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Blood Cells, Molecules & Diseases

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https://www.readbyqxmd.com/read/29137845/enumeration-of-bone-marrow-plasmacytoid-dendritic-cells-by-multiparameter-flow-cytometry-as-a-prognostic-marker-following-allogeneic-hematopoietic-stem-cell-transplantation
#1
Ruijun Jeanna Su, Ralph Green, Mingyi Chen
Plasmacytoid dendritic cells (pDCs) promote tolerance in solid organ transplants and hematopoietic stem cell transplantation (HSCT). pDCs originate from CD34(+) hematopoietic progenitors. Following allogeneic hematopoietic stem cell transplant (allo-HSCT), pDC reconstitution in the BM and PB gradually attain levels similar to those in healthy individuals. We have investigated the recovery of pDC following allo-HSCT as a means to predict successful marrow engraftment. We retrospectively studied immune reconstitution of pDC in the BM of 48 patients following allo-HSCT for initial diagnoses of leukemia or other malignancies...
October 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29126700/structure-and-function-of-haemoglobins
#2
REVIEW
David A Gell
Haemoglobin (Hb) is widely known as the iron-containing protein in blood that is essential for O2 transport in mammals. Less widely recognised is that erythrocyte Hb belongs to a large family of Hb proteins with members distributed across all three domains of life-bacteria, archaea and eukaryotes. This review, aimed chiefly at researchers new to the field, attempts a broad overview of the diversity, and common features, in Hb structure and function. Topics include structural and functional classification of Hbs; principles of O2 binding affinity and selectivity between O2/NO/CO and other small ligands; hexacoordinate (containing bis-imidazole coordinated haem) Hbs; bacterial truncated Hbs; flavohaemoglobins; enzymatic reactions of Hbs with bioactive gases, particularly NO, and protection from nitrosative stress; and, sensor Hbs...
October 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29108692/a-novel-nonsense-mutation-in-a-patient-with-hermansky-pudlak-syndrome-type-4
#3
Kirstin Sandrock-Lang, Doris Böckelmann, Wolfgang Eberl, Sophie Schmitt-Kästner, Barbara Zieger
No abstract text is available yet for this article.
October 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29100877/prospective-studies-of-the-incidence-of-pediatric-arterial-ischaemic-stroke
#4
LETTER
Andrew A Mallick, Finbar J O'Callaghan
No abstract text is available yet for this article.
October 25, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29079134/impact-of-chromosome-alterations-genetic-mutations-and-clonal-hematopoiesis-of-indeterminate-potential-chip-on-the-classification-and-risk-stratification-of-mds
#5
REVIEW
Bani Bandana Ganguly, Debasis Banerjee, Mohan B Agarwal
The advent of technological development has undoubtedly advanced biological and molecular inputs for better understanding the heterogeneous hematopoietic pre-malignant disorder of the stem cells known as myelodysplastic syndromes (MDS). Chromosomal rearrangements, including del(3q/5q/7q/11q/12p/20q), loss of 5/7/Y, trisomy 8/19, i(17q), etc. frequently detected in MDS with variable frequencies and combinations, are the integral components of the 5-tier risk-stratification and WHO-2016 classification. Observations on mutations in genes involved in RNA-splicing, DNA methylation, chromatin modification, transcription factor, signal transduction/kinases, RAS pathway, cohesin complex, DNA repair and other pathways have given insights in independent effects and biological interaction of co-occurrence on disease-phenotype and treatment outcome...
October 16, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#6
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29032941/ferritin-iron-regulators-pcbp1-and-ncoa4-respond-to-cellular-iron-status-in-developing-red-cells
#7
Moon-Suhn Ryu, Kari A Duck, Caroline C Philpott
Developing red blood cells exhibit multiple, redundant systems for regulating and coordinating the uptake of iron, the synthesis of heme, and the formation of hemoglobin during terminal differentiation. We recently described the roles of poly rC-binding protein (PCBP1) and nuclear coactivator 4 (NCOA4) in mediating the flux of iron through ferritin in developing erythroid cells, with PCBP1, an iron chaperone, delivering iron to ferritin and NCOA4, an autophagic cargo receptor, directing ferritin to the lysosome for degradation and iron release...
September 28, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29030091/clinical-characteristics-of-acute-promyelocytic-leukemiawith-the-stat5b-rara-fusion-gene
#8
LETTER
Congxiao Zhang, Ying Wang, Bingcheng Liu, Benfa Gong, Xiaoyuan Gong, Yuntao Liu, Yingchang Mi, Jianxiang Wang
No abstract text is available yet for this article.
September 27, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28964680/polarization-of-neutrophil-granules-a-characteristic-of-inflammatory-states
#9
LETTER
Kalyan C Mantripragada, Peter J Quesenberry
No abstract text is available yet for this article.
September 25, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29032940/molecular-basis-of-%C3%AE-thalassemia
#10
REVIEW
Samaneh Farashi, Cornelis L Harteveld
α-Thalassemia is an inherited, autosomal recessive, disorder characterized by a microcytic hypochromic anemia. It is one of the most common monogenic gene disorders in the world population. The clinical severity varies from almost asymptomatic, to mild microcytic hypochromic, and to a lethal hemolytic condition, called Hb Bart's Hydrops Foetalis Syndrome. The molecular basis are usually deletions and less frequently, point mutations affecting the expression of one or more of the duplicated α-genes. The clinical variation and increase in disease severity is directly related to the decreased expression of one, two, three or four copies of the α-globin genes...
September 21, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28954710/mechanisms-of-anti-cancer-effects-of-ascorbate-cytotoxic-activity-and-epigenetic-modulation
#11
REVIEW
Domenico Mastrangelo, Elvira Pelosi, Germana Castelli, Francesco Lo-Coco, Ugo Testa
Vitamin C (Vit C or Ascorbate) is essential for many fundamental biochemical processes. Vit C is an essential nutrient with redox functions at normal physiologic concentrations. The main physiologic function of this vitamin is related to its capacity to act as a co-factor for a large family of enzymes, collectively known as Fe and 2-oxoglutarate-dependent dioxygenases. It also modulates epigenetic gene expression through the control of TET enzymes activity. Vit C also has several biological properties allowing to restore the deregulated epigenetic response observed in many tumors...
September 21, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28951038/losartan-therapy-decreases-albuminuria-with-stable-glomerular-filtration-and-permselectivity-in-sickle-cell-anemia
#12
Marianne E Yee, Peter A Lane, David R Archer, Clinton H Joiner, James R Eckman, Antonio Guasch
Sickle cell nephropathy begins with hyperfiltration and microalbuminuria and may progress to renal failure. The aim of this study was to determine the effects of losartan on glomerular function and albumin excretion in sickle cell anemia (SCA). Individuals with SCA on hydroxyurea with persistent albuminuria were enrolled in a 1-year study of losartan. Glomerular filtration rate (GFR) measured by iohexol clearance, albumin excretion rate (AER), and fractional clearance of dextran were assessed at baseline, short-term (1-2month), and long-term (≥12month) intervals...
September 21, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28935503/should-eliglustat-be-first-line-therapy-for-patients-with-type-1-gaucher-disease-definitions-of-safety-and-efficacy
#13
Ari Zimran, Jack Goldblatt, Jeff Szer
No abstract text is available yet for this article.
September 14, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28918238/recent-advances-and-future-challenges-in-gaucher-disease
#14
EDITORIAL
Ari Zimran, Jeff Szer
No abstract text is available yet for this article.
September 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28918237/preface-to-the-special-issue-on-gaucher-disease-2017
#15
EDITORIAL
Ari Zimran, Jeff Szer
No abstract text is available yet for this article.
September 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28927633/the-use-of-mri-to-monitor-iron-overload-in-scd
#16
John C Wood
No abstract text is available yet for this article.
September 6, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28911832/association-of-silent-infarcts-in-sickle-cell-anemia-with-decreased-annexin-a5-resistance
#17
Kerry A Morrone, Lydia H Pecker, Jacob Rand, Jennifer Davila, Suzette Oyeku, Jane A Little, Xue Xiaonan, Deepa Manwani
BACKGROUND: Sickle cell anemia (SCA) is characterized by abnormally shaped, adhesive RBCs that interact with white blood cells and the endothelium, leading to chronic hemolysis, vasculopathy and a prothrombotic state. About 10% of subjects with a thrombotic event in the general population will have an associated antiphospholipid (aPL) antibody. One proposed mechanism for the thrombophilic nature of aPL antibodies is the disruption of the potent anticoagulant annexin A5 or Annexin A5 resistance (A5R)...
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28882478/corrigendum-to-the-expression-of-death-inducer-obliterator-dido-variants-in-myeloproliferative-neoplasms-blood-cells-mol-dis-59-2016-25-30
#18
Maria Gabriela Berzoti-Coelho, Aline Fernanda Ferreira, Natalia de Souza Nunes, Mariana Tomazini Pinto, Maurício Cristiano Rocha Júnior, Belinda Pinto Simões, Carlos Martínez-A, Elizabeth Xisto Souto, Rodrigo Alexandre Panepucci, Dimas Tadeu Covas, Simone Kashima, Fabíola Attié Castro
No abstract text is available yet for this article.
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28893518/curative-approaches-for-sickle-cell-disease-a-review-of-allogeneic-and-autologous-strategies
#19
Daniel E Bauer, Christian Brendel, Courtney D Fitzhugh
Despite sickle cell disease (SCD) first being reported >100years ago and molecularly characterized >50years ago, patients continue to experience severe morbidity and early mortality. Although there have been substantial clinical advances with immunizations, penicillin prophylaxis, hydroxyurea treatment, and transfusion therapy, the only cure that can be offered is hematopoietic stem cell transplantation (HSCT). In this work, we summarize the various allogeneic curative approaches reported to date and discuss open and upcoming clinical research protocols...
September 1, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29113683/technological-advances-in-sickle-cell-disease
#20
EDITORIAL
Russell E Ware
No abstract text is available yet for this article.
September 2017: Blood Cells, Molecules & Diseases
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