Background: Data on frequency distribution of ABO Rh blood group in pediatric dengue patients are not available. Dengue disease an emerging arthropod borne infection of public health concern belongs to Flaviviridae. The studies defining the relationship between blood groups and dengue disease and its severity are limited. Blood group antigens are generally known to act as receptors for various etiological agents. Hence, we hypothesized there will be an association between blood group and dengue disease and its severity and conducted this case-control study. Study Design: Hospital-based case-control study. Materials and Methods: Study was conducted in department of pediatrics, 244 pediatric subjects (age group 1 - 13 years) were enrolled divided into cases who were admitted cases of dengue (119, 65 males, 64 females) and controls who were attending outpatient department for various other ailments. (125, 67 males, 58 females) and demographic data (age, gender, blood group, and dengue infection status) were collected from them. The risk of acquiring dengue disease and severity and its association with factors such as blood group, gender were analyzed statistically. Results: The data of this study showed a possible association between blood groups of the study population with dengue infection. We observed that dengue infections were higher in individuals with O positive blood group 42.8% when compared with controls 32%. (P = 0.043) But blood groups were not associated with severity of infection. These data present further evidence for the association of the blood groups, gender to susceptibility to dengue infection. Further studies are needed to confirm these findings. Conclusion: Dengue disease is more common with blood group O. But severity of the disease is not associated with any blood groups.

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INTRODUCTION: Sickle cell anemia (SCA) is an autosomal recessive illness caused by the formation of abnormal hemoglobin S. Sickle cell disease has many complications such as vaso-occlusive crisis (VOC), gallstones, stroke, acute chest syndrome (ACS), and others. OBJECTIVES: This study aimed to describe the epidemiology and characteristics of sickle cell patients admitted to hospitals in Jazan region. MATERIALS AND METHODS: A cross-sectional study conducted in four hospitals in Jazan region including King Fahad Central Hospital, Prince Mohammed Bin Nasser Hospital, Sabya, and Abu-Arish General Hospital to estimate the prevalence of admitted sickle cell patients and their characteristics. All patients admitted to medicine and pediatric wards from July 15, 2018, to August 15, 2018, were included in the study. Data were collected by interview. RESULTS: Four hundred and two of 446 patients have agreed to participate with a response rate of 90%. Ninety-one (22.6%) were SCA patients, among them, 45 (49.5%) were male and 46 (50.1%) were female, with a mean age of 18.81 ± 11.05 years. Most of them were admitted due to VOC 56% (n = 51) and ACS 12.1% (n = 11). Almost all sickle patients had VOC 91.2% (n = 83), and more than half of them had ACS 58.2% (n = 53) as a complication. CONCLUSIONS: SCA was the number one cause of admissions in this study. Approximately, one out of each four patients admitted to Jazan hospitals is a sickle cell patient. VOC represents the most frequent complication of sickle disease, followed by ACS. Further studies and interventions to reduce this burden and improve sickle cell patients' health status are recommended.

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OBJECTIVES: This study's objectives are to (1) determine the current prevalence of iron-deficiency anemia (IDA) and risk factors for female college students at Prince Sultan Military College of Health Sciences (PSMCHS) in the Eastern Province of Saudi Arabia and (2) address the gap in the current literature. A written survey in both Arabic and English was administrated to 214 female students, and only 201 students completed the survey. It was randomly selected from all registered female students. A number of factors possibly associated with IDA were examined using the Chi-square test. CONTEXT: Globally, iron deficiency is known to be the most common nutritional disorder. About 30% of the world's population are iron deficient (ID). Women seem to be more affected with IDA than men, which constitute an epidemic public health issue. AIMS: The aim of the study was to determine the prevalence of IDA and the risk factors among healthy Saudi undergraduate female college students studying at PSMCHS in Dhahran. SETTINGS AND DESIGN: A cross-sectional study on 201 female students (18–25 years) was conducted between January and March 2019. SUBJECTS AND METHODS: A questionnaire which contains sociodemographic data was completed by each participant. Two blood samples were collected from each participant to estimate the iron profile and to analyze the complete blood counts. STATISTICAL ANALYSIS: The statistical analysis was carried out using the Statistical Packages for the Social Sciences software. RESULTS: The overall prevalence of IDA was 35.3%. The questionnaire analysis of the dietary habit and clinical characteristics revealed that family history of hereditary disease and physical activity have a significant effect on the development of IDA. Statistical analysis showed that having breakfast regularly significantly reduced the development of IDA compared with irregularly having breakfast. CONCLUSIONS: The prevalence of IDA in this study was moderately high, and our results suggest that further education regarding IDA is highly encouraged. This is the first study that demonstrated the prevalence and risk factors of IDA among undergraduate female college students in PSMCHS, and the data generated will provide a database for further studies.

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Tuberculosis (TB) is a mycobacterial infectious disease that can affect any organ system. The wide varieties of manifestations that TB can present with make the disease a challenge for treating physicians. Anemia is commonly seen in patients with TB, and it is usually anemia of chronic disease. However, the occurrence of autoimmune hemolytic anemia in the setting of TB infection is rare.

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Background: Thalassemias and hemoglobinopathies are highly prevalent in India. Identification of these disorders is important for epidemiologic purposes and for prevention of thalassemia major and clinically severe hemoglobinopathies. Objectives: The aim of this study was to determine the prevalence of thalassemias and hemoglobinopathies in patients of a tertiary care hospital of West Bengal. Materials and Methods: A prospective study was undertaken in which 90,210 cases were included over a period of 8 years. Clinical history and family history were obtained from each patient. The venous blood samples were analyzed for complete blood count, liver function tests, serum iron, ferritin, cobalamin and folate levels. High-performance liquid chromatography (HPLC) was performed on the samples with Biorad Variant using beta thalassemia short program. Confirmatory tests were done whenever required. Results: Normal hemoglobin (Hb) pattern was observed in 79,897 (88.57%) cases and abnormalities were detected in 10,313 (11.43%) patients. β (beta) thalassemia trait was the most common abnormality found in 3870 (4.29%) patients. HbE trait was found in 2418 (2.68%) cases, and then Eβ thalassemia in 1406 (1.56%) patients and β thalassemia major/intermedia in 1135 (1.26%) cases. Other variants detected included sickle cell trait, HbE disease, sickle cell disease, sickle β thalassemia, HbD-Punjab trait, double heterozygous state of HbS and HbE, double heterozygous state of HbS and HbD, Hb Lepore, HbJ-Meerut and HbH. Conclusion: Premarital and antenatal screenings are important measures to prevent birth of children with severe Hb disorders. HPLC is a rapid and reliable technique for identification of various Hb fractions.

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Primary renal Burkitt lymphoma (BL) is an unusual occurrence in children. Although the prognosis of this tumor is poor, early diagnosis on biopsy confirmed by immunohistochemistry (IHC) and followed by chemotherapy improves survival of such patients. We present a case of 4-year-old male child with bilateral renal lump suggestive of lymphoma on computerized tomography. Renal biopsy and subsequent IHC was suggestive of bilateral BL in the kidney; following which the child was started on FAB LMB 96 based treatment protocol.

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Introduction: Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide. Patients with SCA present with varied clinical features and complications that may affect many organs in the human body. There are few treatment options for SCA, and patient responses vary; the only curative therapy is a stem cell transplant or gene therapy. We seek to study the clinical features and treatment options for patients with sickle cell disease treated in King Abdulaziz Medical City, Riyadh. Methods: This study was a retrospective cohort study of all adult SCA patients who were admitted to our institution during the period from 1983 to 2016. Data were entered into Excel spreadsheets and managed with SPSS. Chi-square test was used to compare responses to therapy and in patients with different presentations. Results: A total of 106 patients with sickle cell disease were included in this study. The percentages with respect to hospital admissions per year were as follows: 34.9% (37 patients) were never admitted, 26.4% (28 patients) were admitted fewer than 2 times, 24.5% (26 patients) were admitted 3–5 times, and 13.2% (14 patients) were admitted more than 5 times. The number of complications was used to measure the severity of the disease. The disease severity was higher in males than in females (67.3% vs. 32.7%, respectively; P = 0.018). The disease severity was higher in those who were born in the Western and Southwestern areas than in other areas (84.2% vs. 15.8%, respectively; P = 0.007). Hydroxyurea significantly reduced the severity of SCA with a P = 0.002. Conclusion: Our study showed that vaso-occlusive crisis was the most common complication and indication for hospital admission. Treatment with hydroxyurea led to a significant reduction in the number of hospital admissions.

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Background / Objective: The presence of persistent high fetal hemoglobin (HbF) in sickle cells disease (SCD) patients may be a modulator of clinical and biochemical features. This study seeks to test the hypothesis that high level of HbF may regulate the levels of calcium, magnesium, zinc, and copper in SCD patients in a steady clinical state. Materials and Methods: Serum calcium, magnesium, zinc, and copper were assayed in 100 SCD patients in steady clinical state and 50 control subjects using the colorimetric method while blood HbF was determined by alkaline denaturation method. Results: Twenty-five percent of the study group had high (>5%) HbF, while 75% had low (<4.9%) HbF levels. HbF (P < 0.001), serum copper (P < 0.001), and calcium (P = 0.002) were significantly higher in SCD patients compared with controls, while zinc and magnesium were significantly lower (P < 0.001) in SCD patient compared with controls. Serum calcium (P = 0.01) and copper (P = 0.118) were lower in SCD patients with high (≥5%) HbF while magnesium and zinc were significantly higher (P < 0.001) in SCD patients with high HbF compare with those with low (≤4.9). HbF correlated negatively with calcium (r = −0.25, P = 0.011) and copper (r = -0.11, P = 0.287) while magnesium (r = 0.60, P = 0.001) and zinc (r = 0.57, P < 0.001) correlated positively on HbF levels. Conclusion: HbF levels may have modulated the levels of these elements in SCD patients. It is suggested that HbF may be estimated along with hemoglobin electrophoresis in diagnosis, clinical management, and predicting clinical course of SCD patients. Nutritional studies may be routinely conducted in this group of patients for better management.

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Background and Objectives: Donor screening process is one of the most important steps in protecting the safety of blood supply. Donors who do not meet specified criteria are deferred either temporarily or permanently. These criteria are designed to protect both donors and patient safety. Due to the varied rates and reasons for deferrals in the existing literature, we aimed to evaluate the patterns and prevalence of deferrals in our institution. Materials and Methods: This retrospective study was conducted at a Tertiary Care Hospital, Karnataka, Southern India, to evaluate the various reasons for blood donor deferral from January 2011 to January 2014. Demographic data of blood donors was obtained through the blood bank database and secondary measures such as the type of deferral (permanent/temporary, pathogenic/nonpathogenic, and harmful to donor/recipient) were assessed. Results: A total of 54,653 subjects presented to our blood bank during this period out of which 2935 (5.6%) were deferred. The deferral to donor percentage was higher in females (36.54%) than males (3.64%). Low hemoglobin was the major deferral criterion seen in our participants (48.1%) followed by hypertension (16.4%), underweight (8.9%). Low pulse rate and fasting donor were the least prevalent reasons. A total of 36.8% of reasons for deferral were harmful to donors, 88.2% were nonpathogenic, and 98.1% were temporary causes. Conclusion: Variations in donor deferral may be attributed to different donor selection criteria in different regions and centers. Hence, it is important to know the common causes of donor deferral in a region so that measures may be taken to improve the donor pool.

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Context: Pancytopenia is not a disease but a clinico-hematological entity encountered in clinical practice. A prompt intervention is required to avoid complications, which can occur in these patients. The relevant clinical history and complete hematological workup is required to treat patient of pancytopenia. The severity of pancytopenia and the underlying pathology determines the management and prognosis. Aims: To diagnose different conditions producing pancytopenia on the basis of clinical, hematological and bone marrow studies. To estimate the frequency of different diseases producing pancytopenia. Subjects and Methods: The study was conducted in Department of Hematology of our college in capital city of India. A prospective study was carried out among 100 consecutive patients with pancytopenia. Blood samples of the patients were analyzed for red cells, white cells and platelets morphology along with presence and absence of immature cells and abnormal cells. In bone marrow examination, morphology of all cells lineage, cellularity, parasite and abnormal cells were scrutinized. Trephine biopsy was done where ever indicated. Results: The age of patients ranged from 5 to 80 years. 74% of patients in this study were vegetarian. The most common cause of pancytopenia was megaloblastic anemia (66%) followed by aplastic anemia (18%), malaria (6%), kala-azar (4%), acute myeloid leukemia (2%), multiple myeloma (2%), myelodysplastic syndrome (1%), and tuberculosis (1%). Conclusions: The present study concludes that detailed primary hematological investigations along with bone marrow examination in cytopenic patients is helpful for understanding disease process, to diagnose or to rule out the causes of pancytopenia. It is also useful in planning further investigations and management.

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Context : Various hemoglobinopathies are one of the major public health problems of India. Aims: High performance liquid chromatography (HPLC) is a speedy and accurate tool for diagnosis of various hemoglobinopathies. In present study about 7,000 cases have been studied for identification of various hemoglobin (Hb) disorders in western India. Settings and Design : Study is conducted from May 2010 till April 2013 for various hemoglobinopathies and variants. The geographical distribution of all cases included states of western India. Materials and Methods: Complete blood count (CBC) is done on CELL DYN 3700 analyzer and then HPLC is performed on BIO-RAD 'VARIANT II' (beta thalassemia short program) in samples received in our laboratory. Simple Statistical analysis is done with help of Microsoft Office Excel 2007. Results: A total of 7,261 cases were included in our study, out of these 1,615 (22.24%) cases showed abnormal Hb fractions. The major abnormality observed was of high HbA2, a cutoff value of > 3.9% was considered for diagnosis of beta thalassemia trait (BTT). A total of 839 cases (11.55%) of BTT were diagnosed. Other hemoglobinopathies were also identified in varying proportions. Conclusions: HPLC is simple, accurate, and superior technique combined with complete automation makes it an ideal method for diagnosis of hemoglobinopathies.

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Background: Tumor lysis syndrome (TLS) comprises a number of metabolic and renal abnormalities of variable spectrum. It is associated with lymphoproliferative disorder e.g., acute lymphoblastic leukemia (ALL), non-Hodgkin lymphoma (NHL). The aim of this study to evaluate the incidence, severity and outcome in Saudi children with ALL. Methods: Medical records of Children age <14 years and diagnosed with ALL were reviewed. We adopted the Hande and Garrow definitions for tumor lysis Syndrome. All patients received hydration, alkalinization with sodium bicarbonate and allopurinol as prophylaxis. Results: Seventy-four patients developed TLS (19% of all patients). Boys were affected than girls with ratio of 2.4:1. LTLS occurred in 93.3% and CTLS in 6.7%. Hyperphosphatemia was found in 94.6% of the patients. Hyperkalemia occurred only in 17 patients (23%). Hypokalemia was found in significant number of patients (12%). Lymphomatous presentation was seen in 60 patients (82%). Hemodialysis was required in 5 patients (6.6%) and 4 of them had hyperleukocytosis. There were no deaths encounterd due to TLS. Male sex, lymphomatous presentation, high LDH, high pretreatment WBC and central nervous system involvement were found to have statistical significance as risk factors for TLS development. Conclusion: TLS is a common event in childhood ALL. Spontaneous TLS indicate highly proliferating tumor and possible delayed presentation to our institute. Prophylactic measures are important to improve the outcome particularly when initiated as soon as the diagnosis is established.

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Middle East respiratory syndrome corona virus (MERS-CoV) is becoming a major health concern in Saudi Arabia resulting in an associated high case fatality. The rapid deterioration of vital organs functions and high mortality rate seen in MERS-CoV has been observed previously in Epstein-Barr virus (EBV), severe acute respiratory syndrome caused by a coronavirus (SARS-CoV) and avian and bird flu-related hemophagocytic lymphohistiocytosis (HLH). There are many reports of successful treatments for severe cases of EBV-HLH using HLH-94/2004-type immunochemotherapy with etoposide as a pro-apoptotic agent. The use of immunochemotherapy in severe MERS-CoV-related HLH merits an urgent investigation in a well-designed clinical trial.

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Peliosis is an extremely rare condition of unknown etiology, and its pathogenesis is characterized by multiple, cyst-like, blood-filled cavities predominantly involving a mononuclear phagocytic system. Isolated splenic peliosis is extremely uncommon, but may present as a potentially life-threatening situation. An awareness of its clinical condition and prompt management may improve the clinical outcomes. The role of prophylactic splenectomy is not clear in this condition.

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Aim: Acute myeloid leukemia (AML) is a type of malignancy that is associated with a malignant alteration of normal hematopoietic stem cells in the bone marrow. The aim of this study was to study the demographics and pathological subtypes of AML, evaluate the response and outcome to different treatment modalities. Methods: This was a retrospective study of adult patients diagnosed with AML at King Abdulaziz Medical City - Riyadh, between 2006 and 2013. Data were retrieved from patients' files, electronic medical files and laboratory information system. Results: 91 patients were included in the study with a male dominance. M1 was the most common French-American-British subtype with 23 (32%) cases. Patients with intermediate-risk AML were the most common subgroup with 41 (48%) cases followed by high and low-risk subgroups, 29 (33%) and 16 (19%), respectively. 74 patients were treated with intensive chemotherapy, and 17 were on palliative chemotherapy or best supportive treatment. Remission rate was found to be 84% in patients who received induction chemotherapy while 41% of them relapsed. 93% of low-risk patients underwent complete remission (CR) compared to intermediate and high-risk patients (79% and 87% respectively), but it was not statistically significant (P = 0.4). The median follow-up was 19 months, with overall survival (OS) of 46% for all groups. The low-risk patients had the highest OS 57% compared to intermediate and high risk (52% and 36%, respectively), but it was not statistically significant (P = 0.3). 18 patients had been treated with allogeneic stem cell transplant and at a median follow-up of 17 months posttransplant the OS was 72%. Conclusion: This study shows M1 subtype to be the most common of AML in this population. In addition, the CR was better with similar survival rate as compared to other local and internationally published experiences. These results, albeit with its limitations, need to be confirmed in a prospective clinical trial or national disease registry.

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Background: The risk of acquiring transfusion transmissible viral infections is said to be higher in patients requiring regular blood transfusions such as those with sickle cell disease (SCD). Aim: We determined the seroprevalence of blood transfusion viral markers among our patients with SCD. Subjects and Methods: This a case–control analytical study consisting of 82 confirmed SCD patients on routine follow-up at our facility in steady state and 90 age-matched controls. Demographic and transfusion history were recorded while 5 ml of blood was drawn for hematocrit levels, and serum tested for transfusion transmissible viral markers for hepatitis B, (hepatitis B surface antigen [HBsAg], hepatitis B surface antibody [HBsAb], hepatitis B e antigen [HBeAg], hepatitis B e antibody [HBeAb], hepatitis B core antibody [HBcAb]), hepatitis C virus (HCV) and human immunodeficiency viruses (HIVs) using the rapid test kits. Ethical approval for the study was obtained from the Institutional Review Board and each participant gave informed consent. Data were analyzed using descriptive and inferential statistics. Results: The seroprevalence of HBsAg, HBsAb, and HBeAg in cases was 2 (2.4%), 7 (8.5%), and 0 (0.0%), respectively, whereas it was 7 (8.5%), 11 (13.4%), 6 (7.3%), and 2 (2.4%) for HBeAb, HBcAb, HCV, and HIV antibodies. Compared to the controls, cases had higher prevalence rate of HBeAb (P = 0.005). No significant difference was observed in those with or without low hematocrit (≤18%) or those that received blood transfusion and those that did not (P > 0.05). Conclusion: We conclude that blood transfusion did not significantly increase the seroprevalence of markers of transfusion transmissible viral infection in SCD patients.

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Background and Objective: Recently totally implanted vascular access devices (TIVADs) have played an essential role in the management of pediatric patients. The purpose of this study was to describe the indications, techniques and complications of the procedures. Design and Setting: A retrospective study of all patients' files underwent TIVADs procedures between 2009-2012, in a single service providing institute in Saudi Arabia (King Saud Medical city-Riyadh). Patients and Methods: Fifty four TIVADs were inserted in fifty one patients (thirty males and twenty one females). Age ranged six months and twelve years (average 5.6 years). All TIVADs were implanted by pediatric consultant surgeons under general anesthesia. Seldinger technique used in fifty procedures and open approach in four patients. Results: The main indications for TIVADs use were acute lymphocytic leukemia forty six (90%), congenital nephrotic syndrum two (4%), hemophilia two (4%) and solid tumor one (2%). TIVADs were in situe for a total of 35222 days (average 652 days/catheter). Over all complications rate was (30%). Conclusion: TIVADs provided a simple almost pain free venous system access, allow children to conduct a normal life style and sport activities. We believe this study is one of very few reviews originating from developing countries with results comparable to those of world literature.

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Context: Role of youngsters in voluntary blood donation (VBD) is crucial to meet the demand of safe blood. Therefore understanding the various factors contributing to knowledge, attitude, and practice of VBD among college students is important. Aims: The present study aims to assess the level of knowledge, attitude, and practice regarding blood donation among the health care students. Settings and Design: Cross-sectional study done at Anand from July 2012 to December 2013. Subjects and Methods: A cross-sectional study was conducted among 1000 health care medical students at Anand, Gujarat, India using a structured survey questionnaire. Statistical Analysis Used: The data were tabulated, and master chart was prepared. Analysis was performed by using the percentage. Results: Overall knowledge on blood donation among respondents was 37.3%, majority of the participants (78.7%) never donated blood in which 43.7% of the nondonors had negative attitude like blood donation leads to weakness (10.2%), anemia (25.5%), and 8.0% of nondonors felt that blood donation leads to reduced immunity. Majority of nondonors showed a positive attitude (53.1%) by expressing their willingness to donate blood if they were asked to donate blood (35.3%), about 9.8% of the nondonors don't know the importance of blood donation and 11.2% of nondonors don't know where to donate blood. In this study, 11.5% of students had donated blood only once, in which 38.8% of blood donors said no privacy was provided while donation, few (12.2%) had experienced some discomfort after donation and 50.4% of respondent said that they have not tried again. Conclusions: The present study recommends that even health care students' needs to be educated about the importance, awareness and motivation of blood donation on a regular basis and addressing the problems faced, while blood donation will strengthen the recruitment and retention of blood donors to donate blood on a regular basis.

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Background: Despite the confirmed favorable prognosis of childhood t(8;21) acute myeloid leukemia (AML), recent reports suggest heterogeneity in survival outcomes in this subtype of AML may be influenced by ethnicity. Therefore, we aimed to assess the outcome of childhood t(8;21) AML in an Arab population to evaluate if survival outcomes were inferior and determine the predictive relevance of additional cytogenetic abnormalities. Methods: This multicenter retrospective study analyzed 175 de novo AML children of 14 years of age or younger consecutively diagnosed between January 2005 and December 2012. Survival outcomes were analyzed and patients with t(8;21) were stratified on the basis of karyotype into sole and additional cytogenetic groups. Results: A total of 33 (18.9%) patients had t(8;21) AML. Complete remission (CR) was achieved in 31 (93.9%) patients. The 5-year overall survival, event-free survival, cumulative incidence of relapse (CIR), and remission death rates were 59.9 ± 9.2, 45.6 ± 9.1, 36.4, and 9.1%, respectively. Despite the administration of hematopoietic stem-cell-transplant salvage therapy in first relapse, five out of 11 (45.5%) relapsed patients died of disease. Subanalysis of sole vs. additional cytogenetic abnormalities revealed no significant difference in outcome. Conclusion: In the present study, childhood t(8;21) AML was associated with inferior survival and resistance to salvage therapy compared to reports from international groups. The inferior outcomes were unrelated to additional cytogenetic abnormalities. Further detailed genetic studies are warranted to unmask the biological and clinical differences between racial/ethnic groups. Given the high CR rate of childhood t(8;21) AML, further modification of postremission therapy to improve the CIR rate is needed.

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Background: Septicemia is one of the major causes of neonatal morbidity and mortality. Objective- The objective of our study was to determine the role of hematologic scoring system (HSS) in early diagnosis of neonatal septicemia and provide an effective guideline in decision making regarding judicious use of antibiotics. Materials and Methods: The study was done taking 60 neonates admitted in the hospital. The hematological parameters were studied in all of them. Blood cultures were performed as gold standard for diagnosing septicemia. Results: Analysis of the hematologic profiles in the light of the HSS found that an abnormal immature to total neutrophil (I:T) ratio followed by an abnormal immature to mature neutrophil (I:M) ratio were the most sensitive indicators in identifying infants with sepsis. The study also found that the higher the score the greater the certainty of sepsis being present. The score ≥ 4 was more reliable as a screening tool than any individual hematological parameter. Conclusion : HSS is rapid, easy to perform, cost-effective screening tool for early diagnosis of neonatal sepsis and thereby helps in making of decisions regarding judicious use of antibiotics.

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Context: Manual examination of peripheral blood smear in diagnosis of anemia has taken a backseat with the advent of automated counters. Though a lot of studies have been done to assess the efficacy and significance of red blood cell parameters in different hematological conditions fewer efforts have been made to standardize the visual examination of peripheral blood smears for diagnosing anemias. Aims: Standardization and grading of abnormal red cell morphology in peripheral blood smear and counter based red cell indices in cases of anemia of various etiologies. Settings and Design: Cross-sectional study of one year duration conducted in the Hematology laboratory, in a tertiary care hospital in North India. Materials and Methods: In 60 anemic patients, automated counts and peripheral blood smear were prepared and evaluated by three observers, according to a red cell morphology grading guide. Statistical Analysis Used: ANOVA, Tukey post hoc test were used. Results: Objective grading of peripheral blood smears in cases of anemia have a good inter observer correlation and hence have reduced subjective variation. Manual parameters like microcytosis, macrocytosis and hypochromia expressed as a percentage, have shown significant correlation, with their corresponding automated parameters, and the regression model so generated may provide a novel way for quality control of automated counters, if calculated for different models. Conclusions: Even in the age of molecular analysis, the blood smear remains an important diagnostic tool and sophisticated modern investigations of hematologic disorders should be interpreted in the light of peripheral blood features as well as the clinical context.

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Objectives: Many Iranian studies have reported the high prevalence of osteoporosis and low bone mass in patients with thalassemia. Considering the importance of prevention in lowering the risk of osteoporosis and its complications in patients with thalassemia major (TM), the present study was conducted to assess the knowledge, attitude and preventive practice (KAP) of a group of TM patients regarding the importance of calcium and Vitamin D supplementation and its role in preventing osteoporosis. Materials and Methods: The cross-sectional study was conducted on all adult beta-TM patients (200) who visited the Specific Disease Center, in the Iranian Capital, Tehran. A four-section questionnaire on demographic, lifestyle, and socioeconomic information as well as their KAP about the importance of calcium and Vitamin D in preventing osteoporosis in TM patients was filled out for each patient. Results: About 63%, 62%, and 33% of those with poor knowledge, attitude and practice towards the importance of calcium and Vitamin D were not physically active, correspondingly. There was no association between age and gender and knowledge or attitude score. Conclusion: While a large number of the studied population consumed Ca and Vitamin D supplementation, their poor knowledge about the importance of these supplements points out the need for educating TM patients and their families about the importance of calcium and Vitamin D supplementation in preventing osteoporosis.

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Severe aplastic anemia (SAA) is a serious bone marrow disease that needs a comprehensive and service-intense treatment with either bone marrow transplantation (BMT) or immunosuppressive therapy (IST); both are difficult to optimally offer in resources-limited countries. Here, we report the outcome of IST using horse Antithymocyte globulin (ATG) in 18 (7 children; 11 adults) patients with SAA referred to our center in west India. Only 18 patients out of 102 diagnosed as AA in 2 years could receive IST, largely due to costs restraints. Although CR was seen in 30% in adults and 33% in pediatric cases, but overall 50% cases were able to enjoy transfusion-independence, requiring no further treatment. Treatment related mortality occurred in 6.2%, relapse in 6.2% and 6.2% had clonal evolution. This makes IST a valuable option for managing SAA in absence of bone marrow transplantation.

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Hemophilia is an X-linked heritable coagulopathy with an overall prevalence of approximately 1 in 10,000 individuals. The most common form is factor VIII deficiency (hemophilia A), which comprises approximately 80% of cases. Factor IX deficiency (hemophilia B), comprises approximately 20% of cases. In patients with hemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of hemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (hemophilic synovitis) that contribute to end-stage degeneration (hemophilic arthropathy); with pain and limitation of motion severely affecting patients' quality of life. The author conducted a comprehensive review and synthesis of the relevant literature. The author reviewed all compiled reports from computerized searches. Searches were limited to English-language sources and human subjects. Literature citations were generally restricted to published manuscripts appearing in journals listed in Index Medicus and reflected literature published up to July 1, 2013. The aim of this updated paper is to provide comprehensive and timely evidence-based guidelines and recommendations on the treatments of damaged joint among hemophilia patient. Further recommendations included the control of risk factors, interventional approaches for treatment of hemophilia, the use of bypassing factors for the inhibitors, and preventing recurrent bleeding especially in high-risk populations are provided. Prophylaxis by replacement of the missing factor in patients with hemophilia is the optimal way to prevent the occurrence of haemarthrosis and thereby the onset of arthropathy, provided that it is started early in life. Dosing should be individualized and increased in case of bleeding. Prevention of bleeding episodes through early treatment will prevent accumulation of blood in joint and the subsequent inflammation and potential hemophilic arthropathy. Treatment must be maintained until bleeding remission and patients have recovered as much of their ROM and muscular strength as possible. Clinical evaluation of the joints, gait, motion, muscle tone, functional level of disability, pain, and swelling, as well as imaging techniques, must be performed to assist in the diagnosis of chronic synovitis and to guide treatment decisions. The first step to treating synovitis, refractory to medical treatment, is the use of synovectomies, non-surgical or surgical interventions. In many cases, joint deformities have to be treated by open orthopaedic surgery. State-of-the-art treatment of patients with hemophilia requires a multidisciplinary team. Level of evidence: Level III (Systematic Literature review).

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