HamostaseologiePub Date : 2023-08-01DOI: 10.1055/a-2080-6602
Caroline Bargehr, Ralf Knöfler, Werner Streif
{"title":"Treatment of Inherited Platelet Disorders: Current Status and Future Options.","authors":"Caroline Bargehr, Ralf Knöfler, Werner Streif","doi":"10.1055/a-2080-6602","DOIUrl":"https://doi.org/10.1055/a-2080-6602","url":null,"abstract":"<p><p>Inherited platelet disorders (IPDs) comprise a heterogeneous group of entities that manifest with variable bleeding tendencies. For successful treatment, the underlying platelet disorder, bleeding severity and location, age, and sex must be considered in the broader clinical context. Previous information from the AWMF S2K guideline #086-004 (www.awmf.org) is evaluated for validity and supplemented by information of new available and future treatment options and clinical scenarios that need specific measures. Special attention is given to the treatment of menorrhagia and risk management during pregnancy in women with IPDs. Established treatment options of IPDs include local hemostatic treatment, tranexamic acid, desmopressin, platelet concentrates, and recombinant activated factor VII. Hematopoietic stem cell therapy is a curative approach for selected patients. We also provide an outlook on promising new therapies. These include autologous hematopoietic stem cell gene therapy, artificial platelets and nanoparticles, and various other procoagulant treatments that are currently tested in clinical trials in the context of hemophilia.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10063080","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}
HamostaseologiePub Date : 2023-08-01DOI: 10.1055/a-2010-6484
Lennart Beckmann, Maximilian Lennartz, Annika Poch, Katharina Holstein, Carsten Bokemeyer, Florian Langer
{"title":"Expression and Release of Tumor Cell Tissue Factor Triggers Recurrent Thromboembolism in a Patient with Endometrial Cancer.","authors":"Lennart Beckmann, Maximilian Lennartz, Annika Poch, Katharina Holstein, Carsten Bokemeyer, Florian Langer","doi":"10.1055/a-2010-6484","DOIUrl":"https://doi.org/10.1055/a-2010-6484","url":null,"abstract":"<p><p>Although cancer-associated thrombosis (CAT) is a frequent complication in patients with malignancies, its treatment remains a challenge in daily practice. Here, we report the clinical course of a 51-year-old woman presenting with a highly thrombogenic paraneoplastic coagulopathy. Despite therapeutic anticoagulation with various agents, including rivaroxaban, fondaparinux, and low-molecular-weight heparin, the patient suffered from recurrent venous and arterial thromboembolism. Locally advanced endometrial cancer was identified. Tumor cells showed strong expression of tissue factor (TF), and significant concentrations of TF-bearing microvesicles were detected in patient plasma. Coagulopathy was controlled only by continuous intravenous anticoagulation with the direct thrombin inhibitor, argatroban. Multimodal antineoplastic treatment, including neoadjuvant chemotherapy followed by surgery and postoperative radiotherapy, resulted in clinical cancer remission, which was paralleled by normalization of tumor markers, CA125 and CA19-9, D-dimer levels, and TF-bearing microvesicles. In summary, continuous anticoagulation with argatroban and multimodal anticancer treatment may be necessary to control TF-driven coagulation activation with recurrent CAT in endometrial cancer.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10479002","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}
HamostaseologiePub Date : 2023-08-01DOI: 10.1055/s-0043-1774284
{"title":"Erhöhte Faktor-VIII-Aktivität – gesteigertes Risiko für Pfortaderthrombosen.","authors":"","doi":"10.1055/s-0043-1774284","DOIUrl":"https://doi.org/10.1055/s-0043-1774284","url":null,"abstract":"PFORTADERTHROMBOSEN KOMMEN BEI PATIENTEN MIT LEBERZIRRHOSE HäUFIG VOR. SIE KöNNEN, AUCH WENN SIE HäUFIG ALS ZUFALLSBEFUND DIAGNOSTIZIERT WERDEN, EINE PORTALE HYPERTENSION AGGRAVIEREN UND DAS RISIKO FüR GASTROINTESTINALE VARIZENBLUTUNGEN ERHöHEN. EINE AKTUELLE STUDIE ZEIGTE, DASS EINE ERHöHTE FAKTOR-VIII-AKTIVITäT MIT EINER ERHöHTEN INZIDENZ UND SCHWERE VON PFORTADERTHROMBOSEN EINHERGEHEN KöNNTE.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10152638","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}
HamostaseologiePub Date : 2023-08-01DOI: 10.1055/s-0043-1774285
{"title":"Postthrombotisches Syndrom: SOX-PTS-Score und Méan-Modell zeigen beste Prädiktion.","authors":"","doi":"10.1055/s-0043-1774285","DOIUrl":"https://doi.org/10.1055/s-0043-1774285","url":null,"abstract":"DAS POSTTHROMBOTISCHE SYNDROM STELLT DIE HäUFIGSTE KOMPLIKATION EINER TIEFEN VENENTHROMBOSE DAR: 20-50% DER PATIENTEN MIT PROXIMALER TIEFER VENENTHROMBOSE DER UNTEREN EXTREMITäT SIND DAVON BETROFFEN. VON DREI VERSCHIEDENEN ETABLIERTEN RISIKOPRäDIKTIONSMODELLEN FüR DIE MANIFESTATION EINES POSTTHROMBOTISCHEN SYNDROMS WIESEN ZWEI EINE GUTE PRäDIKTION AUF.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10151063","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":"Comparison of Performances among Four Bleeding-Prediction Scores in Elderly Cancer Patients with Venous Thromboembolism.","authors":"Shaozhi Xi, Chaoyang Liu, Shuihua Yu, Jingxuan Qiu, Shuibo He, Zhong Yi","doi":"10.1055/a-1984-7210","DOIUrl":"https://doi.org/10.1055/a-1984-7210","url":null,"abstract":"<p><p>The performances of RIETE, VTE-BLEED, SWITCO65 + , and Hokusai-VTE scores for predicting major bleeding events in hospitalized elderly cancer patients with venous thromboembolism (VTE) have not been evaluated. This study validated the performances of these scoring systems in a cohort of elderly cancer patients with VTE. Between June 2015 and March 2021, a total of 408 cancer patients (aged ≥ 65 years) with acute VTE were consecutively enrolled. The overall rates of in-hospital major bleeding and clinically relevant bleeding (CRB) were 8.3% (34/408) and 11.8% (48/408), respectively. RIETE score could categorize patients with increasing rate of major bleeding and CRB into low-/intermediate- and high-risk categories (7.1 vs. 14.1%, <i>p</i> = 0.05 and 10.1 vs. 19.7%, <i>p</i> = 0.02, respectively). The discriminative power of the four scores for predicting major bleeding was poor to moderate, indicated by areas under the receiver operating characteristic curves (0.45 [95% confidence interval, CI: 0.35-0.55] for Hokusai-VTE, 0.54 [95% CI: 0.43-0.64] for SWITCO65 + , 0.58 [95% CI: 0.49-0.68] for VTE-BLEED, and 0.61 [95% CI: 0.51-0.71] for RIETE). RIETE score might be used to predict major bleeding in hospitalized elderly cancer patients with acute VTE.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d3/ca/10-1055-a-1984-7210.PMC10446890.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10119549","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}
HamostaseologiePub Date : 2023-08-01Epub Date: 2023-08-31DOI: 10.1055/a-2031-7804
Jürgen Koscielny, Günther Kappert, Christoph Sucker
{"title":"Nachtrag zur kritischen Diskussion über das Deutsche Hämophilieregister (DHR) anlässlich der GTH-Jahrestagung in Frankfurt 2023, aktuelle Informationen zur Gefährdung der Versorgung mit Blutplasmaprodukten und Aus für Siemens Atellica in Deutschland.","authors":"Jürgen Koscielny, Günther Kappert, Christoph Sucker","doi":"10.1055/a-2031-7804","DOIUrl":"10.1055/a-2031-7804","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10151657","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}
HamostaseologiePub Date : 2023-06-01Epub Date: 2023-06-20DOI: 10.1055/a-2017-8694
Christoph Sucker, Jürgen Koscielny
{"title":"Veranstaltung des BDDH im Rahmen der 67. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung in Frankfurt.","authors":"Christoph Sucker, Jürgen Koscielny","doi":"10.1055/a-2017-8694","DOIUrl":"10.1055/a-2017-8694","url":null,"abstract":"Im Rahmen der 67. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) in Frankfurt veranstaltete der BDDH am 23.2.2022 nach der Mitgliederversammlung eine öffentliche Sitzung zu berufspolitischen Themen. Die Sitzung war sehr gut besucht, wobei neben BDDH- Mitgliedern auch andere interessierte Ärzte und Vertreter der Industrie teilnahmen. In dieser Ausgabe der „BDDH News“ berichten wir über 2 Themenkomplexe aus dieser Sitzung und fassen die gehaltenen Vorträge zusammen.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10046941","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}
HamostaseologiePub Date : 2023-06-01DOI: 10.1055/a-1848-3649
Hannah Glonnegger, Nicole Glatthaar, Matthias Eckenweiler, Michael Barth, Markus Uhl, Martin Büchsel, Barbara Zieger
{"title":"Laboratory Findings, Medical Imaging, and Clinical Outcome in Children with Cerebral Sinus Venous Thrombosis.","authors":"Hannah Glonnegger, Nicole Glatthaar, Matthias Eckenweiler, Michael Barth, Markus Uhl, Martin Büchsel, Barbara Zieger","doi":"10.1055/a-1848-3649","DOIUrl":"https://doi.org/10.1055/a-1848-3649","url":null,"abstract":"<p><strong>Introduction: </strong> Cerebral sinus venous thrombosis (CSVT) is a rare disease, especially in children. Therefore, thrombophilia markers, risk factors, treatment strategy, and MRI, as well as clinical outcome need further investigation to support future diagnostic and therapeutic guidelines for children.</p><p><strong>Methods: </strong> We retrospectively identified all children with CSVT treated in our center between January 1, 2000, and December 31, 2015. Risk factors and laboratory findings were investigated. Furthermore, outcome and treatment satisfaction were evaluated using magnetic resonance imaging (MRI) analyses and a modified questionnaire.</p><p><strong>Results: </strong> All 43 patients, who agreed to participate, were treated with therapeutic levels of heparin; 86% of children had an increased risk for thromboembolic events upon onset of CSVT (acute disease: 58.1%, perinatal risk factors: 9.3%, medical intervention/immobility: 14%, chronic disease: 16.3%). Thrombophilia markers showed positive results (e.g., reduced values for protein C/S, factor-V-Leiden mutation) in 58% of children at the time of CSVT diagnosis but dropping to 20.9% over the course of the disease. Forty-two of 43 patients received MRI follow-ups and the outcome showed complete recanalization in 69% of the patients and partial recanalization in 31%. At the onset of CSVT, 88% of patients reported restrictions in everyday life due to CSVT; at follow-up this percentage declined to 18%. Satisfaction with the outcome among parents/patients according to the questionnaire was high with 1.7 (German school grades from 1 to 6).</p><p><strong>Conclusions: </strong> All 42 children with MRI follow-up demonstrated complete or partial recanalization under anticoagulation. This positive result underlines the need for future studies on anticoagulation to optimize therapy regimens of pediatric CSVT.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10025947","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}
HamostaseologiePub Date : 2023-06-01DOI: 10.1055/a-2020-4643
Sinan Genç, Ahmet Sefa Yeter, Ahmet Burak Oğuz, Ayça Koca, Onur Polat, Müge Günalp Eneyli
{"title":"Rectus Sheath Hematoma in a Patient with Dual-Antiplatelet Therapy Including Ticagrelor: A Case Report.","authors":"Sinan Genç, Ahmet Sefa Yeter, Ahmet Burak Oğuz, Ayça Koca, Onur Polat, Müge Günalp Eneyli","doi":"10.1055/a-2020-4643","DOIUrl":"https://doi.org/10.1055/a-2020-4643","url":null,"abstract":"<p><p>Rectus sheath hematoma (RSH) is an uncommon cause of abdominal pain associated with several risk factors including trauma, asthma, chronic obstructive pulmonary disease, pregnancy, and anticoagulation as it can be iatrogenic. Dual-antiplatelet therapy (DAPT), combined usage of a P<sub>2</sub>Y<sub>12</sub> receptor inhibitor and aspirin, is a cornerstone treatment for patients with acute coronary syndromes. Ticagrelor is a P<sub>2</sub>Y<sub>12</sub> receptor inhibitor with several adverse hemorrhagic complications. An 86-year-old male patient was admitted to the emergency department with abdominal pain and a palpable abdominal mass at the left upper quadrant of the abdomen. His medical history revealed coronary artery disease with medications including acetylsalicylic acid and ticagrelor. Contrast-enhanced abdominal computed tomography revealed RSH. The patient was treated conservatively with bed rest and analgesia. DAPT is an essential component of the management of acute coronary syndromes to prevent recurrent cardiac thrombotic events. However, hemorrhagic complications such as RSH may be encountered with DAPT. Emergency medicine physicians and cardiologists should keep in mind RSH in patients presenting with abdominal pain and using DAPT with ticagrelor.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9671248","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}