahan University of Medical Science, Isfahan, Iran AND Department of Pathology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran Azar Baradaran Department of Pathology, Isfahan University of Medical Science, Isfahan, Iran Zahra Kasaei Koopaei Department of Pathology, Isfahan University of Medical Science, Isfahan, Iran Hamidreza Sajjadieh Department of Internal Medicine, Isfahan University of Medical Science, Isfahan, Iran 2019 04 23 2019 09 02 Background: Acute myeloid leukemia (AML) is the most prevalent acute leukemia in adults. Bone marrow angiogenesis is crucial for pathogenesis of leukemia, and increasing bone marrow Mean Vascular Density (MVD) and level of angiogenesis factors are seen in patients with AML. Higher level of bone marrow MVD is associated with poor prognosis of AML according to previous studies. The present study aimed to compare bone marrow MVD in AML patients and controls and evaluate the relation between bone marrow MVD and number of residual blast cells after AML treatment. Materials and Methods: This study is a longitudinal study on AML patients who were admitted to Omid hospital. The bone marrow biopsies of patients with AML and patients with normal diagnosis –as control group- were taken from archives of pathology laboratory. Immunohistochemistry staining was used for all specimens by using thrombomodulin markers for calculating MVD. Flow cytometry findings of AML patients were assessed for percent of minimal residual disease (MRD) after AML treatment in AML patients group.                                                                 Results: In this study, 27 AML patients and 24 healthy individuals with mean age of 40.92±15.13 years were evaluated, of whom 56.86% were male. The mean bone marrow MVD was significantly higher in AML patients than controls. The mean bone marrow MVD was significantly higher in males and there was insignificant reverse correlation between bone marrow MVD and MRD. About 59.3% of AML patients had response to treatment and there was no significant relationship between MVD and response to treatment.                      Conclusion: Bone marrow MVD was higher in AML patients than controls and there was no remarkable relationship between bone marrow MVD and MRD and response to treatment. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1111 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1111/817

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 99 109 Halimeh Khoshamooz Department of Hematology and Blood Banking, Faculty of Medicine, Tarbiat Modares University, Tehran, Iran Saeid Kaviani Department of Hematology, Faculty of Medicine, Tarbiat Modares University, Tehran, Iran Amir Atashi Department of Basic Sciences, School of Medicine, Shahroud University of Medical Sciences, Shahrood, Iran Seyed Hossein Mirpour Hassankiadeh Department of Internal Medicine, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran 2019 08 05 2019 10 07 Background: Acute T lymphoblastic Leukemia (T-ALL) is a highly aggressive hematologic malignancy. Chemotherapy resistance is one of the most important challenges in T-ALL treatment. Alterations in cellular signaling pathways such as Notch1 and PI3K / AKT / mTOR play a role in cell proliferation, survival, and resistance to chemotherapy. Combination of Notch1 and PI3K / AKT / mTOR inhibitors is an interesting and rational strategy in treatment of T-ALL. Interaction of AZD5363 as an inhibitor of PI3k / AKT / mTOR and Compound E as an inhibitor of Notch1 signaling pathway was investigated in a T-ALL pre-clinical model. Materials and Methods: T-ALL cell lines included Jurkat, Molt-4, and HPB- ALL cells were treated with AZD5363 and Compound E alone and in combination. Cell viability was determined by MTT assay. Flow cytometry was used to measure apoptosis. Interaction between AZD5363 and Compound E was assessed by Chou-Talalay method. Results: Combination treatment with AZD5363 and Compound E decreased cell viability with synergistic effect in all cell lines at 72 hours. Drug combination increased apoptosis even in Jurkat and HPB-ALL cells resistant to Compound E and AZD5363, respectively. Conclusion: Combination of AZD5363 with Compound E in T-ALL cell lines exhibited a synergistic effect. Cytotoxicity of drug combination increased in all T-ALL cell lines compared to each as a single drug. Simultaneous inhibition of Notch1 and PI3K / AKT signaling pathways as a possible treatment of T-ALL, provides a basis for future investigations. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1166 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1166/818

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 110 117 Poonam Tripathi Department of Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, India Sarita Agarwal Department of Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow 226014, Uttar Pradesh, India Aditya Sarangi Department of Biomedical Informatics Center, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, India 2019 07 11 2019 12 07 Objective: A genetic polymorphism of 50 bp insertion/deletion (Ins/Del) (rs 36232792) in the promoter region of the SOD1 was reported to influence the enzyme activity. The aims of the present study are to evaluate the status of this polymorphism on the SOD1 levels in human peripheral blood cells and its association with risk of oxidative stress in beta thalassemia major patients. Material and Methods: The study was carried out on 200 thalassemia major patients and 200 healthy controls healthy. The SOD1 genotypes were determined using a polymerase chain reaction (PCR)-based method. Serum SOD activity were assessed using SOD assay kit. In-silico analysis was assessed using loss-of-function (LofTool) (PMID: 27563026). Results: No association was found between the insertion/deletion (Ins/Del) polymorphism of SOD1 and risk of oxidative stress in thalassemia major patients Conclusion: The results of this study indicated that the oxidative sterss is not affected by the Ins/ Del polymorphism of SOD1 in thalassemia major patients. Further research with larger samples size and with other genes of antioxidant system is required. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1156 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1156/819

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 118 122 Mohammad Mahdi Adib Sereshki Department of Hematology and Oncology, Iran University of Medical Sciences, Tehran, Iran Babak Bahar Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran Ardeshir Ghavamzadeh Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran Seyed Asadollah Mousavi Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran Kamran Alimoghaddam Hematology, Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran 2019 01 22 2020 01 07 At present, hematopoietic stem cell transplantation is the only curative treatment for β thalassemic patients. Conventional myeloablative stem cell transplantation is associated with significant morbidity and mortality, and non-myeloablative stem cell transplantation is associated with high graft failure rate. Some modification in this treatment approach can result in successful transplantation in thalassemic patients. Two successful Fludarabine - based non-myeloablative stem cell transplantation in two Class III β thalassemic patients are reported here. The first patient was a 14-year old girl that developed rapid engraftment and full Chimerism after rapid tapering of cyclosporine as graft-versus-host disease (GVHD) prophylaxis drug according to our protocol. Another patient was a 24-year old female patient that developed cyclosporine toxicity, and early tapering of cyclosporine helped for rapid engraftment and successful transplantation. After these two successful experiments in non-myeloablative peripheral blood stem cell transplantation for our class III β thalassemic patients, we concluded that Fludarabine-based nonmyeloablative stem cell transplantation with adequate number of stem cells at the time of transplantation and rapid tapering of GVHD prophylaxis drugs after transplantation can potentially help for rapid engraftment and successful stem cell transplantation in high risk β-thalassemic patients. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1062 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1062/820

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 123 126 Flávia Russo 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Maria Cristina Macedo 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Pedro Fernandes 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Larissa Okada 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Lucas da Silva Faculdade de Ciências Médicas Santa Casa de São Paulo, São Paulo, Brazil Camila Simões 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Jamilla Cavalcante 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Aline Simões 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Manuella Almeida 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Maricy Lopes 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil Roberto da Silva 1) Instituto Brasileiro de Controle do Câncer (IBCC), São Paulo, Brazil 2) Bio Sana`s serviços Médicos, São Paulo, Brazil 2019 07 27 2019 11 09 Acute Lymphoblastic Leukemia is a very aggressive malignant disorder of lymphoid cells in adults, with recurrence (30 to 60% of the cases) after the initial treatment. Until this moment, there is no gold standard therapy for the treatment of adult patients with acute relapsed/refractory lymphoblastic leukemia. In this case report, we describe two cases of relapsed leukemia: one of lymphocytic leukemia B and one of trilineage leukemia, which presented a satisfactory response to treatment with Bortezomib associated with Vincristine, Dexamethasone, and Bendamustine. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1161 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1161/821

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 127 129 Marjan Shakiba Department of Pediatric Endocrinology and Metabolism, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahin Shamsian Pediatric Congenital Hematologic Disorders Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Hamid Malekzadeh Department of Pediatric Endocrinology and Metabolism, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mehrdad Yasaei Department of Pediatric Endocrinology and Metabolism, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2019 07 30 2019 09 02 Ghosal hematodiaphyseal dysplasia (GHDD) is a rare autosomal recessive disorder presenting with steroid-responsive anemia and diaphyseal dysplasia of long bones. We report a 3-year-old Iranian girl with refractory anemia, splenomegaly and radiologic signs of metadiaphyseal dysplasia in long bones. The diagnosis was established by clinical presentation and X-ray bone survey. The patient was treated with oral prednisolone therapy with considerable improvement in anemia and splenomegaly. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1162 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1162/822

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 14 2 2020 04 07 130 139 EN Mehdi Mohammadi Kanesbi Cancer Molecular Pathology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Lida Jarahi Department oime of 8 to 10 months. Recent reports have identified that anti HER2 target therapy improved the median survival rate in patients with HER2 positive GC; but a wide range of HER2 overexpression incidence has been reported in different studies. This study was conducted to determine the incidence of HER2 overexpression and its relationship to tumor characteristics. Methods: All gastric or GEJ adenocarcinoma patients who underwent a curative surgical resection in Firoozgar Hospital, Tehran, Iran between 2010 and 2011 were entered into this cross-sectional study. The formalin-fixed and paraffin wax-embedded tissue blocks used in the present study were surgical resection specimens obtained from 115 patients. All tumor samples were examined for HER2 expression by an immunohistochemistry (IHC) assay and HER2 IHC score 3+ was considered positive. Results: The study sample consisted of 115 subjects, 81 males and 34 females with a mean age of 61.01 years (30-85 years, SD=11.605). HER2 overexpression was detected in 13 (11.3%) of the patients. HER2 overexpression was significantly higher in tumors ≥5cm compared to those <5cm (P=0.01). But, there was no significant relationship between tumor histological subtype, GC stage or tumor location and HER2 overexpression (P=0.607, 0.745, 0.491). Conclusion: The findings of the present study indicated that the incidence of HER2 overexpression in gastric adenocarcinoma was 11.3%. The higher incidence of HER2 overexpression in patients with tumors ≥5cm maybe indicate that anti HER2 target therapy administration for these patients could be more beneficial. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/358 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/358/326

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 6 4 2015 10 13 30 32 EN Zineb Benbrahim Christine Haie-Meder Pierre Duvillard Omar El-Mesbahi Axel Le-Cesne Patricia Pautier 2015 10 13 2015 10 13 Ewing’s sarcoma is a round cell malignancy of bone and soft tissue that occurs predominately in adolescents and young adults.It is an uncommon malignancy, but is recognized as the second most prevalent primary bone tumor worldwide. Extraosseous Ewing’s sarcoma is extremely rare and can affect the skin, soft tissues, or viscera. Prognostic and therapeutic features of Ewing’s extraosseous tumors are similar to those of Ewing’s sarcoma. A primary Ewing’s sarcoma arising from the cervix is highly rare. Most of these patients presented with abnormal vaginal bleeding. We report a case of extraskeletal Ewing’s sarcoma arising in the cervix in a 25-year-old woman. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/359 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/359/327

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 6 4 2015 10 13