Abstract：
:Patients with myeloproliferative neoplasms (MPNs), such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are at increased risk for arterial and venous thrombosis/thromboembolism. In particular, the risk of splanchnic venous thrombosis, such as portal vein thrombosis or Budd-Chiari syndrome, is significantly higher in patients with MPN than in the normal population. At the same time, MPN patients are at increased risk for severe bleeding. Therefore, the treatment of patients with MPN must be based on their suspected probability of thrombosis/thromboembolism and bleeding. For this purpose, patient and MPN-specific risk factors are used. Patients at expected high risk of thrombosis should receive adequate primary or secondary thromboprophylaxis in addition to cytoreductive therapy. This may consist of antiplatelet agents and/or anticoagulant agents and must be balanced with the individual bleeding risk. The goal is to increase the quality of life and life span of patients with MPNs by preventing (re-)thrombosis and severe bleeding. :Patienten mit Myeloproliferativen Neoplasien (MPN), wie der Polycythämia vera (PV), Essentiellen Thrombozythämie (ET) und Primären Myelofibrose (PMF), sind einem erhöhten Risiko für arterielle und venöse Thromboembolien ausgesetzt. Besonders das Risiko für splanchnische Venenthrombosen, wie die Pfortaderthrombose oder das Budd-Chiari-Syndrom, ist bei Patienten mit MPN vielfach höher als in der Normalbevölkerung. Gleichzeitig besteht bei MPN-Patienten ein erhöhtes Risiko für schwergradige Blutungen. Daher muß sich die Therapie der Patienten mit MPN an der Wahrscheinlichkeit für Thromboembolien und Blutungen orientieren. Hierfür werden patienten- und MPN-spezifische Risikofaktoren herangezogen. Patienten mit zu erwartendem hohem Risiko für Thrombosen sollten zusätzlich zu einer zytoreduktiven Therapie eine angemessene Primär- oder Sekundärprophylaxe erhalten. Diese kann aus Thrombozytenfunktionshemmern und/oder Antikoagulanzien bestehen und muß mit dem individuellen Blutungsrisiko balanciert werden. Ziel ist es, durch die Verhinderung von (Re-)Thrombosen und schwergradigen Blutungen die Lebensqualität und Lebensspanne der Patienten mit MPN zu erhöhen.
journal_name
Hamostaseologiejournal_title
Hamostaseologieauthors
Koschmieder Sdoi
10.1055/s-0040-1701474subject
Has Abstractpub_date
2020-02-01 00:00:00pages
47-53issue
1eissn
0720-9355issn
2567-5761journal_volume
40pub_type
杂志文章,评审相关文献
Hamostaseologie文献大全abstract：:The DHR (Deutsches Hämophilieregister, German Haemophilia Register) records patient data on haemophilia A, haemophilia B, von Willebrand disease, and other coagulation factor deficiency disorders. The DHR has been online since 2009. The participation in the DHR leads to additional administrative workload for the hospi...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2010-11-01 00:00:00
abstract：:Atherosclerosis and its major complications - myocardial infarction and stroke - remain major causes of death and disability in the United States and world-wide. Indeed, with dramatic increases in obesity and diabetes mellitus, the prevalence and public health impact of cardiovascular diseases (CVD) will likely remain...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/HAMO-14-11-0076
更新日期：2016-05-10 00:00:00
abstract：:Clinically overt, preoperative, disseminated intravascular coagulation (DIC) is not common in patient with aortic aneurysm. However, cases with large and expanding aneurysm are especially prone for this coagulopathy. Contrary to the clinically overt form compensated DIC in patients with aortic aneurysm seems frequent ...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.1267/hamo04030162
更新日期：2004-08-01 00:00:00
abstract：:The antiphospholipid syndrome (APS) is defined by the association of arterial and/or venous thrombosis and/or pregnancy complications with the presence of at least one among the main antiphospholipid antibodies (aPL) (i. e., Lupus anticoagulants, LA, IgG and/or IgM anticardiolipin antibodies, aCL, IgG and/or IgM antiβ...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/ha-1165
更新日期：2011-11-01 00:00:00
abstract：:Trauma-induced coagulopathy (TIC) is a heterogeneous entity that contributes to a significant morbidity and mortality following trauma. The activated protein C system, endotheliopathy and platelet dysfunction have been implicated in the pathogenesis of TIC, although there are still controversies on the exact pathogene...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.1055/s-0039-1677853
更新日期：2019-02-01 00:00:00
abstract：:Vitamin K 2,3-epoxide reductase complex, subunit 1 (VKORC1) is an enzyme essential for the vitamin K cycle. VKORC1 catalyses the reduction of vitamin K 2,3-epoxide to the quinone form of vitamin K and further to vitamin K hydroquinone. The generated vitamin K hydroquinone serves as substrate for the enzyme γ-glutamyl-...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：
更新日期：2016-11-08 00:00:00
abstract：:Hermansky-Pudlak syndrome (HPS) is a rare, autosomal recessive disorder affecting lysosome-related organelles (LRO), including dense platelet granules. HPS causes oculocutaneous hypopigmentation, bleeding diathesis and granulomatous colitis or pulmonary fibrosis. To date, there is no curative treatment and the clinica...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/HAMO-14-06-0024
更新日期：2014-01-01 00:00:00
abstract：:Methylation, CpG island, promoter, intron 1 Haemophilia A is the most common X-linked inherited coagulation disorder caused by a deficiency of the factor VIII protein (FVIII). A plethora of different mutations in the factor VIII gene (F8) have been identified as causative for this bleeding disease including a few prom...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2013-01-01 00:00:00
abstract：AIM:In children, screening for haemorrhagic disorders is further complicated by the fact that infants and young children with mild disease in many cases most likely will not have a significant history of easy bruising or bleeding making the efficacy of a questionnaire even more questionable. PATIENTS, METHODS:We compa...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2009-10-01 00:00:00
abstract：:Three selected casuistic studies of individual patient problems from my long-standing professional experience are presented. I intend to highlight the potential contribution of case studies, including new approaches of laboratory investigation, for the advancement of pathophysiological knowledge and would encourage to...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：10.1055/a-1191-7198
更新日期：2020-11-01 00:00:00
abstract：:Current antithrombotic therapy in patients with acute coronary syndrome (ACS) comprises antiplatelet and anticoagulant therapy. Dual antiplatelet therapy composed of aspirin plus a third generation P2Y12 inhibitor (prasugrel or ticagrelor) represents the gold standard, while aspirin plus second generation P2Y12 inhibi...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/HAMO-13-09-0048
更新日期：2014-01-01 00:00:00
abstract：:Superficial vein thrombosis (SVT) is a common disease, characterized by an inflammatory-thrombotic process in a superficial vein. Typical clinical findings are pain and a warm, tender, reddish cord along the vein. Until recently, no reliable epidemiological data were available. The incidence is estimated to be higher ...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：10.5482/HAMO-13-04-0027
更新日期：2013-08-01 00:00:00
abstract：:Obesity is a major cause for a spectrum of metabolic syndrome-related diseases that include insulin resistance, type 2 diabetes, and steatosis of the liver. Inflammation elicited by macrophages and other immune cells contributes to the metabolic abnormalities in obesity. In addition, coagulation activation following t...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/HAMO-14-11-0068
更新日期：2015-01-01 00:00:00
abstract：:An overview is given on congenital and acquired thrombocytopenias, their pathophysiology, symptoms, diagnosis and therapy with emphasizing heparin-induced thrombocytopenia (HIT) and the thrombotic thrombocytopenic purpura (TTP). ...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：
更新日期：2003-11-01 00:00:00
abstract：:Direct oral anticoagulants (DOACs) are increasingly prescribed substances in patients with indication for effective anticoagulation. Patients with chronic kidney disease (CKD) have a high burden of cardiovascular risk and are more likely to develop atrial fibrillation (AF) than patients without CKD. Patients with mild...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/HAMO-17-01-0003
更新日期：2017-01-01 00:00:00
abstract：:Von Willebrand factor (VWF) structure-function relationship has been studied only through in vitro approaches. The VWF-deficient mouse model has been extremely useful to examine the in vivo function of VWF but does not allow a more subtle analysis of the relative importance of its different domains. However, consideri...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2009-01-01 00:00:00
abstract：:This is a very short review on quantitative coagulation factor assays for the beginner. For systematic training several excellent textbooks in German language are available. Quantitative functional assays of coagulation factors and of physiological inhibitor proteins are based on the principle of parallel-line or slop...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：
更新日期：2006-01-01 00:00:00
abstract：:The occurrence of cerebral or retinal ischemic symptoms ipsilateral to high-grade internal carotid artery (ICA) stenosis indicates a status of instability with a substantial risk for future major stroke. Additionally, the detection of microembolic signals downstream of ICA stenosis is predictive for future cerebral is...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：
更新日期：2003-05-01 00:00:00
abstract：:Myocardial infarction (MI) shows a strong heritability. For a long time the identification of responsible genes has been rather unsuccessful. However, with the advent of genome wide association studies (GWAS) using DNA-array technology a number of significant loci for MI have been identified which were widely replicat...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2010-11-01 00:00:00
abstract：UNLABELLED:With the development of clotting-factors in the seventies the haemophilia patients were released from being handicapped and began to live a quite normal life. Thus, psychosocial counselling did not seem to be necessary. But the impact of HIV-infection to the world of haemophilia was so intense that professio...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2010-11-01 00:00:00
abstract：:In Germany one-third of the women in fertile age use combined oral contraceptives (COCs), which consist mostly of ethinylestradiol (EE) and a synthetic progestin. Older COCs with norethisterone or levonorgestrel have a lower risk for venous thromboembolism (VTE) than newer COCs with desogestrel, drospirenone, or gesto...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.1055/s-0039-1677806
更新日期：2019-02-01 00:00:00
abstract：:Recurrent venous thromboembolism is associated with increased mortality in 5-9% of the patients. On the other hand prolonged anticoagulation can increase the bleeding risk which can also be responsible for an increased mortality. Therefore, it is necessary to validate the recurrence risk of venous thromboembolism on a...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/ha-1144
更新日期：2011-02-01 00:00:00
abstract：:Nearly every second patient, who needs an oral anticoagulation with vitamin K antagonists, is feasible to perform INR-self-management after participation in a structured teaching program. Patient self-testing or measuring the INR value by relatives or nurses would increase this method of control by the aid of telemedi...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2008-10-01 00:00:00
abstract：:This case report concerns a pregnant multipara (age: 27 years) in the 16th gestational week. She developed a sudden onset of paraesthesia in her left lower arm although injecting dalteparin 5000 IU once daily subcutaneously (s. c.) due to a heterozygous factor V Leiden mutation and a prior miscarriage in the first pre...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：10.5482/HAMO-14-03-0022
更新日期：2014-01-01 00:00:00
abstract：INTRODUCTION: Regular visits at haemophilia treatment centres (HTCs) in rural regions are often dependent on the access to a private car due to lack of or limited availability of public means. Therefore, a mobile haemophilia outpatient care (MHOC) concept providing home visits to haemophilia patients has been developed...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：10.1055/s-0038-1654721
更新日期：2018-08-01 00:00:00
abstract：UNLABELLED:In recent years thrombophilia has gained much attention as a risk factor for pregnancy complications. Whereas there is an established correlation between antiphospholipid-antibodies and pregnancy loss, data for other risk factors of thrombosis are less well established. Data suggest associations with antithr...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2008-01-01 00:00:00
abstract：:For the last decades, anticoagulation for stroke prevention in atrial fibrillation (AF) as well as for the prophylaxis and long-term treatment of venous thromboembolism has been entirely based on vitamin K antagonists (VKA). Although very effective under optimal conditions, long-term treatment with these drugs is flaw...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.5482/ha-12050008
更新日期：2012-01-01 00:00:00
abstract：:Acquired haemophilia A (AHA) is a rare autoimmune disease caused by antibodies directed against clotting factor VIII. About half of cases are idiopathic, but AHA may also be secondary to autoimmune, dermatologic, or oncologic diseases. In approximately 10% of non-idiopathic cases, the disease occurs after or with the ...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：10.1055/s-0038-1668570
更新日期：2019-02-01 00:00:00
abstract：UNLABELLED:Molecular genetic testing for factor VIII (FVIII) mutations is indicated in haemophilia A since determination of FVIII activity cannot reliably identify female carriers. Given the large number of FVIII mutations the identification of novel mutations is not uncommon. Since amino acid polymorphisms of FVIII ar...
journal_title：Hamostaseologie
pub_type： 杂志文章
doi：
更新日期：2009-05-01 00:00:00
abstract：:The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has bee...
journal_title：Hamostaseologie
pub_type： 杂志文章,评审
doi：10.1055/s-0038-1675355
更新日期：2019-06-01 00:00:00
