Development of Haemophilia Treatment in the Eastern Part of Germany over the Last Decade in the Kompetenznetz H ämorrhagische Diathese Ost (KHDO).
CONCLUSIONS:  Within the last decade more patients with severe haemophilia were switched to a prophylactic regimen going along with a moderate increase in factor consumption achieving a low ABR and AJBR. PMID: 31711245 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - November 11, 2019 Category: Hematology Authors: Mahn R, Schilling K, Klamroth R, Kentouche K, Aumann V, Fischer L, Holzhauer S, Sirb H, Scholz U, Trautmann K, Halm-Heinrich I, Krammer-Steiner B, Koscielny J, Kreibich U, Pietrzak-Büttner A, Tregel M, Knöfler R, Pfrepper C, and the Kompetenznetzwerk H Tags: Hamostaseologie Source Type: research

How Do We Treat Pregnancy-Related Venous Thromboembolism?
Abstract Venous thromboembolism (VTE) is a major cause of maternal morbidity and mortality during pregnancy and the postpartum period. Due to a lack of adequate study data, therapeutic strategies for pregnancy-related VTE are deduced from observational studies and extrapolated from recommendations for nonpregnant patients. Because heparins do not cross the placenta, weight-adjusted therapeutic-dose low-molecular-weight heparins (LMWHs) are the anticoagulant treatment of choice in cases of VTE during pregnancy. Once- and twice-daily dosing regimens are suitable. There is no evidence that measurement of factor Xa ac...
Source: Hamostaseologie - November 4, 2019 Category: Hematology Authors: Linnemann B, Seelbach-Goebel B, Heimerl S, Hart C Tags: Hamostaseologie Source Type: research

[82 years old, male high-risk cardiac patient to planned Y-prosthesis implantation for abdominal aortic aneurysm : Preparation for the medical specialist examination: part 38].
g O PMID: 31673751 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - October 31, 2019 Category: Hematology Authors: Gürtler K, Giebl A, Spring O Tags: Anaesthesist Source Type: research

Long-Term Safety and Efficacy Data of a Plasma-Derived Factor VIII Concentrate with von Willebrand Factor for Treatment of Patients with Hemophilia A Covering 18 Years.
We describe the results of the (to our knowledge) longest long-term noninterventional study so far performed to obtain real-life data on the treatment of hemophilia A patients with a single plasma-derived FVIII concentrate containing von Willebrand factor (pdFVIII; Haemoctin/Faktor VIII SDH Intersero). A total of 198 patients (146 in Germany and 52 in Hungary), of whom 160 had severe and 38 nonsevere hemophilia A, representing all age groups (0-88 years; mean ∼25 years at inclusion) were analyzed during prophylactic or on-demand treatment over 18 years (overall 1,418 patient-years; mean>7 years). pdFVIII was very ef...
Source: Hamostaseologie - October 24, 2019 Category: Hematology Authors: Kittler SFK, Miesbach W, Bauhofer A, Becker T, Schüttrumpf J, Dubovy P, Nemes L, Richter H, Königs C Tags: Hamostaseologie Source Type: research

Causes and Secondary Prevention of Acute Ischemic Stroke in Adults.
Abstract Stroke still remains a major cause of death and disability worldwide. Ischemic stroke is the most common type of stroke. Causes of ischemic stroke can be classified into large-artery atherosclerosis, cardiogenic embolism, small-vessel disease, stroke of other determined etiology, and stroke of undetermined etiology. Stroke causes in adults are mainly attributable to classical cardiovascular risk factors such as hypertension, diabetes, hypercholesterolemia, and smoking. In neuroimaging, stroke subtypes can be defined according to lesion localization and distribution (territorial infarct, lacunar infarct, h...
Source: Hamostaseologie - October 24, 2019 Category: Hematology Authors: Jensen M, Thomalla G Tags: Hamostaseologie Source Type: research

A Prolonged Treatment Response in Acquired Von Willebrand Syndrome.
We report herein a case of AVWS due to a monoclonal gammopathy of undetermined significance, in which a transient but prolonged response to a treatment by intravenous immunoglobulin (IVIG) was observed. The diagnosis was fortuitously made in a preoperative setting for neurosurgery, after biological exploration of an isolated prolonged activated partial thromboplastin time. AVWS was confirmed by an accelerated clearance of an infused plasma-derived von Willebrand factor (VWF) concentrate. High doses of IVIG were used to perform the neurosurgery. Fifty-four days after IVIG, the patient was still responding to treatment with ...
Source: Hamostaseologie - September 23, 2019 Category: Hematology Authors: Désage S, Le Quellec S, Karlin L, Lienhart A, Meunier S, Rugeri L Tags: Hamostaseologie Source Type: research

Functional and clinical characterization of the alternatively spliced isoform AML1-ETO9a in adult patients with translocation t(8;21)(q22;q22.1) acute myeloid leukemia (AML).
aschka P, Döhner H, Geiger H, Borggrefe T, Döhner K, Oswald F PMID: 31462736 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - August 28, 2019 Category: Hematology Authors: Agrawal M, Schwarz P, Giaimo BD, Bedzhov I, Corbacioglu A, Weber D, Gaidzik VI, Jahn N, Rücker FG, Schroeder T, Kindler T, Wattad M, Götze K, Lübbert M, Salwender H, Ringhoffer M, Lange E, Koller E, Thol F, Heuser M, Ganser A, Bullinger L, Paschka P, D Tags: Leukemia Source Type: research

Therapie des Antiphospholipid-Syndroms (APS) mit DOAKs.
t E PMID: 31404932 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - August 12, 2019 Category: Hematology Authors: Bauersachs R, Schellong S, Stücker M, Oldenburg J, Kalka C, Scholz U, Lindhoff-Last E Tags: Hamostaseologie Source Type: research

Immune Thrombocytopenia-A Disease or a Group of Disorders? Where Do We Stand in 2019?
PMID: 31390662 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - August 7, 2019 Category: Hematology Authors: Bakchoul T Tags: Hamostaseologie Source Type: research

[Prevalence of cancer patients in German intensive care units].
Böll B Abstract INTRODUCTION: Cancer is one of the leading causes of death worldwide. Due to increasing comorbidities, age and aggressive chemotherapy, care of cancer patients in intensive care units (ICUs) is more and more necessary. So far, little is known about the care structure of cancer patients in German ICUs. The aim of this work is to collect and evaluate the prevalence and care data of cancer patients on two reference dates. METHODS: German ICUs were invited to participate in a 2-day, prospective, multicenter point prevalence study in ICU cancer patients. Participation in the study was vo...
Source: Hamostaseologie - July 30, 2019 Category: Hematology Authors: Kochanek M, Shimabukuro-Vornhagen A, Rüß K, Beutel G, Lueck C, Kiehl M, Schneider R, Kroschinsky F, Liebregts T, Kluge S, Schellongowski P, von Bergwelt-Baildon M, Böll B Tags: Med Klin Intensivmed Notfmed Source Type: research

A Sardinian Family with Factor XI Deficiency.
CONCLUSION:  A mixing test of a prolonged aPTT should be performed because it will be present both in patients with or without the inhibitor. A molecular analysis in severe FXI deficiency is warranted as it may have prognostic significance. CWA may be helpful for better understanding the pathophysiology of this kind of defect. PMID: 31364091 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2019 Category: Hematology Authors: Barcellona D, Favuzzi G, Vannini ML, Piras SM, Ruberto MF, Grandone E, Marongiu F Tags: Hamostaseologie Source Type: research

A Retrospective Analysis of Clinical and Laboratory Data of Patients with Factor VII Deficiency: A Single Centre Experience.
In conclusion, the clinical picture of the analysed group is similar to the previously described. It should be stressed that a significant risk of bleeds, including joint haemorrhages, can be expected in patients with mildest FVII deficiency (>26%) as well. The rate of thrombosis (4.7%) was similar to other reports, with all affected patients having strong prothrombotic risk factors or being intensively treated with FVII concentrate. PMID: 31364092 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2019 Category: Hematology Authors: Iwaniec T, Zdziarska J, Musiał J, Sanak M Tags: Hamostaseologie Source Type: research

Replacement Therapy in Patients with Von Willebrand Disease-Indications and Monitoring.
The objective of this review is to provide recommendations for specific constellations of replacement therapy based on the VWD-specific guidelines available in Europe, the available evidence, own experiences and the consensus of the interdisciplinary German author group. PMID: 31364093 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2019 Category: Hematology Authors: Nowak-Göttl U, Miesbach W, Koscielny J, Dempfle CE, Maegele M, Prondzinski MVD, Westrup D, Spannagl M Tags: Hamostaseologie Source Type: research

Genotype/phenotype correlations of childhood-onset congenital sideroblastic anaemia in a European cohort.
This study emphasizes the frequency of ALAS2 and SLC25A38 mutations and provides the largest comprehensive analysis to date of genotype/phenotype correlations in CSA. Further studies of CSA patients with data recorded in an international registry would be helpful to improve patient management and establish standardized guidelines. PMID: 31338833 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 23, 2019 Category: Hematology Authors: Fouquet C, Le Rouzic MA, Leblanc T, Fouyssac F, Leverger G, Hessissen L, Marlin S, Bourrat E, Fahd M, Raffoux E, Vannier JP, Jäkel N, Knoefler R, Triolo V, Pasquet M, Bayart S, Thuret I, Lutz P, Vermylen C, Touati M, Rose C, Matthes T, Isidor B, Kannengi Tags: Br J Haematol Source Type: research

First-Line Therapy for Immune Thrombocytopenia.
Abstract Immune thrombocytopenia (ITP) is an autoimmune disease affecting blood platelets that causes thrombocytopenia and an increased risk of bleeding. First-line therapy is indicated for patients with bleeding complications or who are at increased risk of bleeding, and the decision to initiate therapy depends not only on the platelet count, but also on other endpoints including quality of life. The choice of first-line therapy depends primarily on how quickly a platelet count response is required, with intravenous immune globulin providing the more rapid response, followed by high-dose dexamethasone and prednis...
Source: Hamostaseologie - June 6, 2019 Category: Hematology Authors: Mithoowani S, Arnold DM Tags: Hamostaseologie Source Type: research

Immunomodulatory Second-Line Therapies for Immune Thrombocytopenia.
Abstract Management of immune thrombocytopenia (ITP) is complex requiring communication between patients and caregivers to establish a mutual understanding of the impact of the patient's disease on quality of life, the current symptoms and risk of morbidity/mortality and the goals of therapy. The currently available second-line therapies for ITP provide potential for management of thrombocytopenia and bleeding symptoms with medical therapy or surgical intervention potentially offering long-term remission. All therapies are associated with potential side effects and necessary monitoring or modifications/risks and c...
Source: Hamostaseologie - June 5, 2019 Category: Hematology Authors: Lambert MP Tags: Hamostaseologie Source Type: research

The Prominent Pioneering 'Plateleteer': Reflections on and Personal Reminiscences of Gustav V. R. Born (1921-2018).
Abstract Among the pioneers in platelet research, Gustav Born was perhaps the most prominent representative, just a real "plateleteer". He achieved scientific fame for the invention and application of light transmission aggregometry. Importantly, he paved the way for contemporary antiplatelet therapy. Integrating his fundamental knowledge of platelet biology into the pathology of vascular disorders, Born provided seminal contributions to the understanding of atherogenesis and thrombogenesis. He also generated visions for pharmacological strategies that, nowadays, are translated into reality. Inside and o...
Source: Hamostaseologie - May 27, 2019 Category: Hematology Authors: Scharf RE Tags: Hamostaseologie Source Type: research

Coagulation Signalling and Metabolic Disorders: Lessons Learned from Animal Models.
Abstract Nutrient excess in obesity drives metabolic reprogramming in multiple tissues involving extensive interorgan and intercellular crosstalk. Experimental and clinical studies show that prolonged nutrient excess often compromises metabolic adaptation propagating proobesogenic and proinflammatory responses. Chronic inflammation further promotes insulin resistance and associated comorbidities. Obesity and type 2 diabetes are characterized by a hypercoagulable state and clinical studies show a strong correlation of markers of coagulation activation in metabolic disorders. Coagulation protease-dependent signallin...
Source: Hamostaseologie - May 27, 2019 Category: Hematology Authors: Madhusudhan T, Ruf W Tags: Hamostaseologie Source Type: research

[Modern treatment of deep vein thrombosis and pulmonary embolism].
Abstract Virchow's triad has been known for a 100 years. The development of therapeutic possibilities during this time was enormous. Today anticoagulant therapy is much more differentiated. Four new oral substances have replaced the traditional treatment with vitamin K antagonists in angiology. A standardized dosage is available. The monitoring of the coagulation parameters is no longer necessary, but it is important to monitor renal function. Direct oral anticoagulants are approved for the treatment of venous thrombosis and pulmonary embolism, but not during pregnancy or in children. Severe bleedin...
Source: Hamostaseologie - May 22, 2019 Category: Hematology Authors: Renczes J, Lindhoff-Last E Tags: Internist (Berl) Source Type: research

[Leitlinie der Gesellschaft f ür Thrombose- und Hämostaseforschung (GTH) zur Struktur- und Prozessqualität von Hämophilie-Zentren].
[Leitlinie der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) zur Struktur- und Prozessqualität von Hämophilie-Zentren]. Hamostaseologie. 2019 Apr 24;: Authors: Eichler H, Pedroni MA, Halimeh S, Königs C, Langer F, Miesbach W, Oldenburg J, Scholz U, Streif W, Klamroth R PMID: 31018219 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - April 24, 2019 Category: Hematology Authors: Eichler H, Pedroni MA, Halimeh S, Königs C, Langer F, Miesbach W, Oldenburg J, Scholz U, Streif W, Klamroth R Tags: Hamostaseologie Source Type: research

[Vitamin  D and omega-3 fatty acid supplementation does not reduce the cancer and cardiovascular risk].
[Vitamin D and omega-3 fatty acid supplementation does not reduce the cancer and cardiovascular risk]. Strahlenther Onkol. 2019 Apr 18;: Authors: Lordick F PMID: 31001644 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - April 18, 2019 Category: Hematology Authors: Lordick F Tags: Strahlenther Onkol Source Type: research

Clot Signature Curve Analysis as a Useful Tool of Thrombin Time Measurement in Case of Failed Optical Reading.
PMID: 30921818 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - March 28, 2019 Category: Hematology Authors: Kozłowski P, Ciepiela O Tags: Hamostaseologie Source Type: research

Fulminant Essential Thrombocythemia Associated with Acquired Von Willebrand Syndrome and Bleeding Episodes in a 14-year-old Girl.
w A Abstract BACKGROUND:  Essential thrombocythemia is a chronic myeloproliferative neoplasm. It is extremely rare in children below 15 years of age with an estimated annual incidence of only 0.09 per million. Usually, clinical symptoms associated with essential thrombocythemia are mild or absent. CASE:  Here, we present the case of a 14-year-old female patient fulminantly presenting with acute symptoms comprising visual impairment, palmar and plantar stabbing pain. Blood count revealed massive thrombocytosis of 2373 × 109/L. Bone marrow morphology showed elevated numbers of matu...
Source: Hamostaseologie - March 20, 2019 Category: Hematology Authors: Schneider C, Stutz-Grunder E, Lüer S, Keller P, Kremer Hovinga JA, Ammann RA, Karow A Tags: Hamostaseologie Source Type: research

Management of Helicobacter pylori in Patients with Immune Thrombocytopenia.
Abstract There is an association between Helicobacter pylori infection and immune thrombocytopenia (ITP), and few studies have suggested that eradicative treatment of H. pylori infection may improve platelet counts in patients with ITP. Conventional treatments for ITP include immunosuppressive agents, and more recently thrombopoietic agents. However, based on clinical reports of association between H. pylori and ITP, several medical societies increasingly suggest detection and eradication of H. pylori as a treatment for ITP. In this article, we reappraise recent medical literature to determine the effectiveness of...
Source: Hamostaseologie - March 19, 2019 Category: Hematology Authors: Vanegas YAM, Vishnu P Tags: Hamostaseologie Source Type: research

Epidemiology and Clinical Manifestations of Immune Thrombocytopenia.
Abstract Immune thrombocytopenia (ITP) occurs with an incidence rate of 1.6 to 3.9 per 100,000 patient-years, which increases with age and has a slight female preponderance. ITP is termed acute, persistent or chronic when its duration is 12 months, respectively. In this narrative review, we discuss the clinical manifestations of ITP in children and adults. ITP is asymptomatic in some patients; however, when present, bleeding is the most common symptom and can be mild as in petechiae, purpura and epistaxis, or severe and even life threatening in cases of intracranial haemorrhage, and massive gastrointestinal or uri...
Source: Hamostaseologie - March 13, 2019 Category: Hematology Authors: Kohli R, Chaturvedi S Tags: Hamostaseologie Source Type: research

[Meningococcal sepsis without cerebrospinal fluid abnormalities under treatment with eculizumab].
Abstract Infections with Neisseria meningitidis are life-threatening conditions, generally presenting as meningitis. This case of a young woman who had a history of paroxysmal nocturnal hemoglobinuria under treatment with the complement inhibitor eculizumab had been presented with septic shock. While blood cultures were positive for Neisseria meningitidis, she showed no evidence for bacterial meningitis in the cerebrospinal fluid. This case shows that meningococcal sepsis without signs for bacterial meningitis despite repetitive vaccinations is possible in adults under eculizumab. PMID: 30848313 [PubMed ...
Source: Hamostaseologie - March 8, 2019 Category: Hematology Authors: Jentzsch M, Schwind S, Vucinic V, Wendt S, Stingu CS, Niederwieser D, Weidhase L Tags: Med Klin Intensivmed Notfmed Source Type: research

IWR-1 Inhibits Collagen-Induced Platelet Activation and Protects against Thrombogenesis.
Abstract Platelets play a crucial role in haemostasis and several pathophysiological processes. Collagen is a main initiator for platelet activation and aggregation. Given that Wnt signalling negatively regulates platelet function, and IWR-1 (a small molecule inhibitor for Wnt signalling) has the potential of inhibiting collagen synthesis, it is essential to investigate whether IWR-1 regulates collagen-induced platelet activation and protects against thrombogenesis. In the present study we found that IWR-1 pretreatment effectively suppressed collagen-induced platelet aggregation in a dose-dependent manner. In addi...
Source: Hamostaseologie - March 8, 2019 Category: Hematology Authors: Wang W, Lai S, Xiao Z, Yan H, Li Y, Zhang Y, Wu Y, Wang L Tags: Hamostaseologie Source Type: research

Diagnosing Immune Thrombocytopenia.
Abstract Although the detection of a characteristic autoantibody can prove immune thrombocytopenia (ITP), this diagnosis is often based on the exclusion of other causes of thrombocytopenia. Direct glycoprotein (GP)-specific tests have the property required to demonstrate such a characteristic autoantibody. In contrast, platelet-associated immunoglobulin G or antibody detection in plasma or serum is an insufficient diagnostic test. Moreover, data for commercial capture assays are sparse and their use is currently not recommended. A significant drawback of direct GP-specific tests is their low sensitivity, and a neg...
Source: Hamostaseologie - February 14, 2019 Category: Hematology Authors: Sachs UJ Tags: Hamostaseologie Source Type: research

Current Trend of Invasive Orthopaedic Interventions for People with Haemophilia in Romania: Single Centre Experience.
CONCLUSION:  Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations. PMID: 30759481 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - February 13, 2019 Category: Hematology Authors: Serban M, Poenaru DV, Patrascu JM, Ritli L, Boia E, Mihailov DM, Ursu CE, Savescu D, Ionita I, Traila A, Cerbu S, Boeriu E, Jinca C, Schramm W, Arghirescu S Tags: Hamostaseologie Source Type: research

Prevalence of Obesity in Young Patients with Severe Haemophilia and Its Potential Impact on Factor VIII Consumption in Germany.
In this study in seven German haemophilia centres, we identified a prevalence of overweight and obesity of 25.2% among 254 young patients
Source: Hamostaseologie - February 5, 2019 Category: Hematology Authors: Olivieri M, Königs C, Heller C, Horneff S, Oldenburg J, Halimeh S, Kentouche K, Knöfler R, Fischer L, Pfrepper C, Kurnik K, Bidlingmaier C Tags: Hamostaseologie Source Type: research

Potential of Multidimensional, Large-scale Biodatabases to Elucidate Coagulation and Platelet Pathways as an Approach towards Precision Medicine in Thrombotic Disease.
Abstract Cardiovascular and especially thrombotic diseases remain a major cause of morbidity and mortality worldwide. In past years, significant improvements in understanding disease processes, risk assessment, and prediction of clinical outcome in the field of thrombosis and haemostasis have been made by using large-scale biodatabases. These important research resources enable a comprehensive research approach by integrating clinical, environmental, genomic, and molecular information. Cutting edge, high throughput technologies open new data dimensions for clinical large-scale investigations. Joining multiple info...
Source: Hamostaseologie - February 5, 2019 Category: Hematology Authors: Panova-Noeva M, Eggebrecht L, Prochaska JH, Wild PS Tags: Hamostaseologie Source Type: research

Laboratory Diagnostics in Thrombophilia.
Abstract A thrombophilic disorder is a hereditary or acquired condition that increases the risk of thrombosis. The most common hereditary thrombophilias that predispose to venous thrombosis in the Caucasian population are the heterozygous forms of the factor V Leiden and prothrombin G20210A mutation that are generally detected by direct DNA genotyping. Immunologic antigen assays and chromogenic or clot-based activity assays are used to identify deficiencies in the natural coagulation inhibitors antithrombin, protein C and protein S. Because pre-analytical errors and acquired causes of low antithrombin, protein C o...
Source: Hamostaseologie - January 31, 2019 Category: Hematology Authors: Linnemann B, Hart C Tags: Hamostaseologie Source Type: research

Trauma-Induced Coagulopathy.
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 pathogenesis. TIC can be modified by hypoperfusion, acidosis, hypothermia, haemodilution, underlying disease conditions, pre-injury medications as well as genetic predispositions. Current definition of this syndrome is based on laboratory abnormalities that do not easily allow a distinction between ad...
Source: Hamostaseologie - January 31, 2019 Category: Hematology Authors: Petros S Tags: Hamostaseologie Source Type: research

Low-Molecular-Weight Heparin in Cancer Patients: Overview and Indications.
Abstract Although venous thromboembolism (VTE) is a well-known cause of death in patients with cancer, both its treatment and prevention remain a challenge in daily practice. Direct oral anticoagulants have emerged as safe and efficacious alternatives to vitamin K antagonists in the general population, and recent clinical trials also support their use in select patients with cancer-associated VTE. Despite this, low-molecular-weight heparins (LMWHs), a comparatively ancient class of antithrombotic drugs, remain the anticoagulants of choice in many indications relevant to modern haematology and oncology. In addition...
Source: Hamostaseologie - January 31, 2019 Category: Hematology Authors: Voigtlaender M, Langer F Tags: Hamostaseologie Source Type: research

Antibodies in the Treatment of Haemophilia A-A Biochemical Perspective.
ng PJ Abstract Replacement therapy has been proven effective in the management of bleedings in haemophilia A. Nevertheless, this approach comes with several shortcomings, like the need for frequent intravenous infusions and the development of neutralizing antibodies in 20 to 30% of the patients with severe haemophilia A replacement. This has led to the development of novel strategies to expand the spectrum of treatment options, some of which are based on antibody technology. These include a bispecific antibody that bridges enzyme factor IXa and substrate factor X, monoclonal antibodies that block the function of t...
Source: Hamostaseologie - January 29, 2019 Category: Hematology Authors: Ferrière S, Lenting PJ Tags: Hamostaseologie Source Type: research

Impact of Adopting Population Pharmacokinetics for Tailoring Prophylaxis in Haemophilia A Patients: A Historically Controlled Observational Study.
CONCLUSION:  Implementing tailored prophylaxis using a Bayesian forecasting approach in a routine clinical practice setting may improve haemophilia clinical outcomes. PMID: 30685872 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - January 27, 2019 Category: Hematology Authors: Stemberger M, Kallenbach F, Schmit E, McEneny-King A, Germini F, Yeung CHT, Edginton AN, von Mackensen S, Kurnik K, Iorio A Tags: Thromb Haemost Source Type: research

Child Abuse or Bleeding Disorder-An Interdisciplinary Approach.
t U Abstract Children with an unexplained bleeding tendency are frequently referred to a haemostaseologist for further evaluation. Careful standardized history taking and clinical evaluation should allow for distinguishing bleeds after minor injury and trauma which are very common in all children. However, in two groups of children bleeding symptoms may be more significant than expected: those with an underlying coagulation disorder and those who have been subjected to physical child abuse. The coexistence of child abuse and a bleeding disorder must always be considered. An extended coagulation diagnostic is requi...
Source: Hamostaseologie - January 25, 2019 Category: Hematology Authors: Knöfler R, Streif W, Watzer-Herberth I, Hahn G, Schmidt U Tags: Hamostaseologie Source Type: research

The risk of infections in multiple myeloma before and after the advent of novel agents: a 12-year survey.
l M Abstract Infections represent a major cause of morbidity and mortality in multiple myeloma and are linked to both therapy- and disease-related factors. Although it has been suggested that the rate of infections increased since the introduction of novel agents, controversies still exist. To better assess the risk factors associated with infections in the era of novel agents, we conducted a large retrospective analysis of 479 myeloma patients treated at Jena University Hospital over a period of 12 years. During their disease history, 65% of patients developed at least one infection, and 37% of therapies wer...
Source: Hamostaseologie - January 24, 2019 Category: Hematology Authors: Brioli A, Klaus M, Sayer H, Scholl S, Ernst T, Hilgendorf I, Scherag A, Yomade O, Schilling K, Hochhaus A, Mügge LO, von Lilienfeld-Toal M Tags: Ann Hematol Source Type: research

Birth Control Pills and Thrombotic Risks: Differences of Contraception Methods with and without Estrogen.
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 gestodene. This is also true for nonoral combined hormonal contraceptives. The risk of newer COCs containing estradiol instead of EE is not clear due to missing data. Progestin-only hormonal contraception is not associated with a significant increase of the risk for VTE with the exception of depot...
Source: Hamostaseologie - January 22, 2019 Category: Hematology Authors: Rott H Tags: Hamostaseologie Source Type: research

Impact of Telemedicine Tools on Record Keeping and Compliance in Haemophilia Care.
CONCLUSION:  e-Diaries' use enables improved recording of information about patients' home treatment and bleeding episodes. Enhanced compliance with therapy may be a further benefit. PMID: 30654393 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - January 17, 2019 Category: Hematology Authors: Banchev A, Goldmann G, Marquardt N, Klein C, Horneff S, Langenkamp R, Frankenberger T, Oldenburg J Tags: Hamostaseologie Source Type: research

Thrombopoietin Receptor Agonist Use for Immune Thrombocytopaenia.
Abstract Management of patients with corticosteroid-refractory immune thrombocytopaenia (ITP) possesses a significant challenge to practitioners. Until recently, options included splenectomy and immunosuppression. With improved knowledge of both thrombopoiesis and the pathophysiology of ITP, novel drug development with thrombopoietin-receptor agonists (TPO-RAs) was undertaken. Two agents, romiplostim and eltrombopag, are currently approved for use in patients with chronic ITP. Both agents have been shown to increase the platelet count, improve health-related quality of life and reduce bleeding symptoms and concomi...
Source: Hamostaseologie - January 15, 2019 Category: Hematology Authors: Neunert CE Tags: Hamostaseologie Source Type: research

[Diagnosis and treatment of kidney involvement in plasma cell diseases : Renal involvement in multiple myeloma and monoclonal gammopathies].
CONCLUSION: The spectrum of renal manifestations in plasma cell dyscrasia has been expanded, particularly since the introduction of the term MGRS. Diagnostic and therapeutic management remain an interdisciplinary challenge. PMID: 30635666 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - January 11, 2019 Category: Hematology Authors: Gödecke V, Schmidt JJ, Bräsen JH, Koenecke C, Haller H Tags: Internist (Berl) Source Type: research

Microvascular Thrombosis and Ischaemic Limb Losses in Critically Ill Patients.
Abstract Relatively little scientific attention has been given to the small subset of critically ill patients with circulatory shock who develop ischaemic limb losses (symmetrical peripheral gangrene [SPG]). The clinical picture consists of acral (distal extremity) tissue necrosis involving lower limbs in a largely symmetrical fashion and with detectable arterial pulses; in one-third of patients the upper extremities are also affected (potential for four-limb amputations). The laboratory picture includes thrombocytopenia, coagulopathy, and normoblastemia (circulating nucleated red blood cells). The explanation for...
Source: Hamostaseologie - January 9, 2019 Category: Hematology Authors: Warkentin TE Tags: Hamostaseologie Source Type: research

Smart Medication ™, an Electronic Diary for Surveillance of Haemophilia Home Care and Optimization of Resource Distribution.
This report describes the technical features and potential advantages of the application of electronic haemophilia treatment diary smart medication ™ and an evaluation of real-life electronic treatment data collected from haemophilia patients. Since 2012, a total of 663 patients from 30 German haemophilia treatment centres (HTCs) have used the device. Data of nine HTCs were merged for real-life data analysis. Patients were divided into four subgroups according to above versus below mean values for annual factor consumption (AFC) and annual joint bleeds (AJB), respectively. The largest subgroup comprised patients with...
Source: Hamostaseologie - January 8, 2019 Category: Hematology Authors: Mondorf W, Eichler H, Fischer R, Holstein K, Klamroth R, Nimtz-Talaska A, Wermes C, Richter H, Severin K Tags: Hamostaseologie Source Type: research

Role of Endothelial Cells in Acute and Chronic Thrombosis.
r K Abstract Haemostasis encompasses a set of strictly regulated actions, such as vasoconstriction, platelet activation and blood coagulation. Endothelial cells play a crucial role in all of these processes and are an integral part of the vascular response to injury resulting in thrombus formation. Healthy endothelium expresses mediators to prevent platelet activation, including prostacyclin and nitric oxide, and to inhibit coagulation, such as thrombomodulin or RNase1. Upon activation, endothelial cells expose von Willebrand factor, integrins and other receptors to interact with activated platelets, erythrocytes ...
Source: Hamostaseologie - January 8, 2019 Category: Hematology Authors: Bochenek ML, Schäfer K Tags: Hamostaseologie Source Type: research

The Role of Low Molecular Weight Heparins for Venous Thromboembolism Prevention in Medical Patients-What Is New in 2019?
Abstract Low molecular weight heparins and fondaparinux have been the cornerstones for prevention of venous thromboembolism (VTE) in acutely ill medical patients for almost two decades. Guidelines recommend the use of these parenteral anticoagulants for 6 to 14 days but advise against extended-duration thromboprophylaxis after hospital discharge because no compelling scientific evidence has been provided for pharmacological prophylaxis beyond hospital stay. Five large randomized clinical trials, one with low molecular weight heparin and four with nonvitamin K antagonist oral anticoagulants, have failed to show sig...
Source: Hamostaseologie - January 8, 2019 Category: Hematology Authors: Haas S Tags: Hamostaseologie Source Type: research

Six versus eight doses of rituximab in patients with aggressive B cell lymphoma receiving six cycles of CHOP: results from the "Positron Emission Tomography-Guided Therapy of Aggressive Non-Hodgkin Lymphomas" (PETAL) trial.
In conclusion, adding two doses of rituximab to six cycles of R-CHOP did not improve outcome in patients with aggressive B cell lymphomas and a fast metabolic treatment response. PMID: 30610279 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - January 4, 2019 Category: Hematology Authors: Hüttmann A, Rekowski J, Müller SP, Hertenstein B, Franzius C, Mesters R, Weckesser M, Kroschinsky F, Kotzerke J, Ganser A, Bengel FM, La Rosée P, Freesmeyer M, Höffkes HG, Hertel A, Behringer D, Prange-Krex G, Griesshammer M, Holzinger J, Wilop S, Kro Tags: Ann Hematol Source Type: research

Thromboinflammation and Vascular Dysfunction.
Abstract Thromboinflammation and vascular dysfunction are inseparably combined with the innate and the adaptive immune response. While the role of this interplay has gained considerable attention in the arena of atherosclerosis/atherothrombosis and in deep vein thrombosis, its role in other forms of vascular disease and risk factors is currently emerging. In this brief review, we will focus on thromboinflammation with regard to cytokine signalling as well as on the novel role of a vascular coagulation-inflammatory circuit in arterial hypertension. PMID: 30513535 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - December 4, 2018 Category: Hematology Authors: Karbach S, Lagrange J, Wenzel P Tags: Hamostaseologie Source Type: research

Functional Classification of Paediatric Patients with Non-syndromic Delta-Storage Pool Deficiency.
Abstract Storage pool disease (SPD) covers a group of platelet defects in which α- and/or delta-granules are reduced or cannot be secreted adequately in response to agonists. The detection of delta-granule release defects is hampered by a lack of fast and feasible tests. We aimed to implement a flow cytometry-based kinetic mepacrine assay to better identify and subgroup childhood patients with a mild to moderate bleeding diathesis and compare our method to established laboratory tests. We analysed 50 children with suspected SPD whose initial parameters were re-assessed in a second site visit. Mepacrine uptak...
Source: Hamostaseologie - November 21, 2018 Category: Hematology Authors: Manukjan G, Eilenberger J, Andres O, Schambeck C, Eber S, Schulze H Tags: Hamostaseologie Source Type: research

The Gut Microbiota as an Influencing Factor of Arterial Thrombosis.
Abstract The mutualistic gut microbiota does not only impact the development and function of various immune cell types, but it also influences the function of the hepatic vascular endothelium and prothrombotic platelet function. With germ-free mouse models, we have demonstrated that gut-derived microbial-associated molecular patterns could stimulate hepatic von Willebrand factor (VWF) synthesis and plasmatic VWF levels through Toll-like receptor-2 (TLR2), thus defining the extent of platelet deposition to the subendothelial matrix of the ligation-injured common carotid artery. In addition to the microbiota-derived...
Source: Hamostaseologie - November 20, 2018 Category: Hematology Authors: Reinhardt C Tags: Hamostaseologie Source Type: research