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BMC Hematology

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https://www.readbyqxmd.com/read/27904750/hemophagocytic-lymphohistiocytosis-complicating-a-t-cell-rich-b-cell-lymphoma
#1
El Mehdi Mahtat, Maryem Zine, Mohamed Allaoui, Malika Kerbout, Nezha Messaoudi, Kamal Doghmi, Mohamed Mikdame
BACKGROUND: Hemophagocytic lymphohistiocytosis in adults is often secundary to an infection or a neoplasm. In this last case, T cell lymphomas are the most frequent causes. Hemophagocytic lymphohistiocytosis secundary to a B cell lymphoma has been rarely reported. CASE PRESENTATION: We describe a case of a hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma treated with conventionnal chemotherapy leading to a complete remission. CONCLUSION: Prompt etiologic diagnosis and treatment of hemophagocytic lymphohistiocytosis leads to satisfactory outcome...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27891232/prevalence-of-transfusion-transmissible-infections-in-blood-donors-of-pakistan
#2
Aisha Arshad, Munira Borhany, Nida Anwar, Imran Naseer, Rehan Ansari, Samson Boota, Naveena Fatima, Mustansir Zaidi, Tahir Shamsi
BACKGROUND: Transfusion-transmitted infections threaten the safety of patients requiring blood transfusion, which in turn imposes serious challenges for the availability of safe blood products that are still affordable in health care systems with limited resources. The aim of the study was to determine the prevalence of transfusion-transmitted infections in blood donors and to evaluate the demographic characteristics of reactive and non-reactive blood donors. METHODS: A prospective cohort study was conducted at our institute in Karachi, Pakistan...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27833753/adherence-to-treatment-regimen-and-bleeding-rates-in-a-prospective-cohort-of-youth-and-young-adults-on-low-dose-daily-prophylaxis-for-severe-hemophilia-a
#3
Terry Mizrahi, Jean St-Louis, Nancy L Young, Francine Ménard, Nichan Zourikian, Evemie Dubé, Georges E Rivard
BACKGROUND: When availability and/or affordability of anti-hemophilic factor concentrates are limited, optimal prophylaxis regimens in severe hemophilia A (HA) remain to be determined. In selected situations, low-dose daily prophylaxis (LDDP) may be an effective and economical option. The goal of our study was to evaluate if subjects on a LDDP regimen could achieve adherence and good clinical outcome. METHODS: Seventeen subjects (age between 15.2 and 28.4) on LDDP suffering from severe/moderate HA were followed prospectively for 2 to 3 years as part of a health-related quality of life (HRQoL) study...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27708784/kir-repertory-in-patients-with-hematopoietic-diseases-and-healthy-family-members
#4
Daniele Kazue Sugioka, Carlos Eduardo Ibaldo Gonçalves, Maria da Graça Bicalho
BACKGROUND: Since the discovery of specific histocompatibility, literature has associated genes involved in the immune response, like the Human Leucocyte Antigen (HLA), with a better prognosis in transplantation. However, other non-HLA genes may also influence the immune process, such as the genes encoding the immunoglobulin-like receptors of natural killer cells (KIRs). The discovery that NK cell KIR receptors interact with conservative epitopes (C1, C2, Bw4) presented in HLA class I molecules that are genetically polymorphic, also observed in KIR genes, led to the investigation of the relevance of the KIR system to hematopoietic stem cell transplant...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27651905/deep-vein-thrombosis-resolution-recurrence-and-post-thrombotic-syndrome-a-prospective-observational-study-protocol
#5
M Bonfield, F Cramp, J Pollock
BACKGROUND: Reasons for the variation in response of deep vein thrombosis (DVT) to anticoagulation treatment are not known. Some patients develop complications such as post-thrombotic syndrome or recurrent DVT but others make a full recovery. The aim of the study is to identify the level of variation in response to anticoagulation treatment and provide more precise and quantitative disease characterisation in response to treatment. METHODS: A prospective observational study using duplex ultrasound to examine changes in thrombus characterisation, evolution and resolution over a 2 year period in patients with a confirmed DVT...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27602227/knowledge-attitude-and-practice-towards-blood-donation-and-associated-factors-among-adults-in-debre-markos-town-northwest-ethiopia
#6
Yenework Acham Jemberu, Ahmed Esmael, Kedir Y Ahmed
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27559477/anticoagulated-patient-s-perception-of-their-illness-their-beliefs-about-the-anticoagulant-therapy-prescribed-and-the-relationship-with-adherence-impact-of-novel-oral-anticoagulant-therapy-study-protocol-for-the-switching-study-a-prospective-cohort-study
#7
Vivian Auyeung, Jignesh P Patel, John K Abdou, Bipin Vadher, Lynda Bonner, Alison Brown, Lara N Roberts, Raj K Patel, Roopen Arya
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27493757/a-randomized-controlled-trial-studying-the-effectiveness-of-group-medical-appointments-on-self-efficacy-and-adherence-in-sickle-cell-disease-team-study-study-protocol
#8
Marlous J Madderom, Jessica Heijdra, Elisabeth M W J Utens, Suzanne Polinder, Anita W Rijneveld, Marjon H Cnossen
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27462400/ferumoxytol-versus-iron-sucrose-treatment-a-post-hoc-analysis-of-randomized-controlled-trials-in-patients-with-varying-renal-function-and-iron-deficiency-anemia
#9
William E Strauss, Naomi V Dahl, Zhu Li, Gloria Lau, Lee F Allen
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27437106/chronic-epstein-barr-virus-infection-leading-to-classical-hodgkin-lymphoma
#10
Nawid M Sarwari, Joseph D Khoury, Cristhiam M Rojas Hernandez
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27375850/diagnostic-predictive-value-of-platelet-indices-for-discriminating-hypo-productive-versus-immune-thrombocytopenia-purpura-in-patients-attending-a-tertiary-care-teaching-hospital-in-addis-ababa-ethiopia
#11
Mikias Negash, Aster Tsegaye, Amha G/Medhin
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)...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27280023/a-systematic-review-of-using-and-reporting-survival-analyses-in-acute-lymphoblastic-leukemia-literature
#12
Chatree Chai-Adisaksopha, Alfonso Iorio, Christopher Hillis, Wendy Lim, Mark Crowther
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27274846/challenges-in-achieving-a-target-international-normalized-ratio-for-deep-vein-thrombosis-among-hiv-infected-patients-with-tuberculosis-a-case-series
#13
C Sekaggya, D Nalwanga, A Von Braun, R Nakijoba, A Kambugu, J Fehr, M Lamorde, B Castelnuovo
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27213048/prevalence-of-neutropenia-in-children-by-nationality
#14
Srdjan Denic, Hassib Narchi, Lolowa A Al Mekaini, Suleiman Al-Hammadi, Omar N Al Jabri, Abdul-Kader Souid
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27190631/the-muk-five-protocol-a-phase-ii-randomised-controlled-parallel-group-multi-centre-trial-of-carfilzomib-cyclophosphamide-and-dexamethasone-ccd-vs-cyclophosphamide-bortezomib-velcade-and-dexamethasone-cvd-for-first-relapse-and-primary-refractory-multiple-myeloma
#15
Sarah Brown, Samantha Hinsley, Mónica Ballesteros, Sue Bourne, Paul McGarry, Debbie Sherratt, Louise Flanagan, Walter Gregory, Jamie Cavenagh, Roger Owen, Cathy Williams, Martin Kaiser, Eric Low, Kwee Yong
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...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27158500/generic-and-disease-specific-quality-of-life-among-youth-and-young-men-with-hemophilia-in-canada
#16
J St-Louis, D J Urajnik, F Ménard, S Cloutier, R J Klaassen, B Ritchie, G E Rivard, M Warner, V Blanchette, N L Young
BACKGROUND: This study was undertaken to explore the longitudinal patterns of health-related quality of life (HRQoL) among youth and young adults with Hemophilia A (HA) over a 3-year period. This report presents the baseline characteristics of the study cohort. METHODS: Males, 14 to 29 years of age, with predominantly severe HA were recruited from six treatment centres in Canada. Subjects completed a comprehensive survey. HRQoL was measured using: the CHO-KLAT2...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27148452/design-and-rationale-of-the-quazar-lower-risk-mds-aza-mds-003-trial-a-randomized-phase-3-study-of-cc-486-oral-azacitidine-plus-best-supportive-care-vs-placebo-plus-best-supportive-care-in-patients-with-ipss-lower-risk-myelodysplastic-syndromes-and-poor-prognosis
#17
Guillermo Garcia-Manero, Antonio Almeida, Aristoteles Giagounidis, Uwe Platzbecker, Regina Garcia, Maria Teresa Voso, Stephen R Larsen, David Valcarcel, Lewis R Silverman, Barry Skikne, Valeria Santini
BACKGROUND: CC-486 is an oral formulation of the epigenetic modifier azacitidine. In an expanded phase 1 trial, CC-486 demonstrated clinical and biological activity in patients with International Prognostic Scoring System (IPSS) lower-risk (low- and intermediate-1-risk) myelodysplastic syndromes (MDS) with poor prognostic features including anemia and/or thrombocytopenia who may have required red blood cell or platelet transfusions. The overall response rate was 40 %, including hematologic improvement in 28 % of patients and RBC transfusion independence sustained for 56 days in 47 % of patients with baseline transfusion dependence...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27127633/kinetics-of-langerhans-cell-chimerism-in-the-skin-of-dogs-following-2%C3%A2-gy-tbi-allogeneic-hematopoietic-stem-cell-transplantation
#18
Sabrina Peters, Christian Junghanss, Anne Knueppel, Hugo Murua Escobar, Catrin Roolf, Gudrun Knuebel, Anett Sekora, Iris Lindner, Ludwig Jonas, Mathias Freund, Sandra Lange
BACKGROUND: Langerhans cells (LC) are bone marrow-derived cells in the skin. The LC donor/recipient chimerism is assumed to influence the incidence and severity of graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT). In nonmyeloablative (NM) HSCT the appearance of acute GVHD is delayed when compared with myeloablative conditioning. Therefore, we examined the development of LC chimerism in a NM canine HSCT model. METHODS: 2 Gy conditioned dogs received bone marrow from dog leukocyte antigen identical littermates...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27110362/characteristics-of-chronic-lymphocytic-leukemia-in-senegal
#19
Abibatou Sall, Awa Oumar Touré, Fatimata Bintou Sall, Moussa Ndour, Seynabou Fall, Abdoulaye Sène, Blaise Félix Faye, Moussa Seck, Macoura Gadji, Tandakha Ndiaye Dièye, Claire Mathiot, Sophie Reynaud, Saliou Diop, Martine Raphaël
BACKGROUND: Chronic lymphocytic leukemia (CLL) is a mature B-cell neoplasm characterized by the expansion of CD5-positive lymphocytes in peripheral blood. While CLL is the most common type of leukemia in Western populations, the disease is rare in Africans. Hence, clinical and laboratory data and studies of CLL in Sub Saharan populations have been limited. The aims of this study were to analyze the characteristics of senegalese patients with CLL at the time of the diagnosis and to identify the correlation between clinical characteristics (Binet stage) with age, gender, laboratory parameters and chromosomal abnormalities...
2016: BMC Hematology
https://www.readbyqxmd.com/read/27081493/endothelial-fibrinolytic-response-onto-an-evolving-matrix-of-fibrin
#20
O Castillo, H Rojas, Z Domínguez, E Anglés-Cano, R Marchi
BACKGROUND: Fibrin provides a temporary matrix at the site of vascular injury. The aims of the present work were (1) to follow fibrin formation and lysis onto the surface of human dermal microvascular endothelial cells (HMEC-1), and (2) to quantify the secretion of fibrinolytic components in the presence of fibrin. METHODS: Fibrin clots at different fibrinogen concentrations were formed on top of (model 1) or beneath (model 2) the endothelial cells. Fibrin formation or lysis onto the surface of HMEC-1 cells, was followed by turbidity...
2016: BMC Hematology
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