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Journal of Blood Medicine

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https://www.readbyqxmd.com/read/30214334/thalassemia-minor-presenting-with-vitamin-b-12-deficiency-paraparesis-and-microcytosis
#1
Arwa Lardhi, Rania Alhaj Ali, Rola Ali, Tarek Mohammed
Vitamin B12 is essential for proper neurological functioning, and its deficiency may cause a wide range of neuropsychiatric and hematological manifestations. We report a case of a previously healthy 32-year-old female who was admitted to our hospital with sudden onset of bilateral lower limb paraparesis and loss of sensation. The serum level of vitamin B12 was mildly decreased with high methylmalonic acid and homocysteine levels. However, her complete blood count showed no evidence of anemia or macrocytosis; instead, her mean corpuscular volume was low...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/30174468/an-investigational-rnai-therapeutic-targeting-antithrombin-for-the-treatment-of-hemophilia-a-and-b
#2
REVIEW
Nicoletta Machin, Margaret V Ragni
Fitusiran is an RNA interference therapeutic that targets antithrombin (AT) in the liver and interferes with AT translation by binding and degrading messenger RNA-AT, thereby silencing AT gene expression and preventing AT synthesis. In both preclinical and clinical studies, AT knockdown results in dose-dependent AT lowering when fitusiran is given weekly or monthly subcutaneously. In clinical trials, fitusiran dose escalation has resulted in improved thrombin generation and clinical hemostasis as measured by reduction in annualized bleed rate...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/30154676/optimizing-transfusion-strategies-in-damage-control-resuscitation-current-insights
#3
REVIEW
Timothy H Pohlman, Alison M Fecher, Cecivon Arreola-Garcia
From clinical and laboratory studies of specific coagulation defects induced by injury, damage control resuscitation (DCR) emerged as the most effective management strategy for hemorrhagic shock. DCR of the trauma patient who has sustained massive blood loss consists of 1) hemorrhage control; 2) permissive hypotension; and 3) the prevention and correction of trauma-induced coagulopathies, referred to collectively here as acute coagulopathy of trauma (ACOT). Trauma patients with ACOT have higher transfusion requirements, may eventually require massive transfusion, and are at higher risk of exsanguinating...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29983600/hematological-abnormalities-in-hiv-antiretroviral-therapy-na%C3%A3-ve-clients-as-seen-at-an-immune-suppression-syndrome-clinic-at-mbarara-regional-referral-hospital-southwestern-uganda
#4
Crispus Katemba, Conrad Muzoora, Enoch Muwanguzi, Bashir Mwambi, Christine Atuhairwe, Ivan M Taremwa
Aim/objective: To assess the common hematological abnormalities among HIV-antiretroviral therapy (ART) naïve clients attending an immune suppression syndrome (ISS) clinic at Mbarara Regional Referral Hospital (MRRH), southwestern Uganda. Patients and methods: This was a cross-sectional study carried out during the months of March to August 2016 at the ISS clinic of MRRH. We collected approximately 4.0 mL of EDTA anticoagulated blood samples, which were assayed for complete blood count, CD4+ cell count and thin film examination...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29970970/biochemical-changes-in-stored-donor-units-implications-on-the-efficacy-of-blood-transfusion
#5
Caesar Oyet, Benson Okongo, Richard Apecu Onyuthi, Enoch Muwanguzi
Background: Blood transfusion with allogeneic blood products is a common medical intervention to treat anemia or prepare patients for surgical procedures. Generally, the blood units are secured and stored prior to expected transfusion. During storage, a number of biochemical changes occur (generally known as storage lesion), which can affect the efficacy of blood transfusion. The aim of the study was to evaluate the biochemical changes that occur in blood units during storage and to project the impact of these changes on transfusion...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29950916/serum-of-sickle-cell-disease-patients-contains-fetal-hemoglobin-silencing-factors-secreted-from-leukocytes
#6
Tohru Ikuta, Hassan Sellak, Si-Yang Liu, Nadine Odo
Background: The mechanisms that regulate fetal hemoglobin (HbF) expression in sickle cell disease (SCD) remain elusive. We previously showed that steady-state SCD patients with high HbF levels due to a γ-globin gene mutation demonstrate strong inverse correlations between HbF levels and leukocyte counts, suggesting that leukocytes play a role in regulating HbF in SCD. Materials and methods: To further investigate the role of leukocytes in HbF expression in SCD, we examined the presence of HbF silencing factors in the serum of 82 SCD patients who received hydroxyurea (HU) therapy...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29950915/rhesus-blood-group-haplotype-frequencies-among-blood-donors-in-southwestern-uganda
#7
Yona Mbalibulha, Enoch Muwanguzi, Godfrey Mugyenyi
Aim/objective: The study was undertaken to determine the Rhesus blood group system and Rhesus haplotype frequencies among blood donors at Mbarara Regional Blood Bank. Materials and methods: We included ethylene-diaminetetra-acetic acid-containing plasma samples and serum samples from recruited consented blood donors. The Rh blood group system and the Rh haplotypes was established by the incubation of appropriate antisera (anti-D, anti-E, anti-C, anti-e, and anti-c) and cells at a temperature of 24°C in microplates for 1 hour and the reaction was read by gentle shaking and examining for agglutinations...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29950914/implementation-of-a-patient-blood-management-program-in-an-australian-private-hospital-orthopedic-unit
#8
Paul N Morgan, Patrick L Coleman, Cintia Mayel Martinez-Garduno, Anoja W Gunaratne, Elizabeth McInnes, Sandy Middleton
Background: Preoperative anemia in surgical patients has been linked to increased rates of allogeneic blood transfusion (ABT) and associated adverse patient outcomes such as prolonged ventilation in intensive care, increased length of hospital stay, and infections. We conducted a multifaceted implementation for orthopedic surgeons to improve preoperative patient assessment of anemia and iron deficiency to reduce perioperative blood transfusions. Materials and methods: Using a before-and-after study design of independent samples, we recruited a convenience sample of surgeons who performed primary total hip arthroplasty at 1 Australian private hospital...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29892205/the-public-s-risk-perception-of-blood-transfusion-in-saudi-arabia
#9
Adel F Almutairi, Mahmoud Salam, Oraynab Abou Abbas, Maliha Nasim, Abdallah A Adlan
Background: Human beings may face many circumstances, such as surgery, trauma, and anemia, in which they could require an urgent blood transfusion. However, only a few studies have examined people's risk perception of blood transfusion. Therefore, this study aims to evaluate the public's risk perception of blood transfusion in Saudi Arabia, and to identify factors associated with their risk perception. Methods: Self-reported questionnaires on blood transfusion risk perception were distributed to the public during a Saudi national festival in Riyadh...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29713210/inotuzumab-ozogamicin-in-the-treatment-of-relapsed-refractory-acute-b-cell-lymphoblastic-leukemia
#10
REVIEW
Natalie Uy, Michelle Nadeau, Maximilian Stahl, Amer M Zeidan
The improvement in outcomes of adult patients with acute lymphoblastic leukemia (ALL) has been modest, with the exception of Philadelphia chromosome-positive disease, despite advances in supportive care and stem cell transplantation. The recent approvals of novel agents, including the bispecific T-cell engager blinatumomab, the antibody-drug conjugate inotuzumab ozogamicin, and chimeric antigen receptor T-cell products are changing the management of B-ALL, which traditionally relied on chemotherapy-based approaches...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29713209/adoption-of-the-american-academy-of-pediatrics-neonatal-hyperbilirubinemia-guidelines-and-its-effect-on-blood-exchange-transfusion-rate-in-a-tertiary-care-center-in-amman-jordan
#11
Manar Al-Lawama, Eman Al-Rimawi, Rawan Al-Shibi, Eman Badran
Introduction: Severe neonatal hyperbilirubinemia can cause mortality and serious morbidities. When phototherapy fails, neonates with severe hyperbilirubinemia should undergo double volume blood exchange transfusion (BET). As this procedure carries a significant risk of mortality and morbidity, adopting guidelines for the treatment of neonatal hyperbilirubinemia is critical to avoid hyperbilirubinemia toxicity and also the complication of an unindicated procedure. Methods: This study investigated the causes, complications, and trend of BET rate in our unit over a 13-year period...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29713208/educational-needs-of-hematologists-and-laboratory-professionals-regarding-factor-activity-assays
#12
Dorothy M Adcock, Mazi Rasulnia, Natalia Holot, David L Cooper
Introduction: Diagnosis and management of hemophilia require accurate and precise measurements of factor activity levels. Activity is traditionally measured via one-stage (OS) clot-based assay; however, chromogenic substrate (CS) assays may be needed for certain cases. A survey was performed to understand assay-related knowledge gaps among hematologists and laboratory professionals. Methods: Separate web-based surveys were administered to hematologists who manage hemophilia and to laboratory professionals and queried practice patterns, knowledge of/attitudes toward CS assays, and interest in continuing education...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29695943/bosutinib-in-chronic-myeloid-leukemia-patient-selection-and-perspectives
#13
REVIEW
Susanne Isfort, Tim H Brümmendorf
During recent years, the therapeutic landscape in chronic myeloid leukemia (CML) has changed significantly. Since the clinical introduction of tyrosine kinase inhibitors (TKIs) approximately 15 years ago, patients' concerns have shifted from reduced life expectancy toward long-term toxicities of TKI, depth of remission, and the probability of successful treatment discontinuation. Patients with newly diagnosed CML in chronic phase (at least with a Sokal score not exceeding intermediate) may now expect an almost normal life expectancy...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29695942/genotype-phenotype-correlation-among-beta-thalassemia-and-beta-thalassemia-hbe-disease-in-thai-children-predictable-clinical-spectrum-using-genotypic-analysis
#14
Chanchai Traivaree, Chalinee Monsereenusorn, Piya Rujkijyanont, Warakorn Prasertsin, Boonchai Boonyawat
Introduction: Beta-thalassemia is a group of inherited hemolytic anemias and one of the most common genetic disorders in Thailand. The clinical spectrum of beta-thalassemia disease ranges from mild to severe clinical symptoms including mild beta-thalassemia intermedia (TI) and severe beta-thalassemia major (TM). Objective: This study aimed to determine the correlation between beta-globin gene ( HBB ) mutations and their phenotypic manifestations by evaluating patients' clinical characteristics, transfusion requirements, growth and hematologic parameters, and hemoglobin typing among pediatric patients treated at Phramongkutklao Hospital...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29503590/direct-oral-anticoagulants-for-extended-thromboprophylaxis-in-medically-ill-patients-meta-analysis-and-risk-benefit-assessment
#15
Majed S Al Yami, Sawsan Kurdi, Ivo Abraham
Background: Standard-duration (7-10 days) thromboprophylaxis with low molecular weight heparin, low dose unfractionated heparin, or fondaparinux in hospitalized medically ill patients is associated with ~50% reduction in venous thromboembolism (VTE) risk. However, these patients remain at high risk for VTE post-discharge. The direct oral anticoagulants (DOACs) apixaban, rivaroxaban and betrixaban have been evaluated for extended-duration (30-42 days) thromboprophylaxis in this population...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29403325/tafro-syndrome-current-perspectives
#16
REVIEW
Kentaro Sakashita, Kengo Murata, Mikio Takamori
Multicentric Castleman's disease (MCD), a distinct subtype of Castleman's disease, is a rare, nonneoplastic, lymphoproliferative disorder. Patients with MCD present with systemic symptoms and multiple lymphadenopathy. Lymph node biopsy is necessary for the diagnosis of various histological MCD patterns including hyaline vascular, plasma cell, and mixed types. Human herpesvirus 8 (HHV8) infection was identified as an important etiology of MCD among immunocompromised patients such as those positive for human immunodeficiency virus...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29403324/pharmacokinetics-based-clinical-management-of-acquired-von-willebrand-syndrome-a-case-report
#17
Candice M Baldeo, Candido E Rivera, Han W Tun, Prakash Vishnu
von Willebrand disease (VWD) is a common bleeding disorder caused by defective or low levels of von Willebrand factor (VWF). Although most cases of VWD are caused by genetic mutations, some are acquired due to various disease states. In managing VWD, the aim is to normalize plasma levels of both VWF and factor VIII (FVIII), as this aids in hemostasis. Desmopressin usually corrects VWF level in type 1 VWD by inducing the release of endogenous VWF. In cases where desmopressin is ineffective or cannot be used, transfusion of virally inactivated, plasma-derived VWF/FVIII concentrate or infusion of recombinant VWF (Vonvendi) is indicated...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29403323/evaluation-of-thrombosis-related-biomarkers-before-and-after-therapy-in-patients-with-multiple-myeloma
#18
Shosaku Nomura, Tomoki Ito, Hideaki Yoshimura, Masaaki Hotta, Takahisa Nakanishi, Shinya Fujita, Aya Nakaya, Atsushi Satake, Kazuyoshi Ishii
Background: Thrombosis is one of the complications in the clinical course of multiple myeloma (MM). Vascular endothelial cells and/or the hemostatic-coagulatory system are thought to play an important role in thrombosis of MM. In addition to melphalan-prednisone (Mel-P) therapy, several new therapeutic drugs such as lenalidomide or bortezomib have been developed and show effectiveness against MM. However, these new drugs also have risk of therapy-related thrombosis. Methods: We assessed 103 MM patients and 30 healthy controls, using enzyme-linked immunosorbent assays to evaluate five biomarkers: platelet-derived microparticles (PDMP), plasminogen activator inhibitor-1 (PAI-1), high mobility group box protein-1 (HMGB1), endothelial protein C receptor (EPCR), and soluble vascular cell adhesion molecule-1 (sVCAM-1)...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29263710/efficacy-and-safety-of-iv-ferumoxytol-for-iron-deficiency-anemia-in-patients-with-cancer
#19
Saroj Vadhan-Raj, Naomi V Dahl, Kristine Bernard, Zhu Li, William E Strauss
Purpose: Iron deficiency anemia (IDA) is common in cancer patients due to blood loss and inflammation. Many do not tolerate oral iron or adequately respond. Intravenous (IV) iron is commonly used as an adjunct to erythropoiesis-stimulating agents; data on the use of IV iron monotherapy in these patients are limited. This study aimed to evaluate IV ferumoxytol for the treatment of cancer patients with IDA with a history of unsatisfactory oral iron therapy or in whom oral iron could not be used...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29184457/evaluation-of-eu-legislation-on-blood-a-bioethical-point-of-view
#20
EDITORIAL
Carlo Petrini
A review of the European Union (EU) regulations concerning blood, tissues, and cells of human origin is under way in the EU. From the ethical point of view, the non-remuneration of donations and the ban on deriving gain from human biological materials are of particular significance. While the basic ethical principles involved in the procurement, preservation, and use of these materials are the same, their practical application should be adapted to the specific context of each material. In the case of donation and use of blood, in particular, the issue of legitimate reimbursements to donors and for transfusion centers has to be managed in accordance with the principle of non-commercialization...
2017: Journal of Blood Medicine
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