BMC Hematology最新文献

Background: Although the demand for blood supply has progressively increased in developing countries, evidences indicate that there is a major shortage of blood and blood products in these countries, particularly in Ethiopia. Thus, identifying motivational factors affecting blood donation and recruitment of safe and low risk donors is necessary. For this reason, the study aimed at assessing knowledge, attitude, and practice towards blood donation and its associated factors.

Methods: A community based cross-sectional study was conducted in Debre Markos town from February to April, 2015. Multi-stage sampling technique was employed to recruit a total of 845 study participants. Interviewer administered questionnaire was employed as a data collection tool. Binary logistic regression was applied to assess the relationship between explanatory variables and outcome variables.

Results: In this study, 436 (56.5 %) and 403 (52.2 %) were found to be knowledgeable and having favorable attitude, respectively, while the other 124 (16.1 %) reported to have the practice of blood donation. Younger age group, male sex, those who attended formal education and radio listener were significantly associated with the knowledge of blood donation. Attending secondary and above education, having higher income, listening to radio broadcasts, and knowledge of blood donation were found to be the independent predictors of attitude. The practice of blood donation was higher among respondents who were older, attended certificate and above education, knowledgeable, and favorable attitude groups.

Conclusion: The prevalence of knowledge and practice of blood donation is found to be higher compared to similar study conducted in Mekelle, whereas the level of attitude is found to be lower. The finding of this study also justified any possible interventions on the independent predictors. There should be a regularly scheduled awareness creation and voluntary blood donation campaigns organized at the community level to utilize potential blood donors.

Background: Anticoagulant therapy is prescribed for millions of patients worldwide for the prevention and treatment of both arterial and venous thrombosis. Historically, only vitamin K antagonists have been available for clinicians to prescribe. The anticoagulation landscape is changing. The recent availability of the novel oral anticoagulants overcome many of the disadvantages associated with vitamin K antagonists. However the lack of formal monitoring and clinic follow-up is a concern for clinicians, as medication adherence is being assumed, which is known to decline in patients prescribed medications for chronic conditions. The switching study is a programme of work investigating the association between medication adherence and patient's beliefs about anticoagulation therapy (warfarin and subsequently novel oral anticoagulants), together with beliefs about their illness and anticoagulation related quality of life.

Methods/design: The anticoagulation database at King's College Hospital will be interrogated and two groups of patients will be identified; those with a time in therapeutic range on warfarin of ≥75 % and those <50 %. These groups of patients will have their illness perceptions, anticoagulation specific quality of life and beliefs about medications compared. Those patients in the time in therapeutic range <50 % group, will be then be invited to switch to a novel oral anticoagulant, as per local guidance. Those patients, who do switch, will then be followed longitudinally and have their adherence, illness perceptions, anticoagulation specific quality of life and beliefs about medications, re-evaluated on the novel agent. The results from these sub-studies, will inform a clinical pathway to support patients on these novel agents, which will be evaluated in an independent group of patients.

Discussion: The results from the switching study will be used to develop a clinical pathway to support patient's prescribed novel oral anticoagulant therapy long-term.

Background: Sickle cell disease (SCD) is endemic in non-Western countries. Due to migration, the prevalence of SCD in the Netherlands has increased. Adherence to medical treatment is recognized as a major problem area. Therefore, new effective interventions to increase adherence are urgently needed.

Methods/design: The TEAM study is an ongoing randomized controlled trial (RCT) to compare protocolized individual medical appointments (IMA's; care-as-usual) with protocolized group medical appointments (GMA's; novel intervention) in pediatric (n = 40) and adult (n = 60) patients. The study aims to assess the effectiveness of GMA's (over a three year period) on patients' self-efficacy, adherence, quality of life, morbidity, hospital admissions and satisfaction with the treating professional; as well as to test the cost-effectiveness of GMA's. In both the IMA and GMA groups structured assessments will be performed at baseline (start of the study), after 1.5 and after 3 years.

Discussion: This is the first RCT to investigate the effectiveness of GMA's on self-efficacy and adherence in pediatric and adult patients with SCD, including a cost-effectiveness analysis.

Trial registration: NTR4750 (NL42182.000.12). Registered 13 August 2014.

Background: Iron deficiency anemia is highly prevalent in patients with chronic kidney disease and is often treated with intravenous iron. There are few trials directly comparing the safety and efficacy of different intravenous iron products.

Methods: This post-hoc analysis pooled data from 767 patients enrolled in two randomized, controlled, open-label trials of similar design comparing the treatment of iron deficiency anemia with ferumoxytol and iron sucrose across patients with all stages of renal function. One trial was conducted in adults with CKD either on or not on dialysis and the second in adults with IDA of any underlying cause and a history of unsatisfactory oral iron therapy or in whom oral iron could not be used who had normal to no worse than moderately impaired renal function. Patients were categorized by chronic kidney disease stage (i.e., estimated glomerular filtration rate), and the primary efficacy endpoint was the mean change in hemoglobin from Baseline to Week 5.

Results: The overall incidence of adverse events was numerically lower in ferumoxytol-treated patients compared to those treated with iron sucrose (42.4 vs. 50.2 %, respectively); the incidence of treatment-related adverse events was generally similar between the two treatment groups (13.6 vs. 16.0 %, respectively). Adverse events of Special Interest (i.e., hypotension, hypersensitivity) occurred at lower rates in those treated with ferumoxytol compared to those treated with iron sucrose (2.5 vs. 5.3 %, respectively). Overall, mean hemoglobin increased in both treatment groups, regardless of degree of renal insufficiency, although greater increases were seen among those with less severe kidney damage. Mean increases in hemoglobin from Baseline to Week 5 were significantly greater with ferumoxytol than with iron sucrose treatment in the subgroup with an estimated glomerular filtration rate ≥90 mL/min (Least Squares mean difference = 0.53 g/dL; p < 0.001). There were no other consistent, significant differences in hemoglobin levels between treatment groups for the other chronic kidney disease categories except for isolated instances favoring ferumoxytol.

Conclusions: The efficacy and safety of ferumoxytol is at least comparable to iron sucrose in patients with varying degrees of renal function.

Trial registration: (CKD-201; ClinicalTrials.gov identifier: NCT01052779; registered 15 January, 2010), (IDA-302; ClinicalTrials.gov identifier: NCT01114204; registered 29 April, 2010).

Background: Chronic Epstein Barr virus (EBV) infection in an immunocompetent host has been described however it is not a common entity. It has been linked to many lymphoproliferative disorders and achieves such via many molecular mechanisms, some of which are poorly understood. In addition to infectious mononucleosis, the EBV is linked to various other hematological pathologies and autoimmune disorders.

Case presentation: We describe the case of an elderly immunocompetent female who presented with non-specific symptomatology, lymphadenopathy, cytopenias, elevated autoantibody titers and a crescent EBV viral load that were suggestive of a multisystemic inflammatory disease related to EBV. Extensive work up including multiple bone marrow biopsy and lymphoid tissue procedures ultimately led to the diagnosis of Hodgkin lymphoma.

Conclusion: EBV-related lymphomagenesis is complex and through the utilization of its nuclear antigens and latent membrane proteins the virus is able to shape the microenvironment to promote the various pathologies seen. Moreover, the diagnosis of EBV-associated lymphoproliferative disorders might be challenging when they present in immunocompetent individuals. Our case also represents an emphatic reminder for clinicians that spontaneous regression of lymphadenopathy is not exclusive of low-grade lymphoid malignancies.

Background: Bone marrow examination may be required to discriminate causes of thrombocytopenia as hypoproductive or hyperdestructive. However, this procedure is invasive and time consuming. This study assessed the diagnostic value of Mean Platelet Volume (MPV), Platelet Distribution Width (PDW) and Platelet Large Cell-Ratio (P-LCR) in discriminating causes of thrombocytopenia as hypoproductive or hyperdestructive (Immune thrombocytopenia purpura).

Method: A prospective cross-sectional study was conducted on 83 thrombocytopenic patients (Plt < 150 × 10(9)/L). From these, 50 patients had hypoproductive and the rest 33 Immune Thrombocytopenia Purpura (ITP). Age and sex matched 42 healthy controls were included as a comparative group. Hematological analysis was carried out using Sysmex XT 2000i 5 part diff analyzer. SPSS Version16 was used for data analysis. A two by two table and receiver operating characteristic (ROC) curve was used to calculate sensitivity, specificity, positive and negative predictive values, for a given platelet indices (MPV, PDW and P-LCR). Student t test and Mann Whitney U test were used to compare means and medians, respectively. Correlation test was used to determine associations between continuous variables.

Results: All Platelet indices were significantly higher in ITP patients (n = 33) than in hypoproductive thrombocytopenic patients (n = 50) (p < 0.0001). In particular MPV and P-LCR have larger area under ROC curve (0.876 and 0.816, respectively), indicating a better predictive capacity, sensitivity and specificity in discriminating the two causes of thrombocytopenia. The indices were still significantly higher in ITP patients compared to 42 healthy controls (p < 0.0001). A significant negative correlation was observed between platelet count and platelet indices in ITP patients, (p < 0.001).

Conclusion: MPV, PDW and P-LCR help in predicting thrombocytopenic patients as having ITP or hypoproductive thrombocytopenia. If these indices are used in line with other laboratory and clinical information, they may help in delaying/ avoiding unnecessary bone marrow aspiration in ITP patients or supplement a request for bone morrow aspiration or biopsy in hypoproductive thrombocytopenic patients.

Backgrounds: Survival analysis is commonly used to determine the treatment effect among acute lymphoblastic leukemia (ALL) patients who undergo allogeneic stem cell transplantation (allo-SCT) or other treatments. The aim of this study was to evaluate the use and reporting of survival analyses in these articles.

Methods: We performed a systematic review by searching the MEDLINE, EMBASE and Cochrane library databases from inception to April 2015. Clinical trials of patients with ALL comparing allo-SCT compared to another treatment were included. We included only studies that used survival analysis as a part of the statistical methods.

Results: There were 14 studies included in the review. Sample size estimation was described in 4 (29 %) studies. Only 4 (29 %) studies reported the list of covariates assessed in the Cox regression and 6 (43 %) studies provided a description of censorship. All studies reported survival curves using the Kaplan-Meier method. The comparisons between groups were investigated using the log-rank test and Wilcoxon test. Crossing survival curves were observed in 11(79 %) studies. The Cox regression model was incorporated in 10 (71 %) studies. None of the studies assessed the Cox proportional hazards assumption or goodness-of-fit.

Conclusions: The use and reporting of survival analysis in adult ALL patients undergoing allo-SCT have significant limitations. Notably, the finding of crossing survival curves was common and none of the studies assessed for the proportional hazards assumption. We encourage authors, reviewers and editors to improve the quality of the use and reporting of survival analysis in the hematology literature.

Background: Tuberculosis (TB) and HIV are among the risk factors for deep vein thrombosis (DVT). There are several challenges in the management of DVT patients with TB-HIV co-infection including drug-drug interactions and non-adherence due to pill burden.

Methods: HIV infected patients starting treatment for TB were identified and followed up two weekly. Cases of DVT were diagnosed with Doppler ultrasound and patients were initiated on oral anticoagulation with warfarin and followed up with repeated INR measurements and warfarin dose adjustment.

Results: We describe 7 cases of TB and HIV-infected patients in Uganda diagnosed with DVT and started on anticoagulation therapy. Their median age was 30 (IQR: 27-39) years and 86 % were male. All patients had co-medication with cotrimoxazole, tenofovir, lamivudine and efavirenz and some were on fluconazole. The therapeutic range of the International Normalization Ratio (INR) was difficult to attain and unpredictable with some patients being under-anticoagulated and others over-anticoagulated. The mean Time in Therapeutic Range (TTR) for patients who had all scheduled INR measurements in the first 12 weeks was 33.3 %. Only one patient among those with all the scheduled INR measurements had achieved a therapeutic INR by 2 weeks. Four out of seven (57 %) of the patients had at least one INR above the therapeutic range which required treatment interruption. None of the patients had major bleeding.

Conclusion: We recommend more frequent monitoring and timely dose adjustment of the INR, as well as studies on alternative strategies for the treatment of DVT in TB-HIV co-infected patients.

Background: A high prevalence of neutropenia has been reported in several ethnic groups amongst whom many healthy individuals with low neutrophil counts undergo unnecessary investigations. This study aims to ascertain the prevalence of neutropenia (NP) in a large cohort of children from North African, Middle Eastern, and Asian countries residing in the United Arab Emirates.

Methods: Neutrophil counts of 26,542 children (one day to six years of age) from 86 countries were analyzed. The subjects were enrolled in the Well-Child-Care program of Ambulatory Health Services of Emirate of Abu Dhabi, United Arab Emirates. NP was defined as a neutrophil count <1.5 × 10(9)/L and severe NP <0.5 × 10(9)/L.

Results: The neutrophil counts reached a nadir in the fourth week of life and changed slightly from the age of six-months to six-years. The frequency of NP was (from West-to-East): North African Arabs 15.4 %, Green Crescent Arabs 9.8 %, Peninsular Arabs 10.9 %, Iranians 3.1 %, Afghanis 2.5 %, Pakistanis 5.6 %, Indians 10.2 %, and Filipinos 7.3 %. The frequency of severe NP in North African Arabs (Sudanese) was 2.8 %, Green Crescent and Peninsular Arabs ≤1 %, Indians 1.5 %, and Filipinos 1.8 %. In 12,703 Emirati children, the frequency of NP was 10.6 % similar to their adult counterparts.

Conclusion: The prevalence of childhood NP varied considerably by geoethnicity. Measures to prevent the inappropriate investigations of healthy children with benign neutropenia are proposed.

Background: Multiple myeloma is a plasma cell tumour with an annual incidence in the UK of approximately 40-50 per million i.e. about 4500 new cases per annum. The triple combination cyclophosphamide, bortezomib (Velcade®) and dexamethasone (CVD) is an effective regimen at relapse and has emerged in recent years as the standard therapy at first relapse in the UK. Carfilzomib has good activity as a single agent in the relapsed setting, and it is expected that efficacy will be improved when used in combination with dexamethasone and cyclophosphamide.

Methods: MUK Five is a phase II open label, randomised, controlled, parallel group, multi-centre trial that will compare the activity of carfilzomib, cyclophosphamide and dexamethasone (CCD) with that of CVD, given over an equivalent treatment period (24 weeks), in participants with multiple myeloma at first relapse, or refractory to no more than 1 line of treatment. In addition, the study also aims to assess the utility of a maintenance schedule of carfilzomib in these participants. The primary objective of the trial is to assess whether CCD provides non-inferior activity in terms of ≥ VGPR rates at 24 weeks, and whether the addition of maintenance treatment with carfilzomib to CCD provides superior activity in terms of progression-free survival, as compared to CCD with no maintenance. Secondary objectives include comparing toxicity profiles, further summarizing and comparing the activity of the different treatment arms and analysis of the effect of each treatment arm on minimal residual disease status.

Discussion: The development of carfilzomib offers the opportunity to further explore the anti-tumour efficacy of proteasome inhibition and, based on the available evidence, it is important and timely to obtain data on the activity, toxicity and tolerability of this drug. In contrast to ongoing phase III trials, this phase II trial has a unique subset of participants diagnosed with multiple myeloma at first relapse or refractory to no more than 1 line of treatment and will also evaluate the utility of maintenance with carfilzomib for up to 18 months and investigate minimal residual disease status to provide information on depth of response and the prognostic impact thereof.

Trial registration: The trial is registered under ISRCTN17354232, December 2012.