Screening of von willebrand disease in iranian women with menorrhagia

Results: Mean age of our patients was 32.5 ± 10.6 years. The level of von Willebrand factor in 22.5 and von Willebrand activity in 19.6 of patients was abnormal. The prevalence of vWD among patients with menorrhagia was 24. Conclusions: The high prevalence of vWD among our patients was the same as other previous reports, suggesting low awareness about this disease and under diagnosis of mild cases. Background: Menorrhagia is a common health problem in women, particularly those with bleeding disorders. Little is known about the course of menorrhagia or other bleeding symptoms in women with the most common congenital bleeding disorder, von Willebrand disease (vWD). Objectives: The aim of this study was to estimate the prevalence of vWD in women with diagnosed menorrhagia. Materials and Methods: In this cross-sectional study, a total of 460 consecutive patients, presenting menorrhagia, were analyzed. The initial screening and confirmation tests for the diagnosis of vWD included determination of prothrombin time (PT), partial thromboplastin time (PTT), bleeding time (BT), fibrinogen, factor VIII, vWF antigen, and vWF activity. A questionnaire was filled for every patient. The data were then analyzed using the SPSS software. © 2015, Iranian Red Crescent Medical Journal

Polymorphism and synergism of angiotensin-converting enzyme (ACE) and plasminogen activator inhibitor-1 (PAI-1) genes in coronary artery disease

Introduction: Among the genetic factors for coronary artery diseases, PAI-1 4G/5G and ACE I/D polymorphisms can be noted. This study was carried out to investigate the association of these two polymorphisms and their synergism in coronary artery disease (CAD) from a sample of the Iranian population. Materials and methods: Sixty-one patients with a history of CAD and 92 healthy controls participated in our study. After DNA extraction from leukocytes, PCR was performed to characterize PAI-1 4G/5G and ACE I/D polymorphisms, using an amplification refractory mutation system technique. Results: In the studied patients, PAI-1 polymorphisms were 24.6, 45.9, and 29.5 for 4G/4G, 4G/5G and 5G/5G, respectively; the values for controls were 20.7, 42.2 and 37.0. The distribution rates of genotypes I/I, I/D and D/D in patients accounted for 29.5, 45.9 and 24.6; in the control group these figures were estimated to be 40.2, 40.2 and 19.6. Conclusion: Single and multivariate analyses showed a significant difference for the conventional risk factors, including hypertension, diabetes, hyperlipidemia, smoking and family history, for CAD between patients and controls (p value 0.001). However, no significant correlation was demonstrated considering ACE and PAI-1 polymorphisms either in association with 4G/4G or D/D genotypes or a combination of them in the Iranian population in the current study. © 2015 The Author(s)

A Comparison between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients with Congenital Factor VII Deficiency

In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatment of congenital factor VII (FVII) deficiency, patients received either agent at 30 1/4g/kg, intravenously per week for 4 weeks, in a randomized fashion. The primary aim was to compare FVII:coagulation activity (FVII:C), 20 minutes after recombinant activated FVII (rFVIIa) injection, in the 2 groups. A secondary measure was self-reported bleeding. The median interquartile baseline range of the plasma level of activated FVII (FVIIa) activity in the 2 groups was 1.6 (1.1-14.0) IU/dL and 5.0 (1.1-25.5) IU/dL. All patients achieved levels of FVIIa (FVII:C) >30 IU/dL, 20 minutes after the injection of rFVIIa. Bleeding was similar between the 2 groups, with a comparable decrease in severity and frequency compared to the last month prior to treatment. AryoSeven is similar to NovoSeven in increasing postinjection FVIIa activity as well as in clinical safety and efficacy. © The Author(s) 2014

Gonadal function and fertility in males survivors treated for Hodgkin's disease in Iran

Objectives: To investigate the effect of chemotherapy on gonadal function of young men cured of childhood Hodgkin's disease. Methods: Young adult males surviving Hodgkin's disease, aged 17 and over at least 2 years after therapy were studied in Ali Asghar Children's Hospital, Tehran, Iran from March 2000 to March 2005. Clinical evaluation for secondary sexual characteristics, semen analysis, follicle stimulating hormone (FSH), luteinizing hormone (LH), and testosterone was studied in 33 survivors of Hodgkin's disease. Results: The age at diagnosis was 5-15 years, median 9 years, age at study 17-29 years, median 19 years old. The median duration off therapy was 7 years (2-20 years). All 33 patients received chemotherapy as follows: 32 patients received nitrogen mustard (mechlorethamine), vincristine (Oncovin), procarbazine, prednisone (MOPP)/doxorubicin (adriamycin), bleomycin, vinblastine, dacarbazine (ABVD) 6-8 cycles, 5 of whom after relapses received other protocols. One received only MOPP. Twenty-seven (81.8) had azoospermia, 2 had severe oligospermia, 3 had oligospermia, and one had normal sperm count (58000,000). All patients had normal secondary sexual characteristic. The FSH, and LH in 6/33 patients were above normal. Testosterone in 3/33 was below normal. Conclusion: A prepubertal status does not protect the gonads from the harmful effect of chemotherapy, and approximately 87 of male survivors of Hodgkin's disease develop azoospermia or severe oligospermia

Features of childhood acute myeloid leukemia in Iran: A report from double center study

Acute Myeloblastic Leukemia is one of the important malignancies in children. For better managing the prognosis of this disease, there should be enough information about common features of this malignancy. The aim of this study was to evaluate these common features in children with Acute Myeloblastic Leukemia. A total of 104 eligible children less than 15-year-old have been referred from 2007-2011 to two referral centers for childhood malignancies. Basic epidemiological information recorded in checklists for each individual. Analyzes have been done by SPSS version 22. Out of patients, 57 cases were males (54.8). The male/female ratio was 1.2. The mean age of patients was 6.5 ± 4.3 years. The majority subtypes of patients were M3, M4, non-M3, and M2, respectively. The common molecular abnormalities were t (15;17) and inv (16). Of patients, 19.2 had an early relapse. The mean age of relapse in patients was 6.7 ± 3.9 years. Sixty patients (57.7) were alive, and 44 cases (42.3) died during or after therapy. The three years overall survival rate of patients was 42 in this study. According to our data, AML has the same frequency as compared with data from developing countries. But different epidemiological characteristic was a lower rate of three years overall survival in patients. These data may serve the health authorities for more effective environmental and preventive measurements, purposeful allocation of resources for facilitating upto-date diagnostic and treatment modalities, psychological support programs for respective family members and educational purposes. © 2015 Tehran University of Medical Sciences. All rights reserved

Association between p53 codon 72 polymorphism and systemic lupus erythematosus

Aim: Systemic lupus erythematosus (SLE) is a systemic vasculitic disorder, with multiple genes involved in the disease pathogenesis. The p53 gene plays an important role in controlling the cell cycle. We aimed to study the prevalence of p53 polymorphism in SLE patients and analyze the relationship between the p53 polymorphism and clinical-laboratory features of the disease. Material and methods: This case-control study was conducted on patients with confirmed SLE at Namazi Hospital, Shiraz, Iran. Seventy-seven patients with SLE including 9 (11.8) men and 68 (88.2) women with mean age of 25.61 ± 10.69 years and 80 healthy controls with mean age of 51.82 ±14.25 years were included. The patients' information, including the epidemiological profile, disease history, disease symptoms and also the laboratory findings, were extracted from the hospital records. The p53 expression was determined in lyzed lymphocytes. The data were analyzed using SPSS software version 14.00 for Windows considering p < 0.05 as statistically significant. Results: The frequencies of Arg/Arg, Pro/Pro and Arg/Pro among normal controls were 38.8, 28.8 and 37.5, respectively, but in the patients, Arg/Arg, Pro/Pro and Arg/Pro genotypes frequencies were shown to be 29.2, 12.3 and 58.5, respectively. Thus, heterozygous form of this polymorphism was shown to be associated with the disease more than the homozygous alleles. There was a significant relationship between the different allele types of p53 and some clinical features of SLE. There was no association between the different allele types and any of the initial manifestations of the disease and the laboratory findings, as well. Conclusions: In an Iranian population the functional oncoprotein of p53 with codon 72 polymorphism may play an important role in the pathogenesis and clinical presentation of SLE

A double-blind, randomized comparison study between Zytux� vs MabThera® in treatment of CLL with FCR regimen: Non-inferiority clinical trial

Background: Chronic lymphocytic leukemia (CLL) is characterized by accumulation of B cells in blood, lymphoid tissues and bone marrow. Addition of rituximab to CLL chemotherapy regimens has been associated with improved survival. The aim of this study was to establish efficacy and safety of Zytux� in comparison to MabThera® in treatment of CLL. Materials and Methods: Seventy CLL patients who met the criteria for entering the study were randomized into two groups (35 patients in each group). Both groups received Fludarabine and Cyclophosphamide plus Rituximab as part of the FCR regimen. Group A was treated with Zytux�, and group B was treated with MabThera®. A non-inferiority margin of 20 for the primary outcome was defined to examine the similarity between Zytux� and MabThera®. Results: Baseline demographic characteristics showed no statistically significant difference between the two groups. The two treatment groups were comparable in terms of laboratory and clinical findings, cellular index changes and CD (5, 19, 20 and 23) counts during therapy cycles and at the end of the treatment period. Regarding safety results, Zytux� demonstrated a similar profile of adverse reactions in comparison to MabThera®. Moreover, the overall response rate was 88 and 89 for Zytux� and MabThera®, respectively (CI -0.17, 0.18). Conclusion: Results showed non-inferiority of Zytux� in terms of efficacy and adverse events as a biosimilar version of MabThera®. © 2018, Tehran University of Medical Sciences (TUMS). All rights reserved

Immunophenotypic subtyping of leukemic cells from Iranian patients with acute lymphoblastic leukaemia: Association to disease outcome

Background: Immunophenotypic characterization of the leukemic cells has been widely used as a tool for diagnosis, classification, stratification and prognosis of leukaemia. Objective: To investigate the immunophenotypic subtype profiles of Iranian patients with acute lymphoblastic leukemia (ALL) and its association to disease outcome. Methods: In this study, a total of 60 Iranian patients with ALL were immunophenotyped by flow cytometry using a panel of monoclonal antibodies specific for CD2, CD3, CD5, CD10, CD13, CD14, CD19, CD20, CD33, CD34, CD45, HLA-DR and TdT molecules. Results: The samples were initially categorized into T-ALL (n=9), B-ALL (n=50) and mixed lineage (n=1) based on the expression patterns of CD3 and CD19 molecules. B-ALL patients could further be classified into four subtypes, including Pro-B (n=7, 11.7), Pre-B I (n=28, 46.7), Pre-B II (n=13, 21.7) and immature/mature B cells (n=2, 3.3) on the basis of expression of CD10, CD19, CD20, HLA-DR and TdT. Clinical manifestations and laboratory findings of the patients did not reveal association with immunophenotypic subtypes of ALL, with the exception of mediastinal mass and WBC count at the time of diagnosis which were found to be significantly higher in patients with T-ALL compared with BALL (p=0.001 and 0.014), respectively. Conclusion: Our results indicate that overall the immunophenotypic profile of Iranian ALL patients is similar to previous reports and it might be used for monitoring of minimal residual disease and prognosis

A double-blind, randomized comparison study between Zytux� vs MabThera® in treatment of CLL with FCR regimen: Non-inferiority clinical trial

Background: Chronic lymphocytic leukemia (CLL) is characterized by accumulation of B cells in blood, lymphoid tissues and bone marrow. Addition of rituximab to CLL chemotherapy regimens has been associated with improved survival. The aim of this study was to establish efficacy and safety of Zytux� in comparison to MabThera® in treatment of CLL. Materials and Methods: Seventy CLL patients who met the criteria for entering the study were randomized into two groups (35 patients in each group). Both groups received Fludarabine and Cyclophosphamide plus Rituximab as part of the FCR regimen. Group A was treated with Zytux�, and group B was treated with MabThera®. A non-inferiority margin of 20 for the primary outcome was defined to examine the similarity between Zytux� and MabThera®. Results: Baseline demographic characteristics showed no statistically significant difference between the two groups. The two treatment groups were comparable in terms of laboratory and clinical findings, cellular index changes and CD (5, 19, 20 and 23) counts during therapy cycles and at the end of the treatment period. Regarding safety results, Zytux� demonstrated a similar profile of adverse reactions in comparison to MabThera®. Moreover, the overall response rate was 88 and 89 for Zytux� and MabThera®, respectively (CI -0.17, 0.18). Conclusion: Results showed non-inferiority of Zytux� in terms of efficacy and adverse events as a biosimilar version of MabThera®. © 2018, Tehran University of Medical Sciences (TUMS). All rights reserved

Overexpression of orphan receptor tyrosine kinase Ror1 as a putative tumor-associated antigen in Iranian patients with acute lymphoblastic leukemia

Receptor tyrosine kinases (RTKs) are a group of enzymes involved in a variety of physiological and pathological processes. The human Ror1 is a member of the RTK family with unknown ligand and biological function. Overexpression of Ror1 has recently been reported in B-cell chronic lymphocytic leukemia. The aim of this study was to explore the expression profile of Ror1 in acute lymphoblastic leukemia (ALL) cells. Therefore, leukemic cells were isolated from the bone marrow and/or peripheral blood (PB) of 57 ALL patients. Immunophenotyping was performed by flow cytometry and mRNA expression was detected by RT-PCR. Overexpression of Ror1 mRNA was detected in 23 of 57 (40) ALL patients. A similar expression pattern was observed in ALL cell lines, with 4 of 12 (33) being positive. Stimulation of normal PB mononuclear cells with pokeweed mitogen and phorbol myristate acetate induced substantially higher Ror1 mRNA expression compared to unstimulated cultured cells. There has been neither a significant association between Ror1 expression and the immunophenotypic profile of the leukemic cells, nor with other clinical or hematological features of the patients. In conclusion, our findings propose Ror1 as a new tumor-associated antigen and a potential tool for targeted immunotherapy and monitoring of minimal residual disease in ALL. Copyright © 2008 S. Karger AG