Blood Coagulation & Fibrinolysis最新文献

{"title":"Efficacy of adjusted weight-based dosing of desmopressin (1-deamino-8-d-arginine vasopressin in type 1 von Willebrand disease.","authors":"Craig D Seaman","doi":"10.1097/MBC.0000000000001251","DOIUrl":"10.1097/MBC.0000000000001251","url":null,"abstract":"","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 7","pages":"462-464"},"PeriodicalIF":1.1,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10836785/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41190296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Venous thromboembolism prophylaxis practices for patients with sickle cell disease prior to and during the COVID-19 pandemic.","authors":"Jennifer Davila, W. Mitchell, K. Morrone, E. Silver, C. Minniti, H. Billett, P. Desai, S. O’Brien, D. Manwani","doi":"10.2139/ssrn.4208107","DOIUrl":"https://doi.org/10.2139/ssrn.4208107","url":null,"abstract":"Patients with sickle cell disease (SCD) are predisposed to a hypercoagulable state due to alterations in the coagulation system. Despite concern for the development of venous thromboembolism (VTE) in this population, there are no standardized guidelines for routine thromboprophylaxis. The objective of this study was to assess thromboprophylaxis practices of adult and pediatric treaters of SCD before and during the coronavirus disease of 2019 (COVID-19) pandemic. A cross-sectional electronic survey was distributed to pediatric and adult hematology oncology practitioners through seven SCD-specific interest groups between May 29, 2020, and July 13, 2020. Of 93 total responses, 14% (N = 13) reported they only treat patients more than 21 years old; 38.7% (N = 36) only treat patients 0-21 years old and 47.3% (N = 44) reported they treat both. Our study showed that before the COVID-19 pandemic, 96% of adult practitioners would recommend pharmacologic thromboprophylaxis, mechanical thromboprophylaxis or both for hospitalized adults with thromboprophylaxis, but only 76% of pediatric treaters would recommend any thromboprophylaxis in hospitalized children (P < 0.0001), with 24% of pediatric treaters choosing no thromboprophylaxis at all. During the COVID-19 pandemic, pharmacologic thromboprophylaxis specifically was recommended for adults by 94% of treaters and for pediatric patients by 76% of treaters. These findings suggest that despite the lack of evidence-based thromboprophylaxis guidelines in adults and children with thromboprophylaxis, subspecialty treaters routinely provide pharmacologic thromboprophylaxis in their adult patients and will modify their practice in pediatric patients who are considered at a high risk for VTE.","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"2 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2023-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75540511","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Biodistribution of recombinant factor IX, extended half-life recombinant factor IX Fc fusion protein, and glycoPEGylated recombinant factor IX in hemophilia B mice.","authors":"Arjan van der Flier,&nbsp;Vu Hong,&nbsp;Zhan Liu,&nbsp;Peter Piepenhagen,&nbsp;Gregory Ulinski,&nbsp;Jennifer A Dumont,&nbsp;Kelly D Orcutt,&nbsp;Apollina Goel,&nbsp;Robert Peters,&nbsp;Joe Salas","doi":"10.1097/MBC.0000000000001230","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001230","url":null,"abstract":"<p><p>Extended half-life recombinant FIX (rFIX) molecules have been generated to reduce the dosing burden and increase the protection of patients with hemophilia B. Clinical pharmacology studies with recombinant factor IX Fc fusion protein (rFIXFc) report a similar initial peak plasma recovery to that of rFIX, but with a larger volume of distribution. Although the pegylation of N9-GP results in a larger plasma recovery, there is a smaller volume of distribution, suggesting less extravasation of the latter drug. In this study, we set out to compare the biodistribution and tissue localization of rFIX, rFIXFc, and glycoPEGylated rFIX in a hemophilia B mouse model. Radiolabeled rFIX, rFIXFc, and rFIX-GP were employed in in vivo single-photon emission computed tomography imaging (SPECT/CT), microautoradiography (MARG), and histology to assess the distribution of FIX reagents over time. Immediately following injection, vascularized tissues demonstrated intense signal irrespective of FIX reagent. rFIX and rFIXFc were retained in joint and muscle areas through 5 half-lives, unlike rFIX-GP (assessed by SPECT). MARG and immunohistochemistry showed FIX agents localized at blood vessels among tissues, including liver, spleen, and kidney. Microautoradiographs, as well as fluorescent-labeled images of knee joint areas, demonstrated retention over time of FIX signal at the trabecular area of bone. Data indicate that rFIXFc is similar to rFIX in that it distributes outside the plasma compartment and is retained in certain tissues over time, while also retained at higher plasma levels. Overall, data suggest that Fc fusion does not impede the extravascular distribution of FIX.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"353-363"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fe/c9/blcof-34-353.PMC10481914.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10531698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Left ventricular assist device thrombosis in the setting of supratherapeutic international normalized ratio (INR) and bleeding.","authors":"Gaurav Gupta,&nbsp;Crystal L Yan,&nbsp;Tricia Kalwar,&nbsp;Nina Thakkar-Rivera","doi":"10.1097/MBC.0000000000001241","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001241","url":null,"abstract":"<p><p>A 71-year-old female with heart failure who underwent left ventricular assist device (LVAD) placement presented for evaluation of low hemoglobin and dark stools. She also had leg pain, numbness, and weakness for which she was taking ibuprofen. She was found to have a gastrointestinal bleed, INR of 4.3, and arterial thrombi in the left leg. She was stabilized, had her anticoagulation held, and underwent mechanical thrombectomy. On hospital day 6, LVAD interrogation revealed signs of thrombosis, while subsequent labs revealed a persistently supratherapeutic INR of 5.2. The patient had the LVAD removed and underwent further hematologic workup. Her platelets remained normal throughout the admission, indicating this was not acute disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), or heparin induced thrombocytopenia (HIT). Echocardiography identified it as a primary thrombus. This case illustrates the importance of appropriate anticoagulation to balance the bleeding risk with the risk of thrombi, as well as the importance of maintaining high suspicion for LVAD thrombosis regardless of INR.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"414-418"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10010089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of frozen storage conditions and freezing rate on the stability of coagulation proteins in human plasma.","authors":"Tereza Fenclova,&nbsp;Frantisek Marecek,&nbsp;Ingrid Hrachovinova","doi":"10.1097/MBC.0000000000001239","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001239","url":null,"abstract":"<p><strong>Objectives: </strong>Degradation of coagulation proteins in frozen plasma may influence assay results. The aims of this study were to explore the changes in coagulation parameters in patient plasma and internal quality control (IQC) after different freezing and storage conditions during the short-term and long-term periods.</p><p><strong>Methods: </strong>Platelet poor plasma was prepared from citrated peripheral blood collected from a group of healthy donors. The plasma was pooled, frozen and stored in a variety of freezing and storage conditions. The changes were monitored using routine coagulation assays, as well as factor VIII (FVIII) and protein S (PS) assays.</p><p><strong>Results: </strong>Plasma stored in liquid nitrogen (LN 2 ) or in -80°C showed long-term stable values for routine tests for a period of over 12 months, and 6 months for FVIII. Interestingly, the activated partial thromboplastin time (aPTT) showed a temporary significant prolongation over the first two weeks. Plasma frozen and stored in -40°C is not viable for aPTT and FVIII testing, otherwise it can be used for other parameters for up to 4 months. PS showed a significant increase in all frozen samples. Freezing rate has a significant impact on plasma quality and the final storage temperature influences the long-term stability.</p><p><strong>Conclusion: </strong>The optimal storage conditions are ultra-low temperatures (LN 2 or -80°C) and the highest freezing rate possible. However, frozen plasma is not viable for IQC of aPTT during a period of two weeks after freezing. This study is unique in its conception as a practical guide for the handling of frozen plasma samples in modern laboratory settings.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"377-384"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10010090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of single nucleotide polymorphisms (4G/5G) of plasminogen activator inhibitor-1 and the risk factors for placenta-related obstetric complications.","authors":"Hanife Guler Donmez,&nbsp;Mehmet Sinan Beksac","doi":"10.1097/MBC.0000000000001242","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001242","url":null,"abstract":"<p><strong>Background: </strong>Placenta-related obstetric complications (PROCs) such as miscarriage, fetal growth restriction, preeclampsia, and preterm birth are the major causes of maternal and fetal morbidity and mortality. The objective of this study was to search the relevance of plasminogen activator inhibitor-1 (PAI-1) polymorphisms and co-morbidities and the risk factors for PROCs such as miscarriage, fetal growth restriction, preeclampsia, and preterm birth.</p><p><strong>Method: </strong>This retrospective study analyzed the PAI-1 genotype in a cohort of 268 multiparous women with poor obstetric history. Poor obstetric history was defined as the presence of at least one of the PROCs and/or poor gestational outcomes at the previous pregnancy/pregnancies.</p><p><strong>Results: </strong>5G allele frequency was higher than the 4G allele frequency in the cohort (0.767 vs. 0.233). The frequencies of having at least one risk factor are relatively similar among the different PAI-1 genotypes ( P  > 0.05). However, the presence of MTHFR polymorphisms (homozygous and compound heterozygous forms of C677T and A1298G) and hereditary thrombophilia (Factor V Leiden and prothrombin G20210A gene mutations, and FXIII deficiency) were found to be associated with PAI 4G/4G ( P  = 0.048) and 5G/5G ( P  = 0.022) genotypes, respectively. Significant differences were not observed in other risk factors and co-morbidities such as autoimmune disorders, chronic inflammatory diseases, history of venous thromboembolism, carbohydrate metabolism disorders, hyperlipidemia, cardiovascular and cerebrovascular diseases depending on PAI-1 genotypes ( P  > 0.05).</p><p><strong>Conclusion: </strong>MTHFR polymorphisms were found to be associated with PAI 4G/4G genotype, while 5G/5G genotype was observed more frequently in hereditary thrombophilia cases.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"396-402"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10016011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"HbA1c/C-peptide ratio is associated with angiographic thrombus burden and short-term mortality in patients presenting with ST-elevation myocardial infarction.","authors":"Kenan Toprak,&nbsp;Mustafa Kaplangöray,&nbsp;Tolga Memioğlu,&nbsp;Mehmet İnanir,&nbsp;Bahadir Omar,&nbsp;Mustafa Beğenç Taşcanov,&nbsp;Asuman Biçer,&nbsp;Recep Demirbağ","doi":"10.1097/MBC.0000000000001240","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001240","url":null,"abstract":"<p><strong>Objectives: </strong>Angiographic high thrombus burden (HTB) is associated with increased adverse cardiovascular events in patients with ST-elevation myocardial infarction (STEMI). HbA1c and C-peptide are two interrelated bioactive markers that affect many cardiovascular pathways. HbA1c exhibits prothrombogenic properties, while C-peptide, in contrast, exhibits antithrombogenic effects. In this study, we aimed to demonstrate the value of combining these two biomarkers in a single fraction in predicting HTB and short-term mortality in patients with STEMI.</p><p><strong>Methods: </strong>1202 patients who underwent primary percutaneous coronary intervention (pPCI) for STEMI were retrospectively included in this study. The study population was divided into thrombus burden (TB) groups and compared in terms of basic clinical demographics, laboratory parameters and HbA1c/C-peptide ratios (HCR). In addition, short-term mortality of the study population was compared according to HCR and TB categories.</p><p><strong>Results: </strong>HCR values were significantly higher in the HTB group than in the LTB group (3.5 ± 1.2 vs. 2.0 ± 1.1; P  < 0.001; respectively). In the multivariable regression analysis, HCR was determined as an independent predictor of HTB both as a continuous variable [odds ratio (OR): 2.377; confidence interval (CI): 2.090-2.704; P  < 0.001] and as a categorical variable (OR: 5.492; CI: 4.115-7.331; P  < 0.001). In the receiver operating characteristic (ROC) analysis, HCR predicted HTB with 73% sensitivity and 72% specificity, and furthermore, HCR's predictive value for HTB was superior to HbA1c and C-peptide. The Kaplan-Meier cumulative survival curve showed that short-term mortality increased at HTB. In addition, HCR strongly predicted short-term mortality in Cox regression analysis.</p><p><strong>Conclusions: </strong>In conclusion, HCR is closely associated with HTB and short-term mortality in STEMI patients.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"385-395"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10016013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of autoimmune factor XIII deficiency in a frail, elderly patient.","authors":"Masahiro Kaneko,&nbsp;Naoto Ishimaru,&nbsp;Takahiro Nakajima,&nbsp;Yohei Kanzawa,&nbsp;Hiroyuki Seto,&nbsp;Saori Kinami,&nbsp;Tsukasa Osaki,&nbsp;Masayoshi Souri,&nbsp;Akitada Ichinose","doi":"10.1097/MBC.0000000000001202","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001202","url":null,"abstract":"<p><p>Autoimmune factor XIII/13 deficiency (aFXIII deficiency) is a rare hemorrhagic disorder, for which typical guideline-directed treatment is aggressive immunosuppressive therapy. Approximately 20% of patients are over 80 years old; however, and optimum management of such patients has not reached consensus. Our elderly patient had massive intramuscular hematoma, and aFXIII deficiency was diagnosed. The patient opted against aggressive immunosuppressive therapy, so he was managed with conservative treatment only. Thorough survey of other correctable causes of bleeding and anemia is also required in similar cases. Our patient's serotonin-norepinephrine reuptake inhibitor use and multivitamin deficiency (vitamin C, B 12 and folic acid) were revealed to be aggravating factors. Fall prevention and muscular stress prevention are also important in elderly patients. Our patient had two relapses of bleeding within 6 months, which were improved spontaneously by bed rest without factor XIII replacement therapy or blood transfusion. Conservative management may be preferred for frail and elderly patients with aFXIII deficiency when they opt against standard therapy.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"408-413"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10017293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disseminated intravascular coagulation in acute leukemia patients.","authors":"Tomoyuki Kawada","doi":"10.1097/MBC.0000000000001245","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001245","url":null,"abstract":"","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"424-425"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10028941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Measuring coagulopathy in pediatric craniofacial surgery.","authors":"Alexandra J Borst,&nbsp;Christopher M Bonfield,&nbsp;Poornachanda S Deenadayalan,&nbsp;Chi H Le,&nbsp;Meng Xu,&nbsp;Srijaya K Reddy","doi":"10.1097/MBC.0000000000001234","DOIUrl":"https://doi.org/10.1097/MBC.0000000000001234","url":null,"abstract":"<p><p>The goal of this study was to describe hematologic and coagulation laboratory parameters and identify if these laboratory studies could predict blood loss in a cohort of pediatric patients undergoing complex cranial vault reconstruction (CCVR) for repair of craniosynostosis. We reviewed records from 95 pediatric CCVR patients between 2015 and 2019. Primary outcome measures were hematologic and coagulation laboratory parameters. Secondary outcome measures were intraoperative and postoperative calculated blood loss (CBL). Preoperative laboratory values were within normal limits and did not predict outcomes. Intraoperative platelet count and fibrinogen predicted CBL but without clinically relevant thrombocytopenia or hypofibrinogenemia. Intraoperative prothrombin time (PT) and partial thromboplastin time (PTT) predicted perioperative CBL, possibly reflecting surgically induced coagulopathy. Postoperative laboratory values did not predict postoperative blood loss. We found that standard hematologic and coagulation laboratory parameters predicted intraoperative and postoperative blood loss but provided limited mechanistic information to improve our understanding of coagulopathy in craniofacial surgery.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":"34 6","pages":"403-407"},"PeriodicalIF":1.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10371893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}