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    62 results match your criteria BMC Blood Disorders[Journal]

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    Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda.
    BMC Blood Disord 2012 Sep 7;12:11. Epub 2012 Sep 7.
    Life Link Medical Centre, Kampala, Uganda.

    Background: Sickle cell anaemia (SCA) is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF) has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Read More

    Estimating the treatment effect from non-randomized studies: The example of reduced intensity conditioning allogeneic stem cell transplantation in hematological diseases.
    BMC Blood Disord 2012 Aug 16;12:10. Epub 2012 Aug 16.
    Département de Biostatistique et Informatique Médicale, Hôpital Saint-Louis, AP-HP, Paris 75010, France.

    Background: In some clinical situations, for which RCT are rare or impossible, the majority of the evidence comes from observational studies, but standard estimations could be biased because they ignore covariates that confound treatment decisions and outcomes.

    Methods: Three observational studies were conducted to assess the benefit of Allo-SCT in hematological malignancies of multiple myeloma, follicular lymphoma and Hodgkin's disease. Two statistical analyses were performed: the propensity score (PS) matching approach and the inverse probability weighting (IPW) approach. Read More

    Isolated central nervous system relapse of chronic myeloid leukemia after allogeneic hematopoietic stem cell transplantation.
    BMC Blood Disord 2012 Aug 7;12. Epub 2012 Aug 7.
    Hans Berger Department of Neurology, Jena University Hospital, Erlanger Allee 101, D-07747 Jena, Germany.
    Background: This case report highlights the relevance of quantifying the BCR-ABL gene in cerebrospinal fluid of patients with suspected relapse of chronic myeloid leukemia in the central nervous system.

    Case Presentation: We report on a female patient with isolated central nervous system relapse of chronic myeloid leukemia (CML) during peripheral remission after allogeneic hematopoietic stem cell transplantation. The patient showed a progressive cognitive decline as the main symptom. Read More

    Syncytial giant cell hepatitis associated with chronic lymphocytic leukemia: a case report.
    BMC Blood Disord 2012 Jul 19;12. Epub 2012 Jul 19.
    Department of Medicine, St John Hospital and Medical Center, Detroit, MI, 48236, USA.

    Background: Syncytial giant cell hepatitis (GCH) is an uncommon and an underreported disease entity. In two previously reported cases of GCH in patients with Chronic Lymphocytic Leukemia (CLL) liver failure ensued. Autoimmune and infective causes have been implicated but its etiology remains unclear. Read More

    Prevalence and type of monoclonal gammopathy of undetermined significance in an apparently healthy Nigerian population: a cross sectional study.
    BMC Blood Disord 2012 Jun 28;12. Epub 2012 Jun 28.
    Department of Haematology & Blood Transfusion, Faculty of Clinical Sciences, College of Medicine, University of Lagos, P,M,B, 12003, Marina, Lagos, Nigeria.

    Background: The prevalence of monoclonal gammopathy of undetermined significance (MGUS), a premalignant plasma-cell disorder has not been determined in our geographic area Nigeria.

    Methods: A cross sectional survey was carried on apparently healthy Nigerians selected by multistage sampling technique from the cosmopolitan city of Lagos, Nigeria. Subjects enrolled into the study had 2-step screening for the presence, type and concentration of monoclonal band. Read More

    Health related quality of life in Middle Eastern children with beta-thalassemia.
    BMC Blood Disord 2012 Jun 22;12. Epub 2012 Jun 22.
    Centro Trapianti di Midollo Osseo, P,O, "R, Binaghi", Via Is Guadazzonis, 3, 09126, Cagliari, Italy.

    Background: Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL) in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries.

    Methods: We studied 60 thalassemia children from Middle Eastern countries with a median age of 10 years (range 5 to 17 years). Read More

    Zinc finger nucleases for targeted mutagenesis and repair of the sickle-cell disease mutation: An in-silico study.
    BMC Blood Disord 2012 May 14;12. Epub 2012 May 14.
    Unit of Genetics, Genomics & Theoretical Biology, Dept of Pathology, School of Biomedical Science, College of Health Sciences, Makerere University, P o Box 7072, Kampala, Uganda.
    Background: Sickle cell disease (or simply, SCD) is an inherited hemoglobinopathy which is mostly prevalent among persons of African descent. SCD results from a monogenic (Hemoglobin, beta) point-mutation (substitution of the base Adenine with Thymine at position six) that leads to replacement of the amino acid glutamic acid (E) with valine (V). Management of SCD within resource-poor settings is largely syndromic, since the option of cure offered by bone-marrow transplantation (BMT) is risky and unaffordable by most affected individuals. Read More

    Molecular characterization of glucose-6-phosphate dehydrogenase deficient variants in Baghdad city - Iraq.
    BMC Blood Disord 2012 Mar 27;12. Epub 2012 Mar 27.
    Department of Pathology, College of Medicine, University of Dohuk, Azadi Hospital road, 1014 AM Dohuk, Iraq.
    Background: Although G6PD deficiency is the most common genetically determined blood disorder among Iraqis, its molecular basis has only recently been studied among the Kurds in North Iraq, while studies focusing on Arabs in other parts of Iraq are still absent.

    Methods: A total of 1810 apparently healthy adult male blood donors were randomly recruited from the national blood transfusion center in Baghdad. They were classified into G6PD deficient and non-deficient individuals based on the results of methemoglobin reduction test (MHRT), with confirmation of deficiency by subsequent enzyme assays. Read More

    Hemoglobin E syndromes in Pakistani population.
    BMC Blood Disord 2012 Mar 25;12. Epub 2012 Mar 25.
    Department of Pathology and Microbiology, The Aga Khan University Hospital, Stadium road, Karachi 74800, Pakistan.
    Background: Hemoglobin E is an important hemoglobin variant with a worldwide distribution. A number of hemoglobinopathies have been reported from Pakistan. However a comprehensive description of hemoglobin E syndromes for the country was never made. Read More

    Patient-reported treatment burden of chronic immune thrombocytopenia therapies.
    BMC Blood Disord 2012 Mar 22;12. Epub 2012 Mar 22.
    United BioSource Corporation, Lexington, MA, USA.
    Background: Chronic immune thrombocytopenia (ITP) is a debilitating autoimmune disorder that causes a reduction in blood platelets and increased risk of bleeding. ITP is currently managed with various pharmacologic therapies and splenectomy.This study was conducted to assess patient perceived and reported treatment side effects, as well as the perceived burden or bother, and need to reduce or stop treatment, associated with these side effects among adult patients with chronic ITP. Read More

    Fanconi anaemia with bilateral diffuse pulmonary arterio venous fistulae: a case report.
    BMC Blood Disord 2012 Mar 17;12. Epub 2012 Mar 17.
    University Medical Unit, University of Colombo, Colombo, Sri Lanka.
    Background: We report a patient with cytogenetically confirmed Fanconi anaemia with associated diffuse bilateral pulmonary arterio-venous fistulae. This is only the second reported case of diffuse pulmonary arterio-venous fistulae with Fanconi anaemia.

    Case Presentation: A 16 year old Sri Lankan boy, with a cytogenetically confirmed Fanconi anaemia was admitted to University Medical Unit, National Hospital of Sri Lanka for further assessment and treatment. Read More

    Meta-analysis of efficacy and safety of intravenous ferric carboxymaltose (Ferinject) from clinical trial reports and published trial data.
    BMC Blood Disord 2011 Sep 24;11. Epub 2011 Sep 24.
    Pain Research, Nuffield Department of Anaesthetics, University of Oxford, Oxford Radcliffe Hospitals, The Churchill, Oxford, OX3 7LJ, UK.
    Background: Recommendations given for intravenous iron treatment are typically not supported by a high level of evidence. This meta-analysis addressed this by summarising the available date from clinical trials of ferric carboxymaltose using clinical trial reports and published reports.

    Methods: Clinical trial reports were supplemented by electronic literature searches comparing ferric carboxymaltose with active comparators or placebo. Read More

    Lung transplantation for pulmonary fibrosis in dyskeratosis congenita: Case Report and systematic literature review.
    BMC Blood Disord 2011 Jun 15;11. Epub 2011 Jun 15.
    Division of Cancer Epidemiology and Genetics, Clinical Genetics Branch, National Cancer Institute, National Institutes of Health, Rockville 20852, MD, USA.
    Background: Dyskeratosis congenita (DC) is a progressive, multi-system, inherited disorder of telomere biology with high risks of morbidity and mortality from bone marrow failure, hematologic malignancy, solid tumors and pulmonary fibrosis. Hematopoietic stem cell transplantation (HSCT) can cure the bone marrow failure, but it does not eliminate the risks of other complications, for which life-long surveillance is required. Pulmonary fibrosis is a progressive and lethal complication of DC. Read More

    Serum levels of leptin in Nigerian patients with sickle cell anaemia.
    BMC Blood Disord 2011 May 26;11. Epub 2011 May 26.
    Department of Biochemistry and Nutrition, Nigerian Institute of Medical Research, Yaba, Lagos, Nigeria.
    Background: Several studies have shown that the pathophysiology of homozygous sickle cell anaemia (SCA) results in a myriad of metabolic, nutritional, haematological and clinical effects that interact with other co-morbid factors to determine the quality of life and life expectancy of afflicted patients. Because of its critical roles in nutrition and metabolism, inflammation, haematopoiesis and cellular immunity, this study determined the plasma levels of leptin in steady and unsteady states of HbSS in Nigerian patients.

    Methods: A total of 51 SCA patients aged 5 - 35 years with 34 (61. Read More

    Erythrocyte reference values in Emirati people with and without α+ thalassemia.
    BMC Blood Disord 2011 Feb 24;11. Epub 2011 Feb 24.
    Department of Internal Medicine, Faculty of Medicine and Health Sciences, UAE University, Al Ain, UAE.
    Background: Interpreting the erythroid lineage in populations with high frequency of α+ thalassemia allele is challenging due to the high prevalence of α+ thalassemia homozygotes. For such populations, separate reference values for normal and α+ thalassemia homozygotes are needed.

    Methods: We studied the erythroid lineage in 1,079 citizens of United Arab Emirates (UAE). Read More

    Pica associated with iron deficiency or depletion: clinical and laboratory correlates in 262 non-pregnant adult outpatients.
    BMC Blood Disord 2010 Dec 22;10. Epub 2010 Dec 22.
    Southern Iron Disorders Center, Birmingham, Alabama, USA.
    Background: There are many descriptions of the association of pica with iron deficiency in adults, but there are few reports in which observations available at diagnosis of iron deficiency were analyzed using multivariable techniques to identify significant predictors of pica. We sought to identify clinical and laboratory correlates of pica in adults with iron deficiency or depletion using univariable and stepwise forward logistic regression analyses.

    Methods: We reviewed charts of 262 non-pregnant adult outpatients (ages ≥18 y) who required treatment with intravenous iron dextran. Read More

    Patan hospital experience in treating philadelphia chromosome/BCR-ABL1 positive chronic myeloid leukemia patients with gleevec (imatinib mesylate); the first generation specific tyrosine kinase inhibitor.
    BMC Blood Disord 2010 Dec 7;10. Epub 2010 Dec 7.
    Department of Internal Medicine, Patan Hospital, Lalitpur Nepal.
    Background: Chronic Myeloid Leukemia (CML) is caused by the abnormal fusion protein BCR-ABL1, a constitutively active tyrosine kinase and product of the Philadelphia chromosome. Gleevec (Imatinib mesylate) is a selective inhibitor of this kinase. Treatment with this agent is known to result in hematologic, cytogenetic, and molecular responses. Read More

    Molecular measurement of BCR-ABL transcript variations in chronic myeloid leukemia patients in cytogenetic remission.
    BMC Blood Disord 2010 Nov 18;10. Epub 2010 Nov 18.
    Department of Hematology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.
    Background: The monitoring of BCR-ABL transcript levels by real-time quantitative polymerase chain reaction (RT-qPCR) has become important to assess minimal residual disease (MRD) and standard of care in the treatment of chronic myeloid leukemia (CML). In this study, we performed a prospective, sequential analysis using RT-qPCR monitoring of BCR-ABL gene rearrangements in blood samples from 91 CML patients in chronic phase (CP) who achieved complete cytogenetic remission (CCyR) and major molecular remission (MMR) throughout imatinib treatment.

    Methods: The absolute level of BCR-ABL transcript from peripheral blood was serially measured every 4 to 12 weeks by RT-qPCR. Read More

    Prevalence and molecular characterization of Glucose-6-Phosphate dehydrogenase deficient variants among the Kurdish population of Northern Iraq.
    BMC Blood Disord 2010 Jul 5;10. Epub 2010 Jul 5.
    Department of Pathology, College of Medicine, University of Dohuk, Azadi Hospital road, 1014 AM Dohuk, Iraq.
    Background: Glucose-6-Phosphate dehydrogenase (G6PD) is a key enzyme of the pentose monophosphate pathway, and its deficiency is the most common inherited enzymopathy worldwide. G6PD deficiency is common among Iraqis, including those of the Kurdish ethnic group, however no study of significance has ever addressed the molecular basis of this disorder in this population. The aim of this study is to determine the prevalence of this enzymopathy and its molecular basis among Iraqi Kurds. Read More

    An up-date on the prevalence of sickle cell trait in Eastern and Western Uganda.
    BMC Blood Disord 2010 Jun 23;10. Epub 2010 Jun 23.
    Department of Pathology, Makerere University, College of Health Sciences, P,O, Box 7072, Kampala, Uganda.
    Background: The first survey on sickle cell disease (SCD) done in Uganda in 1949, reported the district of Bundibugyo in Western Uganda to have the highest sickle cell trait (SCT) prevalence (45%). This is believed to be the highest in the whole world. According to the same survey, the prevalence of SCT in the districts of Mbale and Sironko in the East was 20-28%, whilst the districts of Mbarara and Ntungamo in the West had 1-5%. Read More

    Differences in the haematological profile of healthy 70 year old men and women: normal ranges with confirmatory factor analysis.
    BMC Blood Disord 2010 Jun 11;10. Epub 2010 Jun 11.
    Geriatric Medicine unit, University of Edinburgh, Edinburgh, UK.
    Background: Reference ranges are available for different blood cell counts. These ranges treat each cell type independently and do not consider possible correlations between cell types.

    Methods: Participants were identified from the Community Health Index as survivors of the 1947 Scottish Mental Survey, all born in 1936, who were resident in Lothian (potential n = 3,810) and invited to participate in the study. Read More

    Hereditary angioedema: New therapeutic options for a potentially deadly disorder.
    BMC Blood Disord 2010 May 14;10. Epub 2010 May 14.
    Department of Allergy and Immunology, Medical Informatics, Cleveland Clinic Florida, 2950 Cleveland Clinic Blvd, Weston, FL 33331, USA.
    Although the biochemistry of hereditary angioedema (HAE) is fairly well understood today, the lag in diagnosis of a decade or more suggests that clinicians have low awareness of this disease. This lag in diagnosis and hence treatment certainly stems from the rarity and complexity of the presentation which can be easily mistaken for allergic and non-allergic reactions alike. The symptoms of the disease include acute swelling of any or multiple parts of the body. Read More

    Effectiveness of communication campaign on iron deficiency anemia in Kyzyl-Orda region, Kazakhstan: a pilot study.
    BMC Blood Disord 2010 Mar 17;10. Epub 2010 Mar 17.
    Department of Young Leaders' Program for Medical Administration and Politics, Graduate School of Medicine, Nagoya University, 65 Tsurumai-Cho, Nagoya, 466-8550, Japan.
    Background: In 2004, wheat flour fortification (WFF) with iron was implemented in Kazakhstan as a public health strategy to increase the iron intake of all women of childbearing age and of children. In 2003, before starting the flour fortification program, a communication campaign on health education took place in a region with a high prevalence of iron deficiency anemia (IDA). The present study aimed to evaluate the prevalence of anemia, iron deficiency and IDA before and after the campaign. Read More

    Factors affecting health-related quality of life in Thai children with thalassemia.
    BMC Blood Disord 2010 Jan 21;10. Epub 2010 Jan 21.
    Department of Pharmacy, Faculty of Pharmacy, Mahidol University, Bangkok, Thailand.
    Background: Knowledge of the factors associated with health-related quality of life (HRQOL) among patients with thalassemia is essential in developing more suitable clinical, counseling, and social support programs to improve treatment outcomes of these patients. In light of the limited research in this area, this study aims to examine factors associated with HRQOL among children and adolescents with thalassemia in Thailand.

    Methods: A cross-sectional survey was conducted in three selected hospitals in Thailand during June to November 2006. Read More

    Iron deficiency anemia is not a rare problem among women of reproductive ages in Ethiopia: a community based cross sectional study.
    BMC Blood Disord 2009 Sep 7;9. Epub 2009 Sep 7.
    School of Public Health, Addis Ababa University, P,O, Box: 27285/1000, Addis Ababa, Ethiopia.
    Background: In Ethiopia, the existence of iron deficiency anemia is controversial despite the fact that Ethiopia is one of the least developed in Africa with a high burden of nutrient deficiencies.

    Methods: The first large nutrition study of a representative sample of women in Ethiopia was conducted from June to July 2005 and a systematically selected sub-sample of 970 of these subjects, 15 to 49 years old, were used in this analysis of nutritional anemia. Hemoglobin was measured from capillary blood using a portable HemoCue photometer. Read More

    The role of religious leaders and faith organisations in haemoglobinopathies: a review.
    BMC Blood Disord 2009 Aug 15;9. Epub 2009 Aug 15.
    Paediatrics Department, West Middlesex University Hospital, Isleworth, Middlesex, UK.
    Background: Sickle cell disease (SCD) is now the most common genetic condition in the world including the UK with an estimate of over 12,500 affected people and over 300 new births per year. Blood transfusion therapy plays a very important role as a disease-modifying strategy in severe SCD e.g. Read More

    Profile of hematological abnormalities of Indian HIV infected individuals.
    BMC Blood Disord 2009 Aug 13;9. Epub 2009 Aug 13.
    Department of Internal Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
    Background: Hematological abnormalities are a common complication of HIV infection. These abnormalities increase as the disease advances. Bone marrow abnormalities occur in all stages of HIV infection. Read More

    Application of tri-colour, dual fusion fluorescence in situ hybridization (FISH) system for the characterization of BCR-ABL1 fusion in chronic myelogenous leukaemia (CML) and residual disease monitoring.
    BMC Blood Disord 2009 Jul 7;9. Epub 2009 Jul 7.
    Department of Pathology, Queen Elizabeth Hospital, Hong Kong SAR, PR China.
    Background: We studied the application of the BCR-ABL1 + 9q34 tri-colour dual fusion fluorescence in situ hybridization (FISH) system in the characterization of fusion signal pattern and the monitoring of residual disease in chronic myelogenous leukaemia (CML). The signal constellation on metaphases with the tri-colour dual fusion system was defined. The knowledge of various signal patterns obtained from the different genetic rearrangements was further applied to the analysis of hybridization signals on interphase nuclei. Read More

    Prevalence, phenotype and inheritance of benign neutropenia in Arabs.
    BMC Blood Disord 2009 Mar 27;9. Epub 2009 Mar 27.
    Department of Internal Medicine, Faculty of Medicine and Health Sciences, UAE University, Al Ain, UAE.
    Background: Benign neutropenia, i.e., neutropenia not associated with an increased risk of infection, may result in serious medical consequences when a 'standard' definition of neutropenia (absolute neutrophil count (ANC) < 1. Read More

    Factors associated with hospital readmission in sickle cell disease.
    BMC Blood Disord 2009 Feb 27;9. Epub 2009 Feb 27.
    Rio de Janeiro Federal University (UFRJ), Rio de Janeiro, RJ, Brazil.
    Background: Sickle cell disease is the most frequent hereditary disease in Brazil, and people with the disease may be hospitalised several times in the course of their lives. The purpose of this study was to estimate the hazard ratios of factors associated with the time between hospital admissions.

    Methods: The study sample comprised all patients admitted, from 2000 to 2004, to a university hospital in Rio de Janeiro State, south-east Brazil, as a result of acute complications from sickle cell disease (SCD). Read More

    FIP1L1-PDGFRA molecular analysis in the differential diagnosis of eosinophilia.
    BMC Blood Disord 2009 Feb 2;9. Epub 2009 Feb 2.
    Department of Immunology and Histocompatibility, University of Thessaly Medical School, University Hospital of Larissa, Larissa, Greece.
    Background: Primary eosinophlia associated with the FIP1L1-PDGFRA rearrangement represents a subset of chronic eosinophilic leukaemia (CEL) and affected patients are very sensitive to imatinib treatment. This study was undertaken in order to examine the prevalence and the associated clinicopathologic and genetic features of FIP1L1-PDGFRA rearrangement in a cohort of 15 adult patients presenting with profound eosinophilia (> 1.5 x 109/L). Read More

    Estimation of transient increases in bleeding risk associated with physical activity in children with haemophilia.
    BMC Blood Disord 2008 Jun 26;8. Epub 2008 Jun 26.
    School of Medical Sciences, University of New South Wales, Randwick NSW 2052, Australia.
    Background: Although it is widely appreciated that vigorous physical activity can increase the risk of bleeding episodes in children with haemophilia, the magnitude of the increase in risk is not known. Accurate risk estimates could inform decisions made by children with haemophilia and their parents about participation in physical activity and aid the development of optimal prophylactic schedules. The aim of this study is to provide an accurate estimate of the risks of bleeding associated with vigorous physical activity in children with haemophilia. Read More

    Safety of intramuscular influenza vaccine in patients receiving oral anticoagulation therapy: a single blinded multi-centre randomized controlled clinical trial.
    BMC Blood Disord 2008 May 29;8. Epub 2008 May 29.
    EAP Gòtic, Institut Català de la Salut, Passatge de la Pau 1, 08001 Barcelona, Spain.
    Background: Influenza vaccines are recommended for administration by the intramuscular route. However, many physicians use the subcutaneous route for patients receiving an oral anticoagulant because this route is thought to induce fewer hemorrhagic side effects. Our aim is to assess the safety of intramuscular administration of influenza vaccine in patients on oral anticoagulation therapy. Read More

    Impact of delayed initiation of erythropoietin in critically ill patients.
    BMC Blood Disord 2007 Oct 4;7. Epub 2007 Oct 4.
    Inpatient Pharmacy Department, Kaiser Permanente, 975 Sereno Dr., Vallejo, CA 94589, USA.
    Background: The purpose of this study was to evaluate the impact of recombinant human erythropoietin (rHuEPO) use for anemia of critical illness at a practice site where delayed initiation is common.

    Methods: Retrospective medical record review involving patients treated with rHuEPO for anemia of critical illness. Those patients given rHuEPO or diagnosed with end-stage renal disease (ESRD) prior to ICU admission were excluded. Read More

    Spontaneous intra-peritoneal bleeding secondary to warfarin, presenting as an acute appendicitis: a case report and review of literature.
    BMC Blood Disord 2006 Oct 11;6. Epub 2006 Oct 11.
    Department of Surgery, Royal Free Hospital, London, UK.
    Background: Warfarin is a coumarin anti-coagulant, used widely for the therapeutic and prophylactic anticoagulation. Although, it is considered as a life saving medicine, it is associated with the significant adverse effects including intra-abdominal bleeding, which have been very well documented in literature. However, the presentation of warfarin induced intra-peritoneal bleeding as an acute appendicitis has not been reported in English literature. Read More

    Genetic background determines response to hemostasis and thrombosis.
    BMC Blood Disord 2006 Oct 5;6. Epub 2006 Oct 5.
    Department of Cardiovascular Medicine, Joseph J. Jacobs Center for Thrombosis and Vascular Biology, Ohio, USA.
    Background: Thrombosis is the fatal and disabling consequence of cardiovascular diseases, the leading cause of mortality and morbidity in Western countries. Two inbred mouse strains, C57BL/6J and A/J, have marked differences in susceptibility to obesity, atherosclerosis, and vessel remodeling. However, it is unclear how these diverse genetic backgrounds influence pathways known to regulate thrombosis and hemostasis. Read More

    Birth characteristics and the risk of childhood leukaemias and lymphomas in New Zealand: a case-control study.
    BMC Blood Disord 2006 Sep 14;6. Epub 2006 Sep 14.
    Department of Preventive and Social Medicine, University of Otago, Dunedin, New Zealand.
    Background: Some studies have found that lower parity and higher or lower social class (depending on the study) are associated with increased risks of childhood acute lymphoblastic leukaemia (ALL). Such findings have led to suggestions that infection could play a role in the causation of this disease. An earlier New Zealand study found a protective effect of parental marriage on the risk of childhood ALL, and studies elsewhere have reported increased risks in relation to older parental ages. Read More

    Extensive myocardial infiltration by hemopoietic precursors in a patient with myelodysplastic syndrome.
    BMC Blood Disord 2006 Sep 5;6. Epub 2006 Sep 5.
    Department of Neurology, Mayo Clinic1, Rochester, USA.
    Background: Although myocardial infiltration with leukemic blasts is a known finding in patients with acute leukemia, this phenomenon in myelodysplasia is not reported in the literature. Cardiac symptoms in patients with myelodysplasia are often due to anemia and may be due to iron overload and side effects of therapy.

    Case Presentation: Herein we report the first case of neoplastic infiltration of the heart with associated myocardial necrosis in a patient with myelodysplasia. Read More

    Insights into age- and sickle-cell-disease-interaction using principal components analysis.
    BMC Blood Disord 2006 Sep 4;6. Epub 2006 Sep 4.
    Lata Medical Research Foundation, Nagpur, India.
    Background: In the context of sickle cell anemia, peripheral blood indexes provide key information that is also potentially influenced by age. Therefore, it is necessary to understand the extent and nature of interactions between sickle cell anemia and age, especially in situations where there is a high prevalence of sickle cell anemia.

    Methods: In a cross-sectional study of 374 subjects with varying hemoglobin S (HbS) status, we characterized the interaction between age and sickle hemoglobin using principal components analysis. Read More

    The effect of an exercise intervention on aerobic fitness, strength and quality of life in children with haemophilia (ACTRN012605000224628).
    BMC Blood Disord 2006 May 29;6. Epub 2006 May 29.
    School of Medical Sciences, Faculty of Medicine, University of New South Wales, Sydney 2052, Australia.
    Background: Children with haemophilia have lower levels of fitness and strength than their healthy peers. We present the protocol of a study designed to determine whether an exercise intervention improves quality of life, aerobic fitness and strength in children with haemophilia.

    Methods/design: The study will be a randomised, assessor-blinded, controlled trial of exercise treatment. Read More

    An unusual cause of acute abdominal pain - a case presentation.
    BMC Blood Disord 2006 Apr 7;6. Epub 2006 Apr 7.
    Department of Colorectal Surgery, Royal Shrewsbury Hospital, Mytton Oak Road, Shrewsbury, UK.
    Background: In 1983, Graham Hughes described a condition of Antiphospholipid Syndrome in which there was a danger of thrombosis. The condition is readily detectable by blood tests and, once diagnosed; the risk of further thrombosis can be significantly reduced by anticoagulation treatments. Affected groups of patients can be distinguished by a specific blood test - the detection of antiphospholipid antibody (Ref-1). Read More

    Total blood lymphocyte counts in hemochromatosis probands with HFE C282Y homozygosity: relationship to severity of iron overload and HLA-A and -B alleles and haplotypes.
    BMC Blood Disord 2005 Jul 25;5. Epub 2005 Jul 25.
    Department of Epidemiology and International Health, University of Alabama at Birmingham, Birmingham, Alabama, USA.
    Background: It has been reported that some persons with hemochromatosis have low total blood lymphocyte counts, but the reason for this is unknown.

    Methods: We measured total blood lymphocyte counts using an automated blood cell counter in 146 hemochromatosis probands (88 men, 58 women) with HFE C282Y homozygosity who were diagnosed in medical care. Univariate and multivariate analyses of total blood lymphocyte counts were evaluated using these variables: sex; age, transferrin saturation, and serum ferritin concentration at diagnosis; units of blood removed by phlebotomy to achieve iron depletion; and human leukocyte antigen (HLA)-A and -B alleles and haplotypes. Read More

    L-glutamine therapy reduces endothelial adhesion of sickle red blood cells to human umbilical vein endothelial cells.
    BMC Blood Disord 2005 Jul 25;5. Epub 2005 Jul 25.
    Department of Medicine, Harbor-UCLA Medical Center, UCLA School of Medicine, Torrance, CA, USA.
    Background: We have previously demonstrated that therapy with orally administered L-glutamine improves nicotinamide adenosine dinucleotide (NAD) redox potential of sickle red blood cells (RBC). On further analysis of L-glutamine therapy for sickle cell anemia patients, the effect of L-glutamine on adhesion of sickle RBC to human umbilical vein endothelial cells (HUVEC) was examined.

    Methods: The first part of the experiment was conducted with the blood samples of the 5 adult sickle cell anemia patients who had been on L-glutamine therapy for at least 4 weeks on a dosage of 30 grams per day compared to those of patient control group. Read More

    An in vitro evaluation of standard rotational thromboelastography in monitoring of effects of recombinant factor VIIa on coagulopathy induced by hydroxy ethyl starch.
    BMC Blood Disord 2005 Feb 15;5(1). Epub 2005 Feb 15.
    Department of Anaesthesia and Intensive Care, Lund University Hospital, Sweden.
    BACKGROUND: Rotational thromboelastography (ROTEG) has been proposed as a monitoring tool that can be used to monitor treatment of hemophilia with recombinant factor VIIa (rFVIIa). In these studies special non-standard reagents were used as activators of the coagulation. The aim of this study was to evaluate if standard ROTEG analysis could be used for monitoring of effects of recombinant factor VIIa (rFVIIa) on Hydroxy Ethyl Starch-induced dilutional coagulopathy. Read More

    Lymphocyte subsets in hemophilic patients with hepatitis C virus infection with or without human immunodeficiency virus co-infection: a nested cross-sectional study.
    BMC Blood Disord 2005 Jan 18;5(1). Epub 2005 Jan 18.
    Viral Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD; USA.
    BACKGROUND: With chronic infection, hepatitis C virus (HCV) RNA can be detected in B cells and associated with B-cell disorders, but these are not well defined. METHODS: The relationship between HCV infection and lymphocyte subpopulations was evaluated rigorously in 120 asymptomatic hemophilic patients, randomly selected from a prospective cohort study. CD4+ T cells, CD8+ T cells, CD19+ B cells, and CD56+ NK cells were quantified by flow cytometry using cryopreserved peripheral blood mononuclear cells of 24 hemophilic patients in each of five age-matched groups [uninfected; chronic HCV with or without human immunodeficiency virus (HIV); and cleared HCV with or without HIV]. Read More

    Hematopoietic chimerism after allogeneic stem cell transplantation: a comparison of quantitative analysis by automated DNA sizing and fluorescent in situ hybridization.
    BMC Blood Disord 2005 Jan 10;5(1). Epub 2005 Jan 10.
    Division of Molecular and Clinical Genetics, Institute of Human Genetics, Strzeszyñska 32, 60-479 Poznañ, Poland.
    BACKGROUND: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed mainly in patients with high-risk or advanced hematologic malignancies and congenital or acquired aplastic anemias. In the context of the significant risk of graft failure after allo-HSCT from alternative donors and the risk of relapse in recipients transplanted for malignancy, the precise monitoring of posttransplant hematopoietic chimerism is of utmost interest. Useful molecular methods for chimerism quantification after allogeneic transplantation, aimed at distinguishing precisely between donor's and recipient's cells, are PCR-based analyses of polymorphic DNA markers. Read More

    A randomized trial of washed red blood cell and platelet transfusions in adult acute leukemia [ISRCTN76536440].
    BMC Blood Disord 2004 Dec 10;4(1). Epub 2004 Dec 10.
    Department of Pathology & Laboratory Medicine (Transfusion Medicine Unit), University of Rochester Medical Center, Box 608, Rochester, NY 14642 USA.
    BACKGROUND: Platelet transfusion is universally employed in acute leukemia. Platelet concentrate supernatants contain high concentrations of biologic mediators that might impair immunity. We investigated whether washed platelet and red cell transfusions could improve clinical outcomes in adult patients with acute leukemia. Read More

    Can mutations in ELA2, neutrophil elastase expression or differential cell toxicity explain sulphasalazine-induced agranulocytosis?
    BMC Blood Disord 2004 Dec 2;4(1). Epub 2004 Dec 2.
    Department of Medical Sciences, Uppsala University, Uppsala University Hospital S- 751 85 Uppsala, Sweden.
    BACKGROUND: Drug-induced agranulocytosis, a severe side effect marked by a deficit or absolute lack of granulocytic white blood cells, is a rare side-effect of the anti-inflammatory drug sulphasalazine. Mutations in the human neutrophil elastase gene (ELA2), causing increased intracellular concentration of this serine protease, inhibits neutrophil differentiation in severe congenital neutropenia (SCN). Since the clinical symptoms of agranulocytosis and SCN are similar, we hypothesized that it may origin from a common genetic variation in ELA2 or that sulphasalazine may affect human neutrophil elastase activity and protein expression. Read More

    Effect of splenectomy on type-1/type-2 cytokine gene expression in a patient with adult idiopathic thrombocytopenic purpura (ITP).
    BMC Blood Disord 2004 Oct 18;4(1). Epub 2004 Oct 18.
    BACKGROUND: In view of clinical observations and laboratory results that support a central role of the spleen in idiopathic thrombocytopenic purpura (ITP) pathophysiology, we studied the effect of splenectomy on type-1 and type-2 cytokine gene expression in an adult ITP case, refractory to conservative treatment. CASE PRESENTATION: The patient was subjected to splenectomy 9 months after the diagnosis with complete response, attaining platelet counts over 150 x 106/L within 10 days after the operation. Two consecutive blood samples were obtained from the patient, 3 and 7 months after the splenectomy for the purposes of this study. Read More

    A mutation in a functional Sp1 binding site of the telomerase RNA gene (hTERC) promoter in a patient with Paroxysmal Nocturnal Haemoglobinuria.
    BMC Blood Disord 2004 Jun 22;4(1). Epub 2004 Jun 22.
    Department of Haematology-Division of Investigative Science, Imperial College London, Hammersmith Hospital, London, UK.
    BACKGROUND: Mutations in the gene coding for the RNA component of telomerase, hTERC, have been found in autosomal dominant dyskeratosis congenita (DC) and aplastic anemia. Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal blood disorder associated with aplastic anemia and characterized by the presence of one or more clones of blood cells lacking glycosylphosphatidylinositol (GPI) anchored proteins due to a somatic mutation in the PIGA gene. METHODS: We searched for mutations in DNA extracted from PNH patients by amplification of the hTERC gene and denaturing high performance liquid chromatography (dHPLC). Read More

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