Abstract:
:Significant advances have been made in our understanding of the structural basis for altered cell function in various inherited red cell membrane disorders with reduced red cell survival and resulting hemolytic anemia. The current review summarizes these advances as they relate to defining the molecular and structural basis for disorders involving altered membrane structural organization (hereditary spherocytosis [HS] and hereditary elliptocytosis [HE]) and altered membrane transport function (hereditary overhydrated stomatocytosis and hereditary xerocytosis). Mutations in genes encoding membrane proteins that account for these distinct red cell phenotypes have been identified. These molecular insights have led to improved understanding of the structural basis for altered membrane function in these disorders. Weakening of vertical linkage between the lipid bilayer and spectrin-based membrane skeleton leads to membrane loss in HS. In contrast, weakening of lateral linkages among different skeletal proteins leads to membrane fragmentation and decreased surface area in HE. The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in these two disorders. Splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span of spherocytic red cells that are normally sequestered by the spleen. Disordered membrane cation permeability and resultant increase or decrease in red cell volume account for altered cellular deformability of hereditary overhydrated stomatocytosis and hereditary xerocytosis, respectively. Importantly, splenectomy is not beneficial in these two membrane transport disorders and in fact contraindicated due to severe postsplenectomy thrombotic complications.
journal_name
Int J Lab Hematoljournal_title
International journal of laboratory hematologyauthors
Narla J,Mohandas Ndoi
10.1111/ijlh.12657subject
Has Abstractpub_date
2017-05-01 00:00:00pages
47-52eissn
1751-5521issn
1751-553Xjournal_volume
39 Suppl 1pub_type
杂志文章,评审abstract:INTRODUCTION:Presence of schistocytes in peripheral blood smear supporting haemolysis is important for diagnosis and decision-making in paediatric haemolytic uraemic syndrome (HUS). High observer dependency and requirement of expertise for peripheral smear evaluation propels us to think of other modalities to overcome ...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13324
更新日期:2021-02-01 00:00:00
abstract:INTRODUCTION:Physiological changes during pregnancy affect routine tests for iron deficiency. The reticulocyte haemoglobin equivalent (RET-He) and serum-soluble transferrin receptor (sTfR) assay are newer diagnostic parameters for the detection of iron deficiency, combined in the Thomas diagnostic plot. We used this pl...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12448
更新日期:2016-04-01 00:00:00
abstract:INTRODUCTION:With the progression of blood analysis technology, hematology analyzers become more complex and diverse. How to choose a superb instrument is a challenge for the laboratories. In the essay, we studied whether the newest BC-6000 hematology analyzer meets the needs of a clinical hematology laboratory. METHO...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
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更新日期:2019-10-01 00:00:00
abstract:INTRODUCTION:We developed and validated reflex testing rules for the microscopic examination (ME) of body fluids (BFs) on the Sysmex XN-550 (Sysmex Corporation) instrument. METHODS:We assessed the detection limits, precision, linearity, and carryover. To develop the reflex testing rules (derivation arm), we tested 515...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12774
更新日期:2018-06-01 00:00:00
abstract::Multiple myeloma (MM) is a malignant plasma cell disorder that involves multiple genetic abnormalities. Chimeric transcription factors, created by gene fusion as a result of chromosomal translocations, have been implicated in the pathogenesis of the disease. Here, we report the conventional cytogenetic analysis of a M...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2006.00837.x
更新日期:2007-06-01 00:00:00
abstract::Acute myeloid leukemia (AML) is a complex disease, for which our understanding of the role of genetic and epigenetic changes has undergone significant advancements. Newer diagnostic and prognostic classifications have increasingly incorporated such information, and novel therapies have been developed to target specifi...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12367
更新日期:2015-05-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/j.1751-553X.2010.01275.x
更新日期:2011-04-01 00:00:00
abstract:INTRODUCTION:The STA R Max® 2 is a new coagulation analyser developed by Diagnostica Stago, able to perform clotting, chromogenic and immuno-turbidimetric tests. A pre-analytical module build into the cap-piercing needle performs the sample integrity verification (sample tube filling and measurement of haemolysis, icte...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13100
更新日期:2019-12-01 00:00:00
abstract:INTRODUCTION:Laboratory investigations for bleeding disorders are warranted when an individual has a personal and/or family history of bleeding, and/or laboratory findings that suggest the possibility of an inherited or acquired bleeding disorder. METHODS:This review summarizes author's experience with ordering and re...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12822
更新日期:2018-05-01 00:00:00
abstract:BACKGROUND:Iron is essential for hemoglobin synthesis during terminal erythropoiesis. To supply adequate iron the carrier transferrin is required together with transferrin receptor endosomal cycle and normal mitochondrial iron utilization. Iron and iron protein deficiencies result in different types of anemia. Iron-def...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12505
更新日期:2016-05-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2011.01384.x
更新日期:2012-06-01 00:00:00
abstract::The consistent multi-ethnic migrations of the last decades have considerably changed the epidemiology of the hemoglobinopathies. Healthy carriers of these conditions are present today in many nonendemic parts of the world, and severely affected children are now born where these diseases were previously rare or unknown...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12037
更新日期:2013-10-01 00:00:00
abstract:INTRODUCTION:Monitoring of factor IX (FIX) replacement therapy in haemophilia B relies on accurate coagulation assays. However, considerable interlaboratory variability has been reported for one-stage clotting (OSC) assays. This study aimed to evaluate the real-world, interlaboratory variability of routine FIX activity...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,多中心研究
doi:10.1111/ijlh.13189
更新日期:2020-06-01 00:00:00
abstract:INTRODUCTION:Participation in external quality assessment (EQA) is central to the maintenance of high-quality laboratory results in patient diagnosis and clinical trials. Laboratories in the TAF112582 DETECTIVE study (ClinicalTrials.gov identifier: NCT01376167) are enrolled in the United Kingdom National Quality Assess...
journal_title:International journal of laboratory hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/ijlh.12627
更新日期:2017-06-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2007.01011.x
更新日期:2009-04-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2009.01219.x
更新日期:2010-10-01 00:00:00
abstract:INTRODUCTION:Clot waveform analysis (CWA), a new methodology to assess coagulation process, can be usefully applied in various clinical settings. However, its clinical use is limited mainly because of the absence of standardization. No consensus exists regarding the wavelengths at which CWA has to be performed what is ...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12975
更新日期:2019-06-01 00:00:00
abstract:INTRODUCTION:In the cytogenetically normal population of AML (CN-AML), FLT3-ITD-positive and wild-type NPM1 is correlated with a worse outcome, and FLT3-ITD-negative with NPM1-mut is correlated with a better outcome. This leaves a large subpopulation of CN-AML patients without NPM1 or FLT3-ITD mutations with heterogene...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12649
更新日期:2017-08-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13099
更新日期:2019-10-01 00:00:00
abstract::The majority, if not all, of human cell types secrete extracellular vesicles (EVs) into their environment, at least partly as a means of intercellular communication. These secreted vesicles can be detected in most bodily fluids including blood, urine, and saliva. The number of secreted vesicles and their composition i...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12247
更新日期:2014-06-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13159
更新日期:2020-04-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2010.01287.x
更新日期:2011-06-01 00:00:00
abstract::Advances in lab-on-a-chip technologies enabled programmable, reconfigurable, and scalable manipulation of a variety of laboratory procedures. Samples, reagents, and fluids can be precisely controlled; buffer temperature, pH, and concentration control systems as well as a variety of detection systems can be integrated ...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.12054
更新日期:2013-10-01 00:00:00
abstract::Before a new method is used for clinical testing, it is essential that it is evaluated for suitability for its intended purpose. This document gives guidance for the performance of verification, validation and implementation processes required by regulatory and accreditation bodies. It covers the planning and executio...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章,评审
doi:10.1111/ijlh.13411
更新日期:2020-11-29 00:00:00
abstract:INTRODUCTION:Women are at higher risk of venous thromboembolism (VTE) during pregnancy and the puerperium. Global coagulation assays (GCAs), including thromboelastography (TEG), thrombin generation using the calibrated automated thrombogram (CAT) and fibrin generation using the overall haemostatic potential assay (OHP)...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13389
更新日期:2020-11-10 00:00:00
abstract:INTRODUCTION:Direct oral anticoagulants (DOAC) are commonly prescribed and measuring drug levels may be useful in a number of contexts. However, data on DOAC level measurement and their clinical utility in real-world studies are limited. METHODS:We carried out a 2-year retrospective cohort study of DOAC levels measure...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12944
更新日期:2019-04-01 00:00:00
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journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/j.1751-553X.2008.01111.x
更新日期:2010-02-01 00:00:00
abstract:INTRODUCTION:FVIII inhibitors consist of a polyclonal population of antibodies. Previous studies have demonstrated different distribution of IgG subclasses. IgG4 was associated to high level of FVIII inhibitors and failure of immune tolerance induction (ITI) treatment. This study monitored the relative distribution of ...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12696
更新日期:2017-12-01 00:00:00
abstract:INTRODUCTION:In daily practice in haematology laboratories, red blood cell (RBC) abnormalities are frequent and their management is a real challenge. The aim of this study is to establish a "decision tree" using RBC and reticulocyte parameters from the SYSMEX XN-10 analyser to distinguish between patients with a heredi...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.13278
更新日期:2020-12-01 00:00:00
abstract:INTRODUCTION:Peripheral blood smear (PBS) review is a routine laboratory test which requires pathologist's interpretation when abnormal indices, atypical cells, or critical findings are identified. Real-time remote digital microscopy (DM) can potentially facilitate rapid review when an on-site pathologist is not availa...
journal_title:International journal of laboratory hematology
pub_type: 杂志文章
doi:10.1111/ijlh.12914
更新日期:2018-12-01 00:00:00