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Journal of Blood Medicine

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https://www.readbyqxmd.com/read/27853395/animal-models-of-%C3%AE-hemoglobinopathies-utility-and-limitations
#1
REVIEW
Bradley McColl, Jim Vadolas
The structural and functional conservation of hemoglobin throughout mammals has made the laboratory mouse an exceptionally useful organism in which to study both the protein and the individual globin genes. Early researchers looked to the globin genes as an excellent model in which to examine gene regulation - bountifully expressed and displaying a remarkably consistent pattern of developmental activation and silencing. In parallel with the growth of research into expression of the globin genes, mutations within the β-globin gene were identified as the cause of the β-hemoglobinopathies such as sickle cell disease and β-thalassemia...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27843377/pain-frequency-severity-and-qt-dispersion-in-adult-patients-with-sickle-cell-anemia-correlation-with-inflammatory-markers
#2
Taysir S Garadah, Ahmed A Jaradat, Mohammed E AlAlawi, Adla B Hassan, Reginald P Sequeira
BACKGROUND: Inflammatory markers are increased during vaso-occlusive crisis (VOC) in adult patients with sickle cell anemia (SCA), but this is not clear in clinical steady state. AIM: The present study aims to establish the frequency and intensity of bone pain episodes in adult patients with SCA in clinical steady state and to determine the correlation between different inflammatory markers, other variables including QT dispersion (QTd) and pain frequency and intensity in SCA...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27822129/bayesian-approach-to-the-assessment-of-the-population-specific-risk-of-inhibitors-in-hemophilia-a-patients-a-case-study
#3
Ji Cheng, Alfonso Iorio, Maura Marcucci, Vadim Romanov, Eleanor M Pullenayegum, John K Marshall, Lehana Thabane
BACKGROUND: Developing inhibitors is a rare event during the treatment of hemophilia A. The multifacets and uncertainty surrounding the development of inhibitors further complicate the process of estimating inhibitor rate from the limited data. Bayesian statistical modeling provides a useful tool in generating, enhancing, and exploring the evidence through incorporating all the available information. METHODS: We built our Bayesian analysis using three study cases to estimate the inhibitor rates of patients with hemophilia A in three different scenarios: Case 1, a single cohort of previously treated patients (PTPs) or previously untreated patients; Case 2, a meta-analysis of PTP cohorts; and Case 3, a previously unexplored patient population - patients with baseline low-titer inhibitor or history of inhibitor development...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27799837/utility-of-preoperative-in-vitro-platelet-function-tests-for-predicting-bleeding-risk-in-patients-undergoing-functional-endoscopic-sinus-surgery
#4
A-Jin Lee, Sang-Gyung Kim
BACKGROUND: It is necessary to predict the bleeding risk in patients undergoing functional endoscopic sinus surgery (FESS). To evaluate the adequacy of primary hemostasis, preoperative hemostatic screening tests are used. In the present study, we determined whether there is a positive correlation between prolonged closure time (CT) with collagen/epinephrine (CT-epi), prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin time (aPTT) and bleeding during FESS...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27799836/erratum-reducing-the-iron-burden-and-improving-survival-in-transfusion-dependent-thalassemia-patients-current-perspectives-corrigendum
#5
(no author information available yet)
[This corrects the article on p. 159 in vol. 7, PMID: 27540317.].
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27785117/a-retrospective-analysis-of-56-children-with-hemophagocytic-lymphohistiocytosis
#6
Yan-Rong Wang, Yi-Ning Qiu, Yan Bai, Xian-Feng Wang
AIM: The aim of this study was to investigate the etiological factors, clinical features, and prognostic factors in children with hemophagocytic lymphohistiocytosis (HLH). METHODS: Fifty-six children with HLH in Wuhan Union Hospital, People's Republic of China, were retrospectively analyzed in recent years. We reviewed the medical records of 56 HLH children hospitalized from 2000 to 2013 to identify the possible prognostic factors. RESULTS: In more than half of the cases (64...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27729820/ruxolitinib-in-the-treatment-of-polycythemia-vera-patient-selection-and-special-considerations
#7
Sabine Blum, Filipe Martins, Lorenzo Alberio
The discovery of JAK2 V617F mutation in the mid-2000s started to fill the gap between clinical presentation of polycythemia vera (PV), first described by Vaquez at the end of the 19th century, and spontaneous erythroid colony formation, reported by Prchal and Axelrad in the mid-1970s. The knowledge on this mutation brought an important insight to our understanding of PV pathogenesis and led to a revision of the World Health Organization diagnostic criteria in 2008. JAK-STAT is a major signaling pathway implicated in survival and proliferation of hematopoietic precursors...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27713652/acquired-hypofibrinogenemia-current-perspectives
#8
Martin W Besser, Stephen G MacDonald
Acquired hypofibrinogenemia is most frequently caused by hemodilution and consumption of clotting factors. The aggressive replacement of fibrinogen has become one of the core principles of modern management of massive hemorrhage. The best method for determining the patient's fibrinogen level remains controversial, and particularly in acquired dysfibrinogenemia, could have major therapeutic implications depending on which quantification method is chosen. This review introduces the available laboratory and point-of-care methods and discusses the relative advantages and limitations...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27703400/body-surface-area-a-predictor-of-response-to-red-blood-cell-transfusion
#9
Louise Man, H Raymond Tahhan
A current focus of transfusion medicine is a judicious strategy in transfusion of blood products. Unfortunately, our ability to predict hemoglobin (Hgb) response to transfusion has been limited. The objective of this study was to determine variability of response to red blood cell transfusion and to predict which patients will have an Hgb rise higher or lower than that predicted by the long-standing convention of "one and three". This was a retrospective chart review in a single hospital. Data for 167 consecutive patient encounters were reviewed...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27695377/therapeutic-and-routine-prophylactic-properties-of-rfactor-viii-fc-efraloctocog-alfa-eloctate-%C3%A2-in-hemophilia-a
#10
Pratima Chowdary, Emma Fosbury, Anne Riddell, Mary Mathias
rFVIIIFc (efraloctocog alfa, Eloctate(®)) is an extended half-life (EHL) factor VIII licensed for use in patients with hemophilia A for prophylaxis and treatment of bleeding and surgical episodes. Pharmacokinetic studies in adults have shown a mean 1.5-fold increase in half-life compared to full-length factor VIII. When compared to adults, the half-life is decreased by 8% in adolescents between 12 and 17 years, by 18% in children 6 to <12 years, and by 33% in children between the ages of 2 and <6 years...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27621680/hematopoietic-cell-transplantation-associated-thrombotic-microangiopathy-a-review-of-pathophysiology-diagnosis-and-treatment
#11
REVIEW
Joseph Rosenthal
Transplant-associated thrombotic microangiopathy (TA-TMA) is a multifactorial disorder caused by systemic vascular endothelial injury that can be triggered by several mechanisms during the transplant process. Thrombotic microangiopathy may affect multiple systems and occurs in ~30% of patients undergoing hematopoietic stem cell transplantation. A subgroup of patients with thrombotic microangiopathy develop TA-TMA, and the other may develop other thrombotic microangiopathic disorders such as thrombotic thrombocytopenic purpura, a condition with similar finding but different pathophysiology involving ADAMTS-13...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27621679/idh1-and-idh2-mutations-as-novel-therapeutic-targets-current-perspectives
#12
REVIEW
Johanna Mondesir, Christophe Willekens, Mehdi Touat, Stéphane de Botton
Isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) are key metabolic enzymes that convert isocitrate to α-ketoglutarate. IDH1/2 mutations define distinct subsets of cancers, including low-grade gliomas and secondary glioblastomas, chondrosarcomas, intrahepatic cholangiocarcinomas, and hematologic malignancies. Somatic point mutations in IDH1/2 confer a gain-of-function in cancer cells, resulting in the accumulation and secretion in vast excess of an oncometabolite, the D-2-hydroxyglutarate (D-2HG). Overproduction of D-2HG interferes with cellular metabolism and epigenetic regulation, contributing to oncogenesis...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27540317/reducing-the-iron-burden-and-improving-survival-in-transfusion-dependent-thalassemia-patients-current-perspectives
#13
REVIEW
Karim Bayanzay, Lama Alzoebie
Hypertransfusion regimens for thalassemic patients revolutionized the management of severe thalassemia; transforming a disease which previously led to early infant death into a chronic condition. The devastating effect of the accrued iron from chronic blood transfusions necessitates a more finely tuned approach to limit the complications of the disease, as well as its treatment. A comprehensive approach including carefully tailored transfusion protocol, continuous monitoring and assessment of total body iron levels, and iron chelation are currently the mainstay in treating iron overload...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27536166/update-and-new-approaches-in-the-treatment-of-castleman-disease
#14
REVIEW
Kah-Lok Chan, Stephen Lade, H Miles Prince, Simon J Harrison
First described 60 years ago, Castleman disease comprises a rare and heterogeneous cluster of disorders, characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical disturbances. Although unicentric Castleman disease is curable with complete surgical excision, its multicentric counterpart is a considerable therapeutic challenge. The recent development of biological agents, particularly monoclonal antibodies to interleukin-6 and its receptor, allow for more targeted disease-specific intervention that promises improved response rates and more durable disease control; however, further work is required to fill knowledge gaps in terms of underlying pathophysiology and to facilitate alternative treatment options for refractory cases...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27486346/clinical-applications-of-therapeutic-phlebotomy
#15
Kyung Hee Kim, Ki Young Oh
Phlebotomy is the removal of blood from the body, and therapeutic phlebotomy is the preferred treatment for blood disorders in which the removal of red blood cells or serum iron is the most efficient method for managing the symptoms and complications. Therapeutic phlebotomy is currently indicated for the treatment of hemochromatosis, polycythemia vera, porphyria cutanea tarda, sickle cell disease, and nonalcoholic fatty liver disease with hyperferritinemia. This review discusses therapeutic phlebotomy and the related disorders and also offers guidelines for establishing a therapeutic phlebotomy program...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27445511/matching-adjusted-indirect-comparisons-of-efficacy-of-bay-81-8973-vs-two-recombinant-factor-viii-for-the-prophylactic-treatment-of-severe-hemophilia-a
#16
Jennifer Pocoski, Nanxin Li, Rajeev Ayyagari, Nikki Church, Monika Maas Enriquez, Quer Xiang, Sneha Kelkar, Ella X Du, Eric Q Wu, Jipan Xie
BACKGROUND: No head-to-head trials comparing recombinant factor VIII (rFVIII) products currently exist. This was a matching-adjusted indirect comparison (MAIC) study of efficacy of BAY 81-8973 with antihemophilic factor (recombinant) plasma/albumin-free method (rAHF-PFM) and turoctocog alfa for the prophylaxis of severe hemophilia A. METHODS: A systematic literature review was conducted to identify trials of rAHF-PFM and turoctocog alfa. Comparisons were conducted using BAY 81-8973 individual patient data (IPD) from LEOPOLD trials and published data from rAHF-PFM and turoctocog alfa trials...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27382347/potential-role-of-a-new-pegylated-recombinant-factor-viii-for-hemophilia-a
#17
REVIEW
Tung Thanh Wynn, Burak Gumuscu
Hemophilia A, a deficiency in the activity of coagulation factor (F) VIII, is an X-linked bleeding disorder with an approximate incidence of one in 5,000 male infants. Bleeding-related complications often result in greater severity of disease, poor quality of life, surgical interventions for severe joint destruction, and shortened life span. With the availability of plasma-derived and recombinant FVIII products, the benefits of primary prophylaxis were demonstrated and is now the standard of care for patients with severe factor deficiencies...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27330333/clinical-association-of-baseline-levels-of-conjugated-dienes-in-low-density-lipoprotein-and-nitric-oxide-with-aggressive-b-cell-non-hodgkin-lymphoma-and-their-relationship-with-immunoglobulins-and-th1-to-th2-ratio
#18
Mustapha Haddouche, Warda Meziane, Zeyneb Hadjidj, Naima Mesli, Mourad Aribi
OBJECTIVE: The aim of this study was to highlight the clinical association of baseline levels of conjugated dienes in low-density lipoprotein (LDL-BCD) and nitric oxide (NO) with immunoglobulins (Igs) and T helper (Th)1/Th2 ratio in patients with newly diagnosed B-cell non-Hodgkin lymphoma (NHL). PATIENTS AND METHODS: Thirty-two newly diagnosed patients with aggressive B-cell NHL and 25 age-, sex-, and body-mass-index-matched healthy controls were randomly selected for a cross-sectional case-control study conducted at the Hematology Department of Tlemcen Medical Centre University (northwest of Algeria)...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27313483/development-of-paroxysmal-nocturnal-hemoglobinuria-in-calr-positive-myeloproliferative-neoplasm
#19
Yarden S Fraiman, Nathan Cuka, Denise Batista, Milena Vuica-Ross, Alison R Moliterno
Paroxysmal nocturnal hemoglobinuria (PNH), a disease characterized by intravascular hemolysis, thrombosis, and bone marrow failure, is associated with mutations in the PIG-A gene, resulting in a deficiency of glycosylphosphatidylinositol-anchored proteins. Many hypotheses have been posed as to whether PNH and PIG-A mutations result in an intrinsic survival benefit of CD55(-)/CD59(-) cells or an extrinsic permissive environment that allows for their clonal expansion within the bone marrow compartment. Recent data have identified the concurrence of PIG-A mutations with additional genetic mutations associated with myeloproliferative disorders, suggesting that some presentations of PNH are the result of a stepwise progression of genetic mutations similar to other myelodysplastic or myeloproliferative syndromes...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27307776/long-term-safety-and-efficacy-of-romiplostim-for-treatment-of-immune-thrombocytopenia
#20
REVIEW
Prakash Vishnu, David M Aboulafia
Inhibition of platelet production and mediated by antiplatelet antibodies is a well-known mechanism causing low platelet counts in immune thrombocytopenia (ITP). Use of thrombopoietin receptor agonists increases platelet counts and decreases the risk of bleeding in patients with ITP. Two such thrombopoietin receptor agonists, romiplostim and eltrombopag, are approved by the US Food and Drug Administration to treat thrombocytopenia in adults, and most recently, children with persistent or chronic ITP. This review focuses on the efficacy data and safety analysis of the pooled data from the clinical trials evaluating romiplostim for treatment of adults with ITP...
2016: Journal of Blood Medicine
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