Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 1 1 Ehsan Shahverdi Department of Transfusion Medicine, Institute of Immunology and Transfusion Medicine, University Medicine Greifswald, Greifswald, Germany. AND Blood Transfusion Research Center, High Institute for Research and Education in Transfusion in Medicine, Tehran, Iran Mostafa Moghaddam Blood Transfusion Research Center, High Institute for Research and Education in Transfusion in Medicine, Tehran, Iran Marcel Baschin Department of Transfusion Medicine, Institute of Immunology and Transfusion Medicine, University Medicine Greifswald, Greifswald, Germany 2018 10 27 2018 11 14 Iran is a member of the International Society for Blood Transfusion (ISBT) working party on rare donors since 20102. Recognition of individuals with Rhnull phenotype was reported to the working party in order to be added to the list of countries with Rhnull donors. Iranian database of rare blood groups is followed by international researchers and is important for the Rare Donor Working Party of the International Society of Blood Transfusion (ISBT). Iran is a country with a relatively high prevalence of rare phenotypes and further new blood groups were newly identified. In our published paper, we reported a case study of siblings (a 43-year-old female and her brother) who were strongly suspicious of presenting a rare Rhnull phenotype. Their extended RBC phenotyping showed that they were negative for D, C, E, c, e antigens. We now found out that the brother passed away of unknown causes a few months ago. This means that now we have only one person with this very rare blood group, which raised our concern for supplying compatible blood at the time of need, since the deceased brother was the only donor for his sister. There are two units of frozen RBC donated previously by him in stock. We are almost sure that blood transfusion services might face challenges to provide compatible blood to this individual who lacks all Rh antigens. In conclusion, at present, there is only one individual (a female) patient with the rare Rhnull phenotype in Iran. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1017 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1017/815

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 2 6 EN Hasan Al Houri Department of Internal Medicine, Al Assad University Hospital and Al Mouwasat University Hospital, Damascus, Syria Tagrid Ahmad Department of Internal Medicine, Tishreen Hospital, Damascus, Syria Sarah Zaher Adden Department of Ophthalmology, Al Mouwassat University Hospital, Damascus University, Damascus, Syria Wisam Assad Department of Pathology, Al Mouwassat University Hospital and Syrian Private University, Damascus, Syria Ammar Raiy Department of Kidney Transplant, Al Mouwassat University Hospital, Damascus, Syria 2018 09 29 2018 12 10 T-Cell Rich B-Cell Lymphoma (TCRBCL) is relatively a new entity, lately classified as a morphologic variant of Diffuse Large B-cell lymphomas (DLBCL). It consists (1-3) % of all B-cell lymphomas. The rate is far less when describing cases of primary splenic involvement with TCRBCL. Pathologically, TCRBCL is described as a limited number of scattered, large, atypical b-cells embedded in a background of abundant t-cells and frequently histiocytes. The similarity of this malignancy with other types makes it difficult to distinguish between them. Thus, it needs expertise in both clinical and pathological fields to make the right diagnosis. Here, we present a case of an adult male patient whose first presentation and previous medical history of renal colic misguided the initial diagnosis and suggested another colic episode as the underlying ailment. However, further physical, radiological and histopathological investigations uncovered the presence of primary TCRBCL within spleen with no involvement of other sites. Moreover, unusual pathologic finding of CD3 positivity was proved by immunohistochemistry. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/978 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/978/763

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 7 11 EN Jan Stratmann Department of Hemostaseology, Johann Wolfgang Goethe University of Frankfurt, Frankfurt am Main, Germany Stefan Gundermann 1Department of Hemostaseology, Johann Wolfgang Goethe University of Frankfurt, Frankfurt am Main, Germany Christof Geisen German Red Cross Blood Donor Department, Frankfurt am Main, Germany Alexandra Dukat Department of Hematology and Oncology, Johann Wolfgang Goethe University of Frankfurt, Frankfurt am Main, Germany Wolfgang Miesbach Department of Hemostaseology, Johann Wolfgang Goethe University of Frankfurt, Frankfurt am Main, Germany 2018 06 07 2018 11 18 Background: Autologous stem cell transplantation is considered a standard of care treatment in eligible patients with multiple myeloma, but puts the patient at high risk for infections and bleeding complications. Acquired von-Willebrand's disease (AVWD) and acquired platelet dysfunction are rare bleeding disorders that are associated with lymphoproliferative disorders such as multiple myeloma. Patients with acquired bleeding disorders who are planned for ASCT to treat the underlying condition are considered at highest risk for bleeding complications, and optimal treatment strategies are not known. Materials and Methods: We summarized the diagnostic and therapeutic approach to a patient affected by AVWD and acquired platelet disorder related to multiple myeloma. The patient who was planned for ASCT presented with moderate to severe bleeding symptoms. Results: Acute bleeding episodes were successfully controlled and prevented during induction and consolidation therapy with immunoglobulins, whereas replacement of plasma-derived VW factor showed no clinical improvement. High-dose melphalan-based consolidation therapy supported with autologous stem-cell transplantation led to an immediate and sustainable rise of von-Willebrand antigen and activity and a subsequent normalization of platelet aggregation activity. After a follow-up of 40 weeks, the patient maintained normalized VW levels and platelet aggregation capacity. There were no further signs or symptoms of bleeding. Conclusion: This case report highlights the necessity for combined supportive and causal treatment in patients with AVWD and paraproteinemic PD. High-dose melphalan with autologous stem cell support may function as a treatment option in patients with myeloma-related AVWD. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/958 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/958/764

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2018 07 31 12 19 EN Malihe Bazpour Department of Nursing, School of Nursing and Midwifery, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Mahin Gheibizadeh Nursing Care Research in Chronic Disease, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Amal Saki Malehi Department of Biostatistics and Epidemiology, School of Public Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Bijan Keikhaei Department of Pediatric Hematology and Oncology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran 2018 05 19 2018 07 31 Background: Lifestyle is a key issue in the concept of health promotion. Lifestyle includes all activities that encourage optimum physical, spiritual, and mental functions. The aim of this study was to determine the effect of a training program based on PRECEDE-PROCEED Model on lifestyle of adolescents with beta thalassemia. Materials and Methods: In this clinical trial study, 64 adolescents (age 16-20) who referred to the Thalassemia Center of Ahvaz (2015) were selected and randomly divided into two groups: experimental and control group. The components of the PRECEDE-PROCEED Model were used for planning, implementation and evaluation of the program. Changes in predisposing, reinforcing, enabling factors and lifestyle were immediately and a month after the intervention were assessed by a questionnaire based on PRECEDE-PROCEED Model and the Health-Promoting Lifestyle Profile. Results: The intervention had significantly positive effect on predisposing, enabling and reinforcing factors immediately and a month after the intervention (P < 0.05). Repeated measures analysis of variance showed a significant positive increase in the six dimensions of lifestyle score in the experimental group from baseline to one-month follow-up (P < 0.05). Conclusion: This study showed that the theory-based training program in adolescents suffered from beta thalassemia disease could improve the adolescent’s awareness and attitude of healthy lifestyle. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/947 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/947/765

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 20 24 EN Bita Nakhost Department of Biology, Islamic Azad University, Arsanjan Branch, Arsanjan, Iran Mahboobeh Nasiri Department of Biology, Islamic Azad University, Arsanjan Branch, Arsanjan, Iran Mehran Karimi Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Somayeh Montazeri Thalassemia and Hemophilia Genetic, PND Research Center, Dastgheib Hospital, Shiraz University of Medical Science, Shiraz, Iran 2018 03 02 2018 12 30 Background: Acute lymphoblastic leukemia (ALL) is resulted from the infiltration of high amount of non-differentiated cells in bone marrow. Differentiation of the hematopoietic stem cells into specific cell lineage occurs through a highly regulated pathway which is mainly monitored during transcription step. Expression level and pattern of transcription factors e.g. PU.1 determine fate and developmental phases in this pathway. This study was performed to evaluate the expression level of the PU.1 gene in a group of children suffering from ALL. Materials and Methods: The mRNA expression level of the PU.1 gene was compared between 30 children diagnosed as new cases of ALL and 30 sex- and gender-matched healthy children in the present case-control study. The quantitative real time PCR (qRT-PCR) was used to determine the level of PU.1 gene expression. The data were analyzed using Graph Pad Prism statistical software. Results: The mRNA level of the PU.1 gene was significantly lower in the blood samples of the ALL patients compared to the controls (p= 0.002). Conclusion: The results of the study indicated that the PU.1 gene seemed to have key roles in the differentiation pathway of blood cells. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/924 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/924/766

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 25 34 EN Somayeh Parsa Department of Immunology, School of Medicine, Rafsanjan University of Medical Sciences, Rafsanjan, Iran Sedigheh Sharifzadeh Diagnostic Laboratory Science and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran Ahmad Monabati Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Noorossadat Seyyedi Diagnostic Laboratory Science and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran Reza Ranjbaran Diagnostic Laboratory Science and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran Mohammad Reza Baghbani Diagnostic Laboratory Science and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran Maryam Nemati Department of Laboratory Sciences, School of Para-Medicine, Kerman University of Medical Sciences, kerman, Iran Abdollah Jafarzadeh Molecular Medicine Research Center, Research Institute of Basic Medical Sciences, Rafsanjan University of Medical Sciences, Rafsanjan, Iran. AND Department of Immunology, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran 2018 02 13 2018 12 31 Background: Semaphorins play prominent roles in physiological and pathological processes such as vascular development, tumor growth and immune responses. Semaphorins have different roles in various kinds of cancers, but there is no study concerning their expression in the chronic lymphocytic leukemia (CLL). This study aimed to assess the SEMA3A, SEMA4A and SEMA4D expression in patients with CLL.  Materials and Methods: Peripheral blood specimens were collected from 30 newly-diagnosed untreated patients with CLL and 30 healthy subjects as a control group. The SEMA3A, SEMA4A and SEMA4D expression was determined by real-time PCR method.  Results: The fold change expression of SEMA3A and SEMA4D was 7.58 ± 2.66 and 3.20 ± 0.99 in patients with CLL, and was 1.01 ± 0.31 and 1.00 ± 0.27 in healthy subjects, respectively. The CLL patients expressed higher amounts of SEMA3A and SEMA4D in comparison with healthy subjects (P<0.02 and P<0.03, respectively). The fold change expression of SEMA3A in patients with stage II (11.12 ± 5.35) was also higher than patients with stage I (4.49 ± 1.61, P<0.05). No significant difference was also observed in the expression of SEMA4A and SEMA4D between patients with stage I and stage II CLL. In both CLL and control groups, the fold change expression of SEMA3A was higher in men than in women (P<0.03 and P<0.02, respectively). Conclusion: The results of the study indicated elevated expression of the SEMA3A and SEMA4D in patients with CLL. The SEMA3A expression was influenced by tumor stage and gender of participants.    https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/915 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/915/772

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 1 2019 01 02 35 Lorena Salazar-Cavazos Department of Hematology, Internal Medicine Division, Dr. José E. González University Hospital of the School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Mexico David Gómez-Almaguer Department of Hematology, Internal Medicine Division, Dr. José E. González University Hospital of the School of Medicine of the Universidad Autónoma de Nuevo León, Monterrey, Mexico 2018 12 08 2019 04 14 Background: Lumbar puncture (LP) is a hematology procedure that can require repeated attempts leading to traumatic LP (TLP), which has been related to the central nervous system (CNS) relapse. LP success can depend on the size and anatomy of the patient and the skill of the hematologist. The main objective was to determine the influence of body mass index (BMI) on LP outcomes. Materials and Methods: Adults with lymphoid malignancies requiring LP were included prospectively over one year; hematology residents performed most procedures. A 22-gauge Quincke needle was employed. Comparison between non-traumatic vs. traumatic LPs according to BMI, CNS relapse, and residents’ year was performed. Results: Fifty-four patients with a mean age of 31.5±15.57 years were included. Diagnosis was Acute Lymphoblastic Leukemia-B (74%), Acute Lymphoblastic Leukemia-T (13%) and Non-Hodgkin Lymphoma (13%). 227 LPs were performed, 121 (53.3%) successful, 98 (43.2%) traumatic, 11 (11.2%) TLPs were macroscopically detectable and 87 (88%) microscopic; 8 (3.5%) were dry-taps. Median time between punctures was 11 days (1-202). Median BMI was 25 (22.8-39.6). Main indication for LP was prophylactic (74.5%); 39.2% were performed by first-year, 35.2% by second-year, 19.6% by third-year hematology residents. No difference (p = 0.145) for a TLP was found among residents. A BMI ≥30 (p = 0.040), non-palpable intervertebral space (p = 0.001) and more than one attempt (p = 0.001) were significant for TLP. TLP was not associated with CNS relapse (p = 0.962). Conclusion: Obesity predicted a TLP. A traumatic puncture did not increase the risk of CNS relapse at one-year follow-up. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1039 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1039/795

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 3 2019 07 10 153 163 EN Abeer Awad Department of Biochemistry, Faculty of Pharmacy, Mansoura University, Mansoura, 35516, Egypt Mohamed Ebrahim Oncology Centre, Faculty of Medicine, Mansoura University, Mansoura, 35516, Egypt Laila Eissa Department of Biochemistry, Faculty of Pharmacy, Mansoura University, Mansoura, 35516, Egypt Mamdouh El-Shishtawy Department of Biochemistry, Faculty of Pharmacy, Mansoura University, Mansoura, 35516, Egypt 2018 11 12 2019 03 11 Background: Hepatocellular carcinoma (HCC) is a highly fatal tumor which represents a major health problem worldwide. Due to asymptomatic nature of HCC, most patients present with the progressive stage of the disease, so, unfortunately, there are no effective therapies. Existing techniques for HCC surveillance and diagnosis lack the required accuracy. Therefore, searching for new diagnostic and/or therapeutic tools could improve patient survival. This study aimed to estimate the diagnostic role of Dickkopf-1 (DKK1) and amphiregulin (AREG) and to find out their correlation with different clinicopathological parameters in HCC patients. Materials and Methods: Serum levels of DKK1 and AREG in 55 HCC patients, 20 cirrhotic patients, and 15 healthy subjects as control group were measured using the ELISA technique. Results: Both of DKK1 and AREG showed a significant increase in the HCC group compared to cirrhotic and healthy groups. DKK1 at a cutoff point of 8.92 ng/ml showed that the area under the curve (AUC) was 0.826 with 87.3% sensitivity and 82.9% specificity. DKK1 showed a significant correlation with tumor size, liver dysfunction, and poor performance status in HCC patients. AREG at a cutoff point of 8.74 pg/ml showed a sensitivity of 74.5% but low specificity (47.1%). AREG showed a significant correlation with portal vein thrombosis and tumor metastasis in HCC patients. Conclusion: Serum DKK1 could be a diagnostic biomarker for HCC. Both of DKK1 and AREG may play significant roles in tumor progression and may offer promising therapeutic targets in HCC patients. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1023 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/1023/790

Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 13 3 2019 07 10 164 171 Ali Tayyebi Medical Student, Shiraz University of Medical Sciences, Shiraz, Iran Maryam Poursadeghfard Clinical Neurology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran