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1.
Eur Rev Med Pharmacol Sci ; 24(17): 9161-9168, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32965009

RESUMO

OBJECTIVE: Coronavirus Disease-2019 (COVID-19) predisposes patients to thrombosis which underlying mechanisms are still incompletely understood. We sought to investigate the balance between procoagulant factors and natural coagulation inhibitors in the critically ill COVID-19 patient and to evaluate the usefulness of hemostasis parameters to identify patients at risk of venous thromboembolic event (VTE). PATIENTS AND METHODS: We conducted an observational study recording VTEs defined as deep vein thrombosis or pulmonary embolism using lower limb ultrasound (92% of the patients), computed tomography pulmonary angiography (6%) and both tests (2%). We developed a comprehensive analysis of hemostasis. RESULTS: Ninety-two consecutive mechanically ventilated COVID-19 patients (age, 62 years [53-69] (median [25th-75th percentiles]); M/F sex ratio, 2.5; body-mass index, 28 kg/m2 [25-32]; past hypertension (52%) and diabetes mellitus (30%)) admitted to the Intensive Care Unit (ICU) from 03/11/2020 to 5/05/2020, were included. When tested, patients were receiving prophylactic (74%) or therapeutic (26%) anticoagulation. Forty patients (43%) were diagnosed with VTE. Patients displayed inflammatory and prothrombotic profile including markedly elevated plasma fibrinogen (7.7 g/L [6.1-8.6]), D-dimer (3,360 ng/mL [1668-7575]), factor V (166 IU/dL [136-195]) and factor VIII activities (294 IU/dL [223-362]). We evidenced significant discrepant protein C anticoagulant and chromogenic activities, combined with slightly decreased protein S activity. Plasma D-dimer >3,300 ng/mL predicted VTE presence with 78% (95%-confidence interval (95% CI), 62-89) sensitivity, 69% (95% CI, 55-81) specificity, 66% (95% CI, 51-79) positive predictive value and 80% (95% CI, 65-90) negative predictive value [area under the ROC curve, 0.779 (95%CI, 0.681-0.859), p=0.0001]. CONCLUSIONS: Mechanically ventilated COVID-19 patients present with an imbalance between markedly increased factor V/VIII activity and overwhelmed protein C/S pathway. Plasma D-dimer may be a useful biomarker at the bedside for suspicion of VTE.


Assuntos
Inibidores dos Fatores de Coagulação Sanguínea/metabolismo , Fatores de Coagulação Sanguínea/metabolismo , Infecções por Coronavirus/diagnóstico , Pneumonia Viral/diagnóstico , Idoso , Área Sob a Curva , Betacoronavirus/isolamento & purificação , Índice de Massa Corporal , Infecções por Coronavirus/complicações , Infecções por Coronavirus/virologia , Estado Terminal , Fator V/análise , Fator VIII/análise , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Fibrinogênio/análise , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/virologia , Proteína C/análise , Proteína S/análise , Curva ROC , Tromboembolia Venosa/complicações , Tromboembolia Venosa/diagnóstico
2.
Clin Appl Thromb Hemost ; 26: 1076029620943671, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32702995

RESUMO

Severe acute respiratory syndrome coronavirus 2 infection (COVID-19) is known to induce severe inflammation and activation of the coagulation system, resulting in a prothrombotic state. Although inflammatory conditions and organ-specific diseases have been shown to be strong determinants of morbidity and mortality in patients with COVID-19, it is unclear whether preexisting differences in coagulation impact the severity of COVID-19. African Americans have higher rates of COVID-19 infection and disease-related morbidity and mortality. Moreover, African Americans are known to be at a higher risk for thrombotic events due to both biological and socioeconomic factors. In this review, we explore whether differences in baseline coagulation status and medical management of coagulation play an important role in COVID-19 disease severity and contribute to racial disparity trends within COVID-19.


Assuntos
Afro-Americanos , Betacoronavirus , Infecções por Coronavirus/etnologia , Pandemias , Pneumonia Viral/etnologia , Trombofilia/etnologia , Tromboembolia Venosa/etnologia , Afro-Americanos/genética , Anemia Falciforme/sangue , Anemia Falciforme/etnologia , Anticoagulantes/uso terapêutico , Proteínas Sanguíneas/análise , Proteínas Sanguíneas/genética , Ensaios Clínicos como Assunto , Comorbidade , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Fator VIII/análise , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Estudos de Associação Genética , Predisposição Genética para Doença , Disparidades em Assistência à Saúde , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Seleção de Pacientes , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Polimorfismo de Nucleotídeo Único , Prevalência , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/etnologia , Fatores de Risco , Determinantes Sociais da Saúde , Fatores Socioeconômicos , Trombofilia/sangue , Trombofilia/tratamento farmacológico , Trombofilia/etiologia , Tromboembolia Venosa/sangue , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/prevenção & controle
4.
Sci Rep ; 10(1): 7871, 2020 05 12.
Artigo em Inglês | MEDLINE | ID: mdl-32398812

RESUMO

Circulating microparticles (MPs) are procoagulant due to the surface containing phosphatidylserine (PS), which facilitates coagulation. We investigated if MPs improve hemostasis in HA plasma models. MPs isolated from pooled normal human plasma were added to severe, moderate and mild HA plasma models (0%, 2.5%, 20% FVIII). The MPs' effect on hemostasis was evaluated by calibrated automated thrombogram (CAT) and overall hemostasis potential (OHP) assays, while fibrin structure was imaged by standard confocal, stimulated emission depletion (STED) microscopy and scanning electron microscopy (SEM). MPs partially restored thrombin generation and fibrin formation in all HA plasma models. The procoagulant effect of MPs requires PS exposure, to a less extent of contact pathway activation, but not tissue factor exposure or in vitro stimulation of MPs. MPs partially normalized the fibrin structure, and using super-resolution STED, MPs attached to fibrin were clearly resolved. In summary, our results demonstrate that PS positive MPs could improve hemostasis in HA plasma models.


Assuntos
Micropartículas Derivadas de Células/metabolismo , Fator VIII/análise , Hemofilia A/sangue , Hemostasia , Fosfatidilserinas/metabolismo , Coagulação Sanguínea , Micropartículas Derivadas de Células/ultraestrutura , Fibrina/metabolismo , Humanos , Microscopia Confocal , Microscopia Eletrônica de Varredura , Trombina/metabolismo , Tromboplastina/metabolismo
5.
Am J Clin Pathol ; 154(1): 78-87, 2020 06 08.
Artigo em Inglês | MEDLINE | ID: mdl-32232366

RESUMO

OBJECTIVES: The term discrepant hemophilia A (DHA) denotes the discrepancy between factor VIII activity (FVIII:C) measured by different assay methodologies in patients with nonsevere hemophilia A (HA). The objective was to review the characteristics and the current understanding of mechanisms contributing to assay discrepancy in DHA. METHODS: Characteristics of the DHA patients treated were examined by retrospective chart review. In addition, a literature review was performed to determine the current understanding of DHA. RESULTS: Three cases of DHA were diagnosed based on bleeding phenotype: 2 cases represented missed diagnoses of HA, and 1 represented misclassification of hemophilia severity. The revised diagnosis and classification of hemophilia directly affected clinical management. Review of the literature identified 18 articles with an estimated pooled prevalence of 36% (95% CI, 23%-56%; I2 = 85%; P < .01) among nonsevere HA. Furthermore, literature indicated that DHA is a feature of how different FVIII gene mutations affect FVIII:C activity within different assay methodologies. CONCLUSIONS: Our experience and literature review suggested that DHA is not only a laboratory phenomenon-it can affect clinical management in a subset of patients. A high index of suspicion for DHA is necessary while evaluating bleeding patients and/or classifying nonsevere HA.


Assuntos
Testes de Coagulação Sanguínea/métodos , Fator VIII/análise , Hemofilia A/sangue , Hemofilia A/diagnóstico , Adolescente , Adulto , Hemofilia A/classificação , Humanos , Masculino
6.
Int J Lab Hematol ; 42 Suppl 1: 19-20, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32311843
8.
Sci Rep ; 10(1): 2005, 2020 02 06.
Artigo em Inglês | MEDLINE | ID: mdl-32029851

RESUMO

Human endothelial cells (ECs) synthesize, store, and secrete von Willebrand factor multimeric strings and coagulation factor (F) VIII. It is not currently known if ECs produce other coagulation factors for active participation in coagulation. We found that 3 different types of human ECs in primary culture produce clotting factors necessary for FX activation via the intrinsic (FVIII-FIX) and extrinsic (tissue factor [TF]-FVII) coagulation pathways, as well as prothrombin. Human dermal fibroblasts were used as comparator cells. TF, FVII, FIX, FX, and prothrombin were detected in ECs, and TF, FVII, FIX, and FX were detected in fibroblasts. In addition, FVII, FIX, FX, and prothrombin were detected by fluorescent microscopy in EC cytoplasm (associated with endoplasmic reticulum and Golgi proteins). FX activation occurred on human umbilical vein EC surfaces without the addition of external coagulation proteins, proteolytic enzymes, or phospholipids. Tumour necrosis factor, which suppresses the generation of activated protein C and increases TF, augmented FX activation. Fibroblasts also produced TF, but (in contrast to ECs) were incapable of activating FX without the exogenous addition of FX and had a marked increase in FX activation following the addition of both FX and FVII. We conclude that human ECs produce their own coagulation factors that can activate cell surface FX without the addition of exogenous proteins or phospholipids.


Assuntos
Coagulação Sanguínea , Fator X/metabolismo , Fibroblastos/metabolismo , Células Endoteliais da Veia Umbilical Humana/metabolismo , Linhagem Celular , Citoplasma/metabolismo , Fator IX/análise , Fator IX/metabolismo , Fator VII/análise , Fator VII/metabolismo , Fator VIII/análise , Fator VIII/metabolismo , Fibroblastos/citologia , Células Endoteliais da Veia Umbilical Humana/citologia , Humanos , Microscopia de Fluorescência , Cultura Primária de Células , Protrombina/análise , Protrombina/metabolismo , Pele/citologia , Tromboplastina/análise , Tromboplastina/metabolismo
9.
Clin Chem ; 66(2): 373-378, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32040575

RESUMO

BACKGROUND: An inversion of intron 22 in the Factor VIII gene (Inv22) is the causative mutation for 45% of severe hemophilia A cases. Available methods for molecular diagnosis of Inv22 are generally tedious and not ideal for routine clinical use. METHODS: We report here a new method using a single closed-tube nested quantitative PCR (CN-qPCR) for rapid detection of Inv22. This method combines a 12-cycle long-distance PCR (LD-PCR) amplifying the int22h regions, followed by a duplex qPCR targeting two specific regions close to the int22h regions. All reagents were added to a single PCR mixture for the closed-tube assay. Sequential LD-PCR and qPCR was achieved by designing primers at substantially different melting temperatures and optimizing PCR conditions. RESULTS: Seventy-nine male hemophilia A patients of different disease severity were tested by both the CN-qPCR assay and the standard LD-PCR assay. CN-qPCR successfully made calls for all samples, whereas LD-PCR failed in eight samples. For the 71 samples where both methods made calls, the concordance was 100%. Inv22 was detected in 17 out of the 79 samples. Additionally, CN-qPCR achieved clear separation for 10 female carriers and 10 non-Inv22 females, suggesting the assay may also be useful for molecular diagnosis of female carriers. CONCLUSIONS: This new CN-qPCR method may provide a convenient and accurate F8 Inv22 test suitable for clinical use.


Assuntos
Fator VIII/genética , Hemofilia A/diagnóstico , Reação em Cadeia da Polimerase em Tempo Real/métodos , Inversão Cromossômica/genética , Fator VIII/análise , Fator VIII/metabolismo , Feminino , Genótipo , Hemofilia A/genética , Humanos , Íntrons/genética , Masculino , Inversão de Sequência/genética
10.
Ann Biol Clin (Paris) ; 78(1): 35-46, 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-32108578

RESUMO

Only a few studies on real-world clinical use of recombinant factor VIII -fusionned with Fc (rFVIIIFc, efmoroctocog alpha) have been performed to date, with data on the annual bleeding rate (ABR), the prophylaxis regimen, and FVIII consumption. The aim of our study was to report the real-world clinical application of rFVIIIFc with additional elements, both biological and clinical. A prospective monocentric study has been conducted in the Haemophilia treatment center (HTC) of the Strasbourg university hospital among the severe haemophilia A patients. Thirty male patients were enrolled in the study. After injection of rFVIIIFc, the average time spent above 5%, 2% and 1% of FVIII was respectively almost 3, 4 and 5 days. The average half-life was 15.8 hours. A strong linear correlation between incremental recovery of rFVIIIFc and weight and between rFVIIIFc half-life and basal VWF:Ag level was observed. FVIII activity measurement for rFVIIIFc showed similar results than those previously published. In the follow-up, residual FVIII activity was on average the one of a mild haemophilia patient, corroborated by the results of endogenous thrombin potential of the thrombin generation assay. In clinical practice, rFVIIIFc was well tolerated and patients were mostly satisfied or indifferent of the switch. A single failure was however noticed. No FVIII inhibitor has been detected. Decrease in FVIII consumption was observed, with reduced or unchanged ABR. The switch was an actual success for almost all of the 30 patients, corroborated by satisfactory clinical and biological results.


Assuntos
Substituição de Medicamentos , Fator VIII/uso terapêutico , Hemofilia A/tratamento farmacológico , Fragmentos Fc das Imunoglobulinas/uso terapêutico , Proteínas Recombinantes de Fusão/uso terapêutico , Adolescente , Adulto , Idoso , Testes de Coagulação Sanguínea , Fator VIII/análise , Fator VIII/farmacocinética , Meia-Vida , Hemofilia A/sangue , Hemofilia A/metabolismo , Hemofilia A/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Satisfação do Paciente , Proteínas Recombinantes de Fusão/farmacocinética , Índice de Gravidade de Doença , Trombina/metabolismo , Adulto Jovem
11.
Haemophilia ; 26(1): 151-155, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31859415

RESUMO

INTRODUCTION: The factor VIII mimetic emicizumab (Hemlibra, Hoffman-la Roche, Basel, Switzerland) has a novel mode of action that affects the laboratory monitoring of patients receiving this treatment. AIM: This guideline from the United Kingdom Haemophilia Centre Doctors Organisation (UKHCDO) aims to provide advice for clinical and laboratory staff on appropriate use of laboratory assays in patients with Haemophilia A treated with emicizumab. METHODOLOGY: The guideline was prepared by a review of the available literature and discussion and revision by the authors. RESULTS: The guideline describes the effect of emicizumab on commonly used coagulations tests and provides recommendations on the use of assays for measurement of factor VIII and factor VIII inhibitor in the presence of emicizumab. The guideline also provides recommendations on measurement of emicizumab. CONCLUSION: Knowledge of the effect of emicizumab on coagulation tests and factor assays is required to ensure appropriate testing and monitoring of therapy in patients receiving this drug.


Assuntos
Anticorpos Biespecíficos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Testes de Coagulação Sanguínea , Hemofilia A/tratamento farmacológico , Guias de Prática Clínica como Assunto , Anticorpos/análise , Anticorpos Biespecíficos/análise , Anticorpos Monoclonais Humanizados/análise , Fator VIII/análise , Fator VIII/antagonistas & inibidores , Fator VIII/uso terapêutico , Humanos , Reino Unido
12.
Acta Haematol ; 143(3): 289-294, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31825922

RESUMO

Approximately 50% of female carriers of hemophilia A have factor VIII (FVIII) levels below 0.5 IU/dL and may be categorized as having mild hemophilia. Females with hemophilia may go undiagnosed for years because the most common symptoms - menorrhagia and bleeding after childbirth - also occur in females without hemophilia. Females with hemophilia can exhibit increased bleeding tendencies despite current guidelines of expected, adequate FVIII levels. The cases described illustrate the clinical variability and presentation of hemophilia in females and highlight the importance of a timely diagnosis, effective management, and monitoring. Prophylactic factor replacement therapy is recommended in females with hemophilia, particularly those with joint disease or gynecologic complications. Affected individuals should receive infusion training and education on treatment options, physical activities, the importance of treatment adherence, and recognizing bleeding symptoms warranting treatment. Further study is needed to increase awareness of hemophilia in females and reassess current guidelines for their management and monitoring.


Assuntos
Fator VIII/genética , Hemofilia A/genética , Adolescente , Idoso , Inversão Cromossômica , Desamino Arginina Vasopressina/efeitos adversos , Desamino Arginina Vasopressina/uso terapêutico , Gerenciamento Clínico , Substituição de Medicamentos , Fator VIII/análise , Fator VIII/uso terapêutico , Feminino , Hemartrose/etiologia , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemofilia A/tratamento farmacológico , Heterozigoto , Humanos , Íntrons/genética , Masculino , Menorragia/etiologia , Pessoa de Meia-Idade , Linhagem , Proteínas Recombinantes/uso terapêutico , Ácido Tranexâmico/uso terapêutico , Adulto Jovem
13.
Acta Haematol ; 143(5): 504-508, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31639805

RESUMO

N8-GP (ESPEROCT®; turoctocog alfa pegol; Novo Nordisk A/S, Bagsvaerd, Denmark) is an extended half-life recombinant factor VIII (FVIII) molecule. FVIII-deficient plasma spiked with N8-GP can be accurately measured using many activated partial thromboplastin time (aPTT)-based one-stage clotting assay reagents with normal human plasma calibrators. To date, there are few data on the measurement accuracy of samples from patients treated with N8-GP. Here, we measure patient samples during routine treatment monitoring. Three previously treated patients with severe hemophilia A (HA) without inhibitors (baseline FVIII activity <0.01 IU/mL) received 50 IU/kg N8-GP every fourth day or twice weekly over 5 years as part of the pathfinder2 trial. Patient samples were monitored using the Pathromtin® SL aPTT reagent (Siemens Healthcare GmbH, Erlangen, Germany), a BCS® XP System analyzer (Siemens), and Standard Human Plasma (Siemens) or product-specific calibrators. Patient age ranged from 36 to 62 years. Overall, measurements performed using product-specific or Standard Human Plasma calibrators were in good agreement, with ratios randomly distributed around 1.0. Peak ratios tended to be closer to 1.0 than trough samples. Pathromtin® SL with Standard Human Plasma calibrator consistently and accurately measured FVIII activity in samples from severe HA patients receiving N8-GP prophylaxis.


Assuntos
Testes de Coagulação Sanguínea/métodos , Fator VIII/análise , Hemofilia A/patologia , Adulto , Fator VIII/uso terapêutico , Hemofilia A/sangue , Hemofilia A/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade
14.
Blood Coagul Fibrinolysis ; 30(1S Suppl 1): S4-S6, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31517708

RESUMO

: In persons with hemophilia, the severity and spontaneity of bleeding episodes depends on the degree of factor deficiency present in the patients. Severe hemophilia is classically characterized by a factor VIII (FVIII) or factor IX (FIX) coagulant activity below 1% of normal (spontaneous and severe bleeds). Moderate hemophilia is associated with factor activity between 1 and 5% of normal (milder spontaneous and traumatic bleeds). Finally, mild hemophilia has factor activity in excess of 5% of normal (traumatic bleeds in general). Nonetheless, this classification is rather simplistic. It is a known fact that there are subjects with identical clotting factor levels who have different bleeding phenotypes. We will analyze different factors to justify this.


Assuntos
Fator IX/análise , Fator VIII/análise , Hemofilia A/sangue , Coagulação Sanguínea , Fator IX/metabolismo , Fator VIII/metabolismo , Hemofilia A/metabolismo , Hemorragia/sangue , Hemorragia/metabolismo , Humanos
15.
J Stroke Cerebrovasc Dis ; 28(11): 104364, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31521516

RESUMO

A 48-year-old woman was admitted to our hospital because of headache and fever. She was diagnosed with aseptic meningitis. Five days later, she had a seizure and developed left hemiparesis. Magnetic resonance imaging showed hyperintensity in the right parietal area on fluid attenuated inversion recovery imaging. She was diagnosed as having cerebral venous thrombosis (CVT) because the suprasagittal sinus was invisible on the venographic studies. Moreover, deep venous thrombosis (DVT) was detected in her left lower extremity. Laboratory findings showed hyperthyroidism and markedly increased factor VIII activity. This is a rare case of concomitant CVT and DVT induced by high factor VIII activity due to hyperthyroidism under the presence of meningitis, an additional risk factor for thrombosis.


Assuntos
Coagulação Sanguínea , Fator VIII/análise , Hipertireoidismo/complicações , Trombose do Seio Sagital/etiologia , Trombose Venosa/etiologia , Anticoagulantes/uso terapêutico , Feminino , Humanos , Hipertireoidismo/sangue , Hipertireoidismo/diagnóstico , Hipertireoidismo/tratamento farmacológico , Iodetos/uso terapêutico , Meningite Asséptica/sangue , Meningite Asséptica/complicações , Pessoa de Meia-Idade , Trombose do Seio Sagital/sangue , Trombose do Seio Sagital/diagnóstico por imagem , Trombose do Seio Sagital/tratamento farmacológico , Resultado do Tratamento , Regulação para Cima , Trombose Venosa/sangue , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/tratamento farmacológico
16.
Am J Surg Pathol ; 43(12): 1711-1719, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31490238

RESUMO

Vascular invasion has been identified as an informative risk factor for relapse in stage I testicular nonseminomas, used to tailor treatment. We investigated interobserver agreement in vascular invasion reporting and studied the potential additional value of immunohistochemistry for vascular markers for predicting relapse. Patients (n=52) with stage I testicular nonseminomas undergoing surveillance (1993-2006) were included (median follow-up of 66 mo). Two formalin-fixed paraffin-embedded blocks with >1 cm tissue and tumor/normal parenchyma interface were stained with hematoxylin and eosin and CD31, FVIII, and D2-40. Slides were assessed by 3 independent testicular germ cell tumor-dedicated pathologists, and agreement was assessed using Cohen κ statistic. Sensitivity, specificity, and accuracy of vascular invasion scoring in predicting relapse were calculated. Agreement among testicular germ cell tumor-dedicated pathologists was moderate (κ=0.49 to 0.54), as was performance in predicting disease relapse (particularly, specificity of 86%). Immunohistochemistry increased overall sensitivity (71%), but decreased specificity (71%) in predicting relapse. All patients (n=8) with both blood and lymphatic vascular invasion developed a relapse. In multivariable analysis (including age, tumor size, rete testis invasion, and serum tumor markers), only vascular invasion had an independent impact in predicting relapse. Assessment of vascular invasion by testicular germ cell tumor-dedicated pathologists is good and is clinically meaningful, predicting disease relapse. Immunohistochemistry for vascular markers improves sensitivity of detecting disease relapse and allows for the identification of high-risk patients with both blood and lymphatic vascular invasion simultaneously, potentially of interest for tailored chemotherapy.


Assuntos
Biomarcadores Tumorais/análise , Vasos Sanguíneos/química , Imuno-Histoquímica , Vasos Linfáticos/química , Recidiva Local de Neoplasia , Neoplasias Embrionárias de Células Germinativas/química , Neoplasias Testiculares/química , Adulto , Anticorpos Monoclonais Murinos , Vasos Sanguíneos/patologia , Bases de Dados Factuais , Fator VIII/análise , Humanos , Vasos Linfáticos/patologia , Masculino , Invasividade Neoplásica , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/mortalidade , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Variações Dependentes do Observador , Molécula-1 de Adesão Celular Endotelial a Plaquetas/análise , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Neoplasias Testiculares/mortalidade , Neoplasias Testiculares/patologia , Neoplasias Testiculares/terapia , Resultado do Tratamento , Adulto Jovem
17.
Acta Med Port ; 32(9): 614-617, 2019 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-31493366

RESUMO

Intramural hematoma of the colon is very rare, particularly when associated with the development of autoantibodies against factor VIII.We report a case of a 66-year-old man with abdominal pain, hematochezia and clots in the left colon, without any radiologic signs of active bleeding or bowel occlusion or analytical changes in routine coagulation screening, but with positive autoantibodies against factor VIII. The clinical instability prompted surgical exploration. An intramural hematoma of the left colon was found, and a left colectomy was performed. The patient was treated with hemoderivatives and corticosteroids with clinical improvement. The diagnosis of spontaneous intramural hematoma might be a challenge, particularly in the absence of clinical suspicion. An early recognition is essential for a positive outcome. This case highlights a rare cause of bleeding and intestinal obstruction, but also the difficulty and relevance of establishing a clinical diagnosis when diagnostic tests are not completely informative.


Assuntos
Dor Abdominal/etiologia , Autoanticorpos/análise , Doenças do Colo/etiologia , Fator VIII/imunologia , Hematoma/etiologia , Hemofilia A/complicações , Idoso , Doenças do Colo/diagnóstico , Doenças do Colo/cirurgia , Colonoscopia , Fator VIII/análise , Hemorragia Gastrointestinal/etiologia , Hematoma/diagnóstico , Hematoma/cirurgia , Hemofilia A/diagnóstico , Humanos , Obstrução Intestinal/etiologia , Masculino
18.
Clin Appl Thromb Hemost ; 25: 1076029619873976, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496264

RESUMO

Patients with von Willebrand disease (VWD) often require treatment with supplemental von Willebrand factor (VWF) prior to procedures or to treat bleeding. Commercial VWF concentrates and more recently recombinant human VWF (rVWF) have replaced cryoprecipitate as the mainstay of therapy. In comparison with cryoprecipitate, the VWF content and multimer distribution under current manufacturing processes of these commercial products has not been reported. We measured the factor VIII (FVIII:C), VWF antigen (VWF:Ag), VWF collagen-binding activity (VWF:CB), VWF platelet-binding activity by GPIbM enzyme-linked immunosorbent assay (VWF:GPIbM), and percentage of high-molecular-weight (HMWM) VWF in 3 pools of group A and O cryoprecipitate, 3 vials of VWF concentrate (Humate-P), and 1 lot of rVWF (Vonvendi). We found that both group O and group A cryoprecipitate have significantly higher ratios of VWF:GPIbM activity and FVIII:C activity relative to VWF:Ag and have better preservation of HMWM than Humate-P. Although not compared statistically, rVWF appears to have more HMWM VWF and a higher ratio of VWF:GPIbM to VWF:Ag than Humate-P and cryoprecipitate. The estimated acquisition cost for our hospital for treating one major bleeding episode was more than 4-fold higher with Humate-P and 7- to 10-fold higher with rVWF than with cryoprecipitate.


Assuntos
Doenças de von Willebrand/tratamento farmacológico , Fator de von Willebrand/análise , Ensaio de Imunoadsorção Enzimática/métodos , Fator VIII/análise , Fibrinogênio/análise , Hemorragia/tratamento farmacológico , Hemorragia/economia , Humanos , Proteínas Recombinantes/análise , Doenças de von Willebrand/economia
20.
Int J Lab Hematol ; 41(5): 679-683, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31421012

RESUMO

INTRODUCTION: The measurements of clotting factor activities are usually performed using a one-stage clotting assay (OSA) or chromogenic substrate assay (CSA). Advances in automated coagulation analysers have led to the utilization of stored calibration curves. There are sometimes substantial intervals between test calibration and analysis of samples. Variability in results can be influenced by calibrant and methodology. Several guidelines recommend calibration and patient samples be performed together in parallel; this incurs costs, but reliance on a stored calibration curve may lead to inaccuracy of results over time. METHODS: We evaluated inclusion of a live truncated (3 point) calibration curve using calibrator plasma alongside test samples and compared results calculated against the stored calibration curves and live truncated calibration to assess the impact on precision and accuracy. The feasibility of this was tested on two hospital sites in the UK; OSA and CSA were performed on Sysmex CS5100 using Actin FS, SynthASil, Innovin, Biophen chromogenic VIII and Rossix chromogenic IX. RESULTS: Results of two batches of IQC were compared for FII, FV, FVII, FX, FIX:C, FXI, FXII and OSA FVIII (FVIII:C1) and CSA FVIII:C (FVIII:CR) and FXI:C. By utilizing a live truncated calibration, precision improved with the most striking examples: FXII %CV 23.1% to 3.1% (site A) FXI 7.3% to 2.4% (site B). The improvement in other clotting factors was more modest. CONCLUSION: To the best of our knowledge, this is the first study that demonstrates that the use of a live truncated calibration curve will improve precision and accuracy.


Assuntos
Fatores de Coagulação Sanguínea/análise , Testes de Coagulação Sanguínea/instrumentação , Testes de Coagulação Sanguínea/métodos , Coagulação Sanguínea , Calibragem/normas , Fatores de Coagulação Sanguínea/metabolismo , Testes de Coagulação Sanguínea/economia , Compostos Cromogênicos , Análise Custo-Benefício , Fator VIII/análise , Fator VIII/metabolismo , Humanos , Proteínas Recombinantes/análise , Proteínas Recombinantes/metabolismo , Padrões de Referência , Reprodutibilidade dos Testes , Tromboplastina/análise , Tromboplastina/metabolismo
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