Flow cytometry as a tool in the diagnosis of Bernard-Soulier syndrome in Brazilian patients.

Abstract:

:Bernard-Soulier Syndrome (BSS) is an inherited recessive bleeding disorder. In some instances, diagnosis might be restricted to routine blood exams, including bleeding time, prothrombin time (PT), and partial thromboplastin time (APTT). Exams such as platelet aggregation, and testing for expression of ristocetin cofactor, or von Willebrand factor may not be commonly performed. This leads to misdiagnosis in a number of patients, which are subsequently treated erroneously. Flow cytometry has been used widely as a tool in the diagnosis of leukemias, lymphomas, and many other immuno-hematological diseases. The purpose of this study was to assess whether flow cytometry could be helpful in the diagnosis of Bernard-Soulier Syndrome in Brazilian patients. For this, we examined a selected group of 15 patients with suspected BSS based on classical diagnosis. We used a panel of antibodies to detect the expression of glycoproteins GPIbalpha, GPIIb, GPIIIa, GPIX, as well as CD9. Abnormalities of GPIb and GPIX were observed in nine of the 15 patients, which included severe reduction of both antigens, of one or the other, or normal levels but weak expression. Strikingly, this abnormality correlated with severely reduced expression of CD9 in all cases. We discuss the implications for flow cytometric diagnosis of BSS.

journal_name

Platelets

journal_title

Platelets

authors

Beltrame MP,Malvezzi M,Zanis J,Pasquini R

doi

10.1080/09537100902893784

subject

Has Abstract

pub_date

2009-06-01 00:00:00

pages

229-34

issue

4

eissn

0953-7104

issn

1369-1635

pii

911473832

journal_volume

20

pub_type

杂志文章
  • Interactions of platelets with Synthocytes, a novel platelet substitute.

    abstract::A platelet substitute, Synthocytes, is being developed for the prevention and treatment of thrombocytopenia. Synthocytes are composed of fibrinogen adsorbed on heat stabilised albumin microcapsules of defined size. The purpose of this study was to perform experiments in vitro to investigate the capacity of Synthocytes...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/0953371027000

    authors: Davies AR,Judge HM,May JA,Glenn JR,Heptinstall S

    更新日期:2002-06-01 00:00:00

  • Purinergic receptors on blood platelets.

    abstract::Extracellular adenine nucleotides interact with P2 purinergic receptors to regulate a broad range of physiological processes among which platelet aggregation P2 purinoceptors were divided into two main groups: the G-protein coupled receptor or 'metabotropic' superfamily termed P2Y and the ligand-gated ion channel or '...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537109609023587

    authors: Gachet C,Hechler B,Léon C,Vial C,Ohlmann P,Cazenave JP

    更新日期:1996-01-01 00:00:00

  • Platelet count and mortality of H7N9 infected patients in Guangdong, China.

    abstract::Avian influenza A (H7N9) is a serve zoonosis with a high mortality rate. Timely and effective diagnosis and early warning is crucial for the clinical treatment of H7N9 patients. The previous studies indicated that thrombocytopenia was associated with the prognosis of influenza cases, but the related evidence of platel...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2019.1665639

    authors: Chen Y,Yang Y,Cheng J,Lu J,Hu W

    更新日期:2020-01-01 00:00:00

  • Cobimetinib and trametinib inhibit platelet MEK but do not cause platelet dysfunction.

    abstract::The MEK inhibitors cobimetinib and trametinib are used in combination with BRAF inhibitors to treat metastatic melanoma but increase rates of hemorrhage relative to BRAF inhibitors alone. Platelets express several members of the MAPK signalling cascade including MEK1 and MEK2 and ERK1 and ERK2 but their role in platel...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2018.1514107

    authors: Unsworth AJ,Bye AP,Kriek N,Sage T,Osborne AA,Donaghy D,Gibbins JM

    更新日期:2019-01-01 00:00:00

  • The relationship of platelet count, mean platelet volume with metabolic syndrome according to the criteria of the American Association of Clinical Endocrinologists: a focus on gender differences.

    abstract::Metabolic syndrome (MS) is becoming globally prevalent and it is clinically important because of its association with type 2 diabetes and cardiovascular disease, and some cancers. Recently, platelet count has been linked to insulin resistance and MS in addition to being a marker of hemostasis. Also, mean platelet volu...

    journal_title:Platelets

    pub_type: 临床试验,杂志文章

    doi:10.3109/09537104.2011.589014

    authors: Park BJ,Shim JY,Lee HR,Jung DH,Lee JH,Lee YJ

    更新日期:2012-01-01 00:00:00

  • The placenta: a site of platelet production?

    abstract::Stripping of cytoplasm from circulating megakaryocytes (MKs) in the pulmonary capillary bed implicates the lungs as an important site for platelet production in normal physiology. The placenta has been proposed as an alternative location for intrauterine platelet production as fetal lungs are essentially non-functioni...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537109209013185

    authors: Woods MJ,Landon CR,Greaves M,Trowbridge EA

    更新日期:1992-01-01 00:00:00

  • Assessment of neonatal, cord, and adult platelet granule trafficking and secretion.

    abstract::Despite the transient hyporeactivity of neonatal platelets, full-term neonates do not display a bleeding tendency, suggesting potential compensatory mechanisms which allow for balanced and efficient neonatal hemostasis. This study aimed to utilize small-volume, whole blood platelet functional assays to assess the neon...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2019.1573314

    authors: Ngo ATP,Sheriff J,Rocheleau AD,Bucher M,Jones KR,Sepp AI,Malone LE,Zigomalas A,Maloyan A,Bahou WF,Bluestein D,McCarty OJT,Haley KM

    更新日期:2020-01-01 00:00:00

  • Thyroid and celiac diseases autoantibodies in patients with adult chronic idiopathic thrombocytopenic purpura.

    abstract::The association of chronic idiopathic thrombocytopenic purpura (cITP) and thyroid autoimmune diseases (TAD) is a known but an uncommon condition. Celiac disease (CD), which is characterized by malabsorption and villous atrophy that occur as a consequence of the ingestion of wheat gluten may also be related to other au...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537100801894651

    authors: Altintas A,Pasa S,Cil T,Bayan K,Gokalp D,Ayyildiz O

    更新日期:2008-06-01 00:00:00

  • High shear induces platelet dysfunction leading to enhanced thrombotic propensity and diminished hemostatic capacity.

    abstract::Thrombosis and bleeding are devastating adverse events in patients supported with blood-contacting medical devices (BCMDs). In this study, we delineated that high non-physiological shear stress (NPSS) caused platelet dysfunction that may contribute to both thrombosis and bleeding. Human blood was subjected to NPSS wit...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2017.1384542

    authors: Chen Z,Mondal NK,Zheng S,Koenig SC,Slaughter MS,Griffith BP,Wu ZJ

    更新日期:2019-01-01 00:00:00

  • Platelet and mean platelet volume kinetics in adult patients with sepsis.

    abstract::The aims of this study were to evaluate the kinetics of platelet counts and mean platelet volume (MPV) in adults with sepsis and to determine whether the responses are infection-specific. This retrospective cohort study included patients admitted to a tertiary-care teaching hospital with microbiologically proven nosoc...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2012.701027

    authors: Aydemir H,Piskin N,Akduman D,Kokturk F,Aktas E

    更新日期:2015-01-01 00:00:00

  • The efficacy and safety of CYP2C19 genotype-guided antiplatelet therapy compared with conventional antiplatelet therapy in patients with acute coronary syndrome or undergoing percutaneous coronary intervention: A meta-analysis of randomized controlled tri

    abstract::Cytochrome P450 (CYP) 2C19 genotype is closely associated with the metabolism and efficacy of clopidogrel, thereby having an important impact on clinical outcomes of patients with acute coronary syndrome (ACS) or undergoing percutaneous coronary intervention (PCI). This study aimed to evaluate the efficacy and safety ...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2020.1780205

    authors: Lyu SQ,Yang YM,Zhu J,Wang J,Wu S,Zhang H,Shao XH,Ren JM

    更新日期:2020-11-16 00:00:00

  • Influence of the alpha-adrenoreceptor naftopidil and doxazosin, on adrenaline-induced serotonin platelets: comparison with the antagonists, collagen and efflux by human effects of nifedipine.

    abstract::Collagen (5 microg/ml) stimulation of washed platelets increased endogenous serotonin (5-HT) release to the medium from 13.88 1.39 to 188.67 26.37 pmol/108 platelets ( P < 0.001). Adrenaline (16 microM) also increased 5-HT release, from 11.0 1.46 to 110.6 29.9 pmol/108 platelets ( P < 0.02). Naftopidil enhanced collag...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537109777519

    authors: Alarayyed NA,Prichard BN,Betteridge DJ,Smith CC

    更新日期:1997-01-01 00:00:00

  • A comparative study of antioxidative activity of calcium-D-glucarate, sodium-D-gluconate and D-glucono-1,4-lactone in a human blood platelet model.

    abstract::D-glucono-1,4-lactone, sodium D-gluconate and calcium D-glucarate are non-toxic glucose derivatives occurring naturally in fruits and vegetables. Calcium D-glucarate is promoted as an orally bioavailability dietary supplement with potential chemopreventive activity without adverse effects. Despite many commercial appl...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2010.512210

    authors: Saluk-Juszczak J

    更新日期:2010-01-01 00:00:00

  • Interactive protein network of FXIII-A1 in lipid rafts of activated and non-activated platelets.

    abstract::Lipid-rafts are defined as membrane microdomains enriched in cholesterol and glycosphingolipids within platelet plasma membrane. Lipid raft-mediated clot retraction requires factor XIII and other interacting proteins. The aim of this study was to investigate the proteins that interact with factor XIII in raft and non-...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2016.1153621

    authors: Rabani V,Montange D,Davani S

    更新日期:2016-09-01 00:00:00

  • Early platelet recovery following cardiac surgery with cardiopulmonary bypass.

    abstract::Coronary artery bypass grafting (CABG) with cardiopulmonary bypass (CPB) is frequently associated with low platelet count (PC) and disturbed platelet function (PF). While PC is easy to measure, PF is more difficult to assess. Moreover, the time-related platelet dysfunction and recovery after CPB is not fully elucidate...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2016.1173665

    authors: Van Poucke S,Stevens K,Wetzels R,Kicken C,Verhezen P,Theunissen M,Kuiper G,van Oerle R,Henskens Y,Lancé M

    更新日期:2016-12-01 00:00:00

  • Antimicrobial properties of single-donor-derived, platelet-leukocyte fibrin for fistula occlusion: An in vitro study.

    abstract::Fibrin glue is a promising alternative for low-output enterocutaneous fistula closure. Bacterial flora colonizing inside the fistula tract, however, may limit the glue application. Single-donor-derived, platelet-rich materials were hypothesized in this study to have antimicrobial activity against Gram-negative microor...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2012.761685

    authors: Wu X,Ren J,Yuan Y,Luan J,Yao G,Li J

    更新日期:2013-01-01 00:00:00

  • Inhibitory effect of glyburide on thrombin-induced platelet aggregation and phosphoinositide metabolism in normal human platelets.

    abstract::We previously reported the effects of diet, sulphonylureas or insulin on thrombin-induced platelet aggregation, phosphoinositide metabolism and protein phosphorylation in non-insulin-dependent diabetes mellitus (NIDDM) patients. To clarify the mechanism of glyburide and insulin on platelet function, here we studied th...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537109976347

    authors: Wada H,Ishizuka T,Itaya S,Yamada K,Kajita K,Kimura M,Miura A,Shibata T,Kanoh Y,Ishizawa M,Yasuda K

    更新日期:1999-01-01 00:00:00

  • Vigorous exercise as a triggering mechanism for late stent thrombosis: A description of three cases.

    abstract::Although (very) late coronary stent thrombosis is a rare complication after percutaneous coronary interventions (PCI), its consequences are devastating with a high morbidity and mortality. Previous studies have identified several clinical, procedural and angiographic characteristics that are associated with stent thro...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537100903388367

    authors: Zwart B,Van Kerkvoorde TC,van Werkum JW,Breet NJ,Ten Berg JM,Van 't Hof AW

    更新日期:2010-01-01 00:00:00

  • Safety and efficacy of IIb/IIIa inhibitors in combination with highly active oral antiplatelet regimens in acute coronary syndromes: A meta-analysis of pivotal trials.

    abstract::The risk and benefit of GP-IIb/IIIa Inhibition (GPI) in combination with recent antiplatelet regimens in acute coronary syndromes (ACS) remain unassessed. The advent of fast-acting highly active oral P2Y12 inhibitors questions the additional value and risk of their association with GPI. We studied the effect of GPI in...

    journal_title:Platelets

    pub_type: 杂志文章,meta分析,评审

    doi:10.1080/09537104.2016.1218453

    authors: Roule V,Agueznai M,Sabatier R,Blanchart K,Lemaître A,Ardouin P,Collet JP,Milliez P,Montalescot G,Beygui F

    更新日期:2017-03-01 00:00:00

  • Effects of autologous fresh platelet concentrate on haemostasis in cardiac reoperations.

    abstract::The aim of this study was to determine whether autologous fresh platelet concentrate (PC) significantly improves haemostasis in cardiac reoperations compared with autologous fresh whole blood (WB). Forty-eight patients who had elective cardiac reoperations with a low-dose aprotinin priming regimen were divided into tw...

    journal_title:Platelets

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1080/09537100120058801

    authors: Miyashita T,Kuro M

    更新日期:2001-06-01 00:00:00

  • Role of protein kinases in mediating [(45)ca(2+)] uptake by human platelets.

    abstract::It has recently been demonstrated that both initiators and inhibitors (viz iloprost) of aggregation stimulate the uptake of [(45)Ca(2+)] by human platelets. Since it was postulated that this calcium uptake reflects changes associated with signal transduction, we investigated the role of cAMP-dependent protein kinases ...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537109309013234

    authors: Gill J,Jeremy JY,Mikhailidis DP

    更新日期:1993-01-01 00:00:00

  • Efficacy and safety of different ticagrelor regimens versus clopidogrel in patients with coronary artery disease: a retrospective multicenter study (SUPERIOR).

    abstract::Current guidelines favor dual anti-platelet therapy with ticagrelor 90 mg BID (T90BID) over clopidogrel 75 mg QD (C75QD) in addition to aspirin for acute coronary syndrome. However, an increased risk of ticagrelor-related adverse events prompted the evaluation of low-dose regimens. This study (NCT03381742) retrospecti...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537104.2020.1732328

    authors: Shi J,He M,Wang W,Liu G,Zhang H,Sun D,Li J,Mu H,Xu M,Zhao C,Wang J,Zhang C,Cang H,Zhao S,Zhang Z,Li Y

    更新日期:2021-01-02 00:00:00

  • Thrombelastographic hypercoagulability and antiplatelet therapy after coronary artery bypass surgery (TEG-CABG trial): a randomized controlled trial.

    abstract::A hypercoagulable state has, in observational studies, been associated with increased risk of thromboembolic events. The aim of this trial was to study whether dual antiplatelet therapy (DAPT) with clopidogrel in addition to aspirin could reduce the rate of graft occlusions, thromboembolic events, and death compared t...

    journal_title:Platelets

    pub_type: 杂志文章,随机对照试验

    doi:10.1080/09537104.2017.1280147

    authors: Rafiq S,Johansson PI,Kofoed KF,Lund JT,Olsen PS,Bentsen S,Steinbrüchel DA

    更新日期:2017-12-01 00:00:00

  • Lung damage may induce thrombocytopenia.

    abstract::Thrombocytopenia is common in patients with lung diseases. Lung injury is associated with deranged platelet homeostasis. The potential mechanisms underlying this association were investigated in this study. The number and morphology of circulating megakaryocytes (MK) were investigated in vivo during cardiopulmonary by...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537100600745120

    authors: Xiao da W,Yang M,Yang J,Hon KL,Fok FT

    更新日期:2006-08-01 00:00:00

  • Update on functional and genetic laboratory assays for the detection of platelet microvesicles.

    abstract::Functional and genetic assays for measuring platelet microvesicles (PMVs) are presented and discussed. Functional assays concern two groups of methods: a) homogeneous assays using the cofactor activity of phospholipids (PPLs) contained in PMVs and present in assayed plasmas, and a coagulation or a thrombin generation ...

    journal_title:Platelets

    pub_type: 杂志文章,评审

    doi:10.1080/09537104.2016.1265925

    authors: Laroche M,Dunois C,Vissac AM,Amiral J

    更新日期:2017-05-01 00:00:00

  • Platelet activation in subjects with impaired glucose tolerance.

    abstract::Impaired glucose tolerance (IGT), a prediabetic state, is associated with an increased risk of cardiovascular disease. Mean platelet volume (MPV), a determinant of platelet activation, is a newly emerging risk factor for atherothrombosis. This study was designed to answer the following questions: (i) Do MPV levels cha...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.1080/09537100701609019

    authors: Coban E,Kucuktag S,Basyigit S

    更新日期:2007-12-01 00:00:00

  • Regulatory Effect of Cell Density on the Platelet-aggregating Activity of Human Melanoma Cell Line.

    abstract::Human melanoma cell Line, HMV-I, has been shown to induce platelet aggregation without thrombin generation. The platelet-aggregating activity of HMV-I cells increased with increasing culture periods of the cells. However, data indicate that the platelet-aggregating activity of HMV-I cells is dependent upon the cell de...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537109509078456

    authors: Katagiri Y,Hayashi Y,Baba I,Yamazaki H,Tanoue K

    更新日期:1995-01-01 00:00:00

  • The role of platelet microvesicles in intercellular communication.

    abstract::In recent years, there has been exponential growth in the interest in microvesicles, which is reflected by the number of publications. Initially referred to as "platelet dust" by Peter Wolf in 1967, platelet microvesicles (PMV) are now recognized as important mediators of intercellular communication. There are example...

    journal_title:Platelets

    pub_type: 杂志文章,评审

    doi:10.1080/09537104.2016.1257114

    authors: Edelstein LC

    更新日期:2017-05-01 00:00:00

  • New insights into thrombopoiesis in neonatal sepsis.

    abstract::Thrombocytopenia is one of the most frequent hematologic abnormalities in the neonatal period, affecting about 18-35% of all patients admitted to the Neonatal Intensive Care Unit (NICU), with sepsis being among the most common causes of severe neonatal thrombocytopenia. It is unclear whether decreased platelet product...

    journal_title:Platelets

    pub_type: 杂志文章

    doi:10.3109/09537104.2012.696748

    authors: Eissa DS,El-Farrash RA

    更新日期:2013-01-01 00:00:00

  • Aspirin intake in the morning is associated with suboptimal platelet inhibition, as measured by serum Thromboxane B2, during infarct-prone early-morning hours.

    abstract::Aspirin is traditionally taken once daily in the morning and considered to be effective throughout the 24h interval. Cardiovascular events occur most frequently in the early morning, suggesting that these hours are critical in terms of adequate platelet inhibition. This study therefore assed platelet function in the e...

    journal_title:Platelets

    pub_type: 杂志文章,随机对照试验

    doi:10.1080/09537104.2018.1528347

    authors: Racca C,van Diemen JJK,Fuijkschot WW,Spit K,Bonten TN,Numans ME,van der Bom JG,Smulders YM,Thijs A

    更新日期:2019-01-01 00:00:00