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Article ; Online: Add-on erythropoietin in autoimmune hemolytic anemia.

Moser, Miriam M / Jilma, Bernd

2024 Volume 8, Issue 5, Page(s) 1320–1321

MeSH term(s)	Humans ; Anemia, Hemolytic, Autoimmune/drug therapy ; Bone Marrow ; Erythropoietin/therapeutic use
Chemical Substances	Erythropoietin (11096-26-7)
Language	English
Publishing date	2024-03-12
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	2915908-8
ISSN	2473-9537 ; 2473-9529
ISSN (online)	2473-9537
ISSN	2473-9529
DOI	10.1182/bloodadvances.2023012297
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Article ; Online: Comparison Evaluation of Automated Nucleated Red Blood Cell Enumeration by Sysmex XN 1000 in Comparison With Microscopic Reference in Children Under 1 Year.

Brunner-Ziegler, Sophie / Jilma, Bernd / Grimm, Gabriele / Jilma-Stohlawetz, Petra

Journal of clinical laboratory analysis

2024 , Page(s) e25037

Abstract: Background: In newborns, elevated nucleated red blood cell (NRBC) levels can be associated with enhanced erythropoietic stress and might be predictive for adverse outcome. Also, the presence of NRBC in peripheral blood might lead to erroneous ... ...

Abstract	Background: In newborns, elevated nucleated red blood cell (NRBC) levels can be associated with enhanced erythropoietic stress and might be predictive for adverse outcome. Also, the presence of NRBC in peripheral blood might lead to erroneous enumeration results of white blood cells in automated hematology analyzers. We aimed to assess the comparability of the Sysmex XN 1000 to manual slide reviews and correlation of NRBC with inflammation markers. Methods: Specimens of 3397 children under 1 year were compared by automated and microscopic NRBC enumeration. Additionally, potential correlations between NRBC and age and inflammation markers were examined. Results: Overall, there was good correlation (r = 0.97) between automated (range: 0%-3883%) and microscopic enumeration (range: 0%-3694%) of NRBC with high comparability up to a NRBC value of 200% and an increase in the variation between the two methods with increasing NRBC numbers. When 94 samples with ≤ 200% NRBC and ≥ 30% divergence between methods were separately reanalyzed with respect to overlapping cell populations in their scattergrams, Sysmex would have generated unrecognized incorrect automated results in 47 samples, corresponding to 1.4% of total study samples. NRBC counts were negatively correlated to age, but not to inflammation markers. Conclusion: Sysmex XN 1000 is highly precise in the enumeration of NRBC in children under 1 year up to counts of 200% and might replace time-intense manual counting in routine diagnostics. In the setting of neonatal and intensive care diagnostics, microscopic control and supervision of scattergrams are highly recommended for any automated NRBC enumeration processes.
Language	English
Publishing date	2024-04-15
Publishing country	United States
Document type	Journal Article
ZDB-ID	645095-7
ISSN	1098-2825 ; 0887-8013
ISSN (online)	1098-2825
ISSN	0887-8013
DOI	10.1002/jcla.25037
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Zs.A 2471: Show issues

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Article ; Online: Progress in von Willebrand Disease Treatment: Evolution towards Newer Therapies.

Moser, Miriam M / Schoergenhofer, Christian / Jilma, Bernd

Seminars in thrombosis and hemostasis

2024

Abstract: von Willebrand disease (VWD) is a very heterogenous disease, resulting in different phenotypes and different degrees of bleeding severity. Established therapies (i.e., desmopressin, antifibrinolytic agents, hormone therapy for heavy menstrual bleeding, ... ...

Abstract	von Willebrand disease (VWD) is a very heterogenous disease, resulting in different phenotypes and different degrees of bleeding severity. Established therapies (i.e., desmopressin, antifibrinolytic agents, hormone therapy for heavy menstrual bleeding, and von Willebrand factor [VWF] concentrates) may work in some subtypes, but not in all patients. In recent years, progress has been made in improving the diagnosis of VWD subtypes, allowing for more specific therapy. The impact of VWD on women's daily lives has also come to the fore in recent years, with hormone therapy, tranexamic acid, or recombinant VWF as treatment options. New treatment approaches, including the replacement of lacking factor VIII (FVIII) function, may work in those subgroups affected by severe FVIII deficiency. Reducing the clearance of VWF is an alternative treatment pathway; for example, rondaptivon pegol is a VWFA1 domain-binding aptamer which not only improves plasma VWF/FVIII levels, but also corrects platelet counts in thrombocytopenic type 2B VWD patients. These approaches are currently in clinical development, which will be the focus of this review. In addition, half-life extension methods are also important for the improvement of patients' quality of life. Targeting specific mutations may further lead to personalized treatments in the future. Finally, a few randomized controlled trials, although relatively small, have been published in recent years, aiming to achieve a higher level of evidence in future guidelines.
Language	English
Publishing date	2024-02-08
Publishing country	United States
Document type	Journal Article
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0044-1779485
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Article ; Online: Clinical pharmacology of antiplatelet drugs.

Gelbenegger, Georg / Jilma, Bernd

Expert review of clinical pharmacology

2022 Volume 15, Issue 10, Page(s) 1177–1197

Abstract: Introduction: Platelets play a key role in arterial thrombosis and antiplatelet therapy is pivotal in the treatment of cardiovascular disease. Current antiplatelet drugs target different pathways of platelet activation and show specific pharmacodynamic ... ...

Abstract	Introduction: Platelets play a key role in arterial thrombosis and antiplatelet therapy is pivotal in the treatment of cardiovascular disease. Current antiplatelet drugs target different pathways of platelet activation and show specific pharmacodynamic and pharmacokinetic characteristics, implicating clinically relevant drug-drug interactions. Areas covered: This article reviews the role of platelets in hemostasis and cardiovascular thrombosis, and discusses the key pharmacodynamics, drug-drug interactions and reversal strategies of clinically used antiplatelet drugs. Expert opinion: Antiplatelet therapies target distinct pathways of platelet activation: thromboxane A
MeSH term(s)	Humans ; Platelet Aggregation Inhibitors/adverse effects ; ST Elevation Myocardial Infarction/drug therapy ; Drug Therapy, Combination ; Clopidogrel ; Thrombosis ; Purinergic P2Y Receptor Antagonists
Chemical Substances	Platelet Aggregation Inhibitors ; Clopidogrel (A74586SNO7) ; Purinergic P2Y Receptor Antagonists
Language	English
Publishing date	2022-09-11
Publishing country	England
Document type	Review ; Journal Article
ISSN	1751-2441
ISSN (online)	1751-2441
DOI	10.1080/17512433.2022.2121702
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Article ; Online: Population-based incidence of all-cause anaphylaxis and its development over time: a systematic review and meta-analysis.

Pühringer, Vanessa / Jilma, Bernd / Herkner, Harald

Frontiers in allergy

2023 Volume 4, Page(s) 1249280

Abstract: Introduction: It is extremely difficult to compare studies investigating the frequency of anaphylaxis making it challenging to satisfactorily assess the worldwide incidence rate. Using a systematic review and meta-analysis, this publication aims to ... ...

Abstract	Introduction: It is extremely difficult to compare studies investigating the frequency of anaphylaxis making it challenging to satisfactorily assess the worldwide incidence rate. Using a systematic review and meta-analysis, this publication aims to determine the current incidence of all-cause anaphylaxis worldwide. Additionally, we investigated whether the incidence of anaphylaxis has changed over time and which factors influence the rates determined by individual studies. Methods: A literature search was performed in four databases. All articles that reported relevant information on population-based incidence rates of all-cause anaphylaxis were included. The protocol was published on INPLASY, the International Platform of Registered Systematic Review and Meta-analysis Protocols. Results: The database query and screening process resulted in 46 eligible articles on anaphylaxis. The current incidence worldwide was found to be approximately 46 cases per 100,000 population per year (95% CI 21-103). Evaluating confounding factors showed that studies using allergy clinics and hospitalizations as data source result in comparably low rates. Moreover, children are less prone to develop anaphylaxis compared to the general population. Using a random effects Poisson model we calculated a yearly increase of anaphylaxis incidence by 7.4% (95% CI 7.3-7.6, Discussion: This seems to be the first approach to analyze every reported all-cause anaphylaxis incidence rate until 2017 for an at most accurate determination of its epidemiology. Based on these results, future research could investigate the underlying causes for the rising incidence in order find ways to decrease the condition's frequency. Systematic review registration: inplasy.com, identifier [INPLASY202330047].
Language	English
Publishing date	2023-12-12
Publishing country	Switzerland
Document type	Journal Article ; Review
ISSN	2673-6101
ISSN (online)	2673-6101
DOI	10.3389/falgy.2023.1249280
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Article ; Online: Monoclonal antibodies for treatment of cold agglutinin disease.

Gelbenegger, Georg / Berentsen, Sigbjørn / Jilma, Bernd

Expert opinion on biological therapy

2023 Volume 23, Issue 5, Page(s) 395–406

Abstract: Introduction: Cold agglutinin disease (CAD) is a difficult-to-treat autoimmune hemolytic anemia and B cell lymphoproliferative disorder associated with fatigue, acrocyanosis, and a risk of thromboembolic events. Cold-induced binding of autoantibody ... ...

Abstract	Introduction: Cold agglutinin disease (CAD) is a difficult-to-treat autoimmune hemolytic anemia and B cell lymphoproliferative disorder associated with fatigue, acrocyanosis, and a risk of thromboembolic events. Cold-induced binding of autoantibody agglutinates red blood cells and triggers the classical complement pathway, leading to predominantly extravascular hemolysis. Areas covered: This review summarizes clinical and experimental antibody-based treatments for CAD and analyzes the risks and benefits of B cell and complement directed therapies, and discusses potential future treatments for CAD. Expert opinion: Conventional treatment of CAD includes a B cell targeted treatment approach with rituximab, yielding only limited treatment success. The addition of a cytotoxic agent (e.g. bendamustine) increases efficacy, but this is accompanied by an increased risk of neutropenia and infection. Novel complement directed therapies have emerged and were shown to have good efficacy against hemolysis and safety profiles but are expensive and unable to address circulatory symptoms. Complement inhibition with sutimlimab may be used as a bridging strategy until B cell directed therapy with rituximab takes effect or continued indefinitely if needed. Future antibody-based treatment approaches for CAD involve the further development of complement directed antibodies, a combination of rituximab and bortezomib, and daratumumab. Non-antibody based prospective treatments may include the use of Bruton tyrosine kinase inhibitors.
MeSH term(s)	Humans ; Anemia, Hemolytic, Autoimmune/drug therapy ; Anemia, Hemolytic, Autoimmune/diagnosis ; Rituximab/therapeutic use ; Antibodies, Monoclonal/adverse effects ; Hemolysis ; B-Lymphocytes
Chemical Substances	Rituximab (4F4X42SYQ6) ; Antibodies, Monoclonal
Language	English
Publishing date	2023-05-15
Publishing country	England
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2052501-1
ISSN	1744-7682 ; 1471-2598
ISSN (online)	1744-7682
ISSN	1471-2598
DOI	10.1080/14712598.2023.2209265
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Zs.A 6094: Show issues

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Article: Progress in von Willebrand Disease Treatment: Evolution towards Newer Therapies

Moser, Miriam M. / Schoergenhofer, Christian / Jilma, Bernd

Seminars in Thrombosis and Hemostasis

(Celebrating 50 years of Seminars in Thrombosis and Hemostasis—Part IV)

2024

Series title	Celebrating 50 years of Seminars in Thrombosis and Hemostasis—Part IV
Abstract	von Willebrand disease (VWD) is a very heterogenous disease, resulting in different phenotypes and different degrees of bleeding severity. Established therapies (i.e., desmopressin, antifibrinolytic agents, hormone therapy for heavy menstrual bleeding, and von Willebrand factor [VWF] concentrates) may work in some subtypes, but not in all patients. In recent years, progress has been made in improving the diagnosis of VWD subtypes, allowing for more specific therapy. The impact of VWD on women's daily lives has also come to the fore in recent years, with hormone therapy, tranexamic acid, or recombinant VWF as treatment options. New treatment approaches, including the replacement of lacking factor VIII (FVIII) function, may work in those subgroups affected by severe FVIII deficiency. Reducing the clearance of VWF is an alternative treatment pathway; for example, rondaptivon pegol is a VWFA1 domain-binding aptamer which not only improves plasma VWF/FVIII levels, but also corrects platelet counts in thrombocytopenic type 2B VWD patients. These approaches are currently in clinical development, which will be the focus of this review. In addition, half-life extension methods are also important for the improvement of patients' quality of life. Targeting specific mutations may further lead to personalized treatments in the future. Finally, a few randomized controlled trials, although relatively small, have been published in recent years, aiming to achieve a higher level of evidence in future guidelines.
Keywords	von Willebrand disease ; efanesoctocog alfa ; rondaptivon pegol ; emicizumab ; von Willebrand factor
Language	English
Publishing date	2024-02-08
Publisher	Thieme Medical Publishers, Inc.
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0044-1779485
Database	Thieme publisher's database

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Zs.A 1259: Show issues

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Article ; Online: Methylnaltrexone to Reduce the Inhibitory Effects of Opioids on Drug Absorption.

Schoergenhofer, Christian / Jilma, Bernd

JACC. Cardiovascular interventions

2019 Volume 12, Issue 16, Page(s) 1550–1552

MeSH term(s)	Analgesics, Opioid ; Coronary Artery Disease ; Humans ; Morphine ; Naltrexone/analogs & derivatives ; Quaternary Ammonium Compounds ; Ticagrelor ; Treatment Outcome
Chemical Substances	Analgesics, Opioid ; Quaternary Ammonium Compounds ; methylnaltrexone (0RK7M7IABE) ; Naltrexone (5S6W795CQM) ; Morphine (76I7G6D29C) ; Ticagrelor (GLH0314RVC)
Language	English
Publishing date	2019-07-31
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	2452157-7
ISSN	1876-7605 ; 1936-8798
ISSN (online)	1876-7605
ISSN	1936-8798
DOI	10.1016/j.jcin.2019.06.029
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Zs.A 6669: Show issues

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Article: Drug-Drug Interactions in Patients with Acute Respiratory Distress Syndrome.

Bischof, Thorsten / Schaller, Christoph / Buchtele, Nina / Staudinger, Thomas / Ullrich, Roman / Kraft, Felix / Andersson, Marine L / Jilma, Bernd / Schoergenhofer, Christian

Pharmaceutics

2024 Volume 16, Issue 3

Abstract: Acute respiratory distress syndrome (ARDS) is a potential life-threatening, heterogenous, inflammatory lung disease. There are no data available on potential drug-drug interactions (pDDIs) in critically ill patients with ARDS. This study analyzed pDDIs ... ...

Abstract	Acute respiratory distress syndrome (ARDS) is a potential life-threatening, heterogenous, inflammatory lung disease. There are no data available on potential drug-drug interactions (pDDIs) in critically ill patients with ARDS. This study analyzed pDDIs in this specific cohort and aimed to investigate possible associations of coronavirus disease 2019 (COVID-19) as an underlying cause of ARDS and treatment with extracorporeal membrane oxygenation (ECMO) with the occurrence of pDDIs. This retrospective study included patients ≥18 years of age diagnosed with ARDS between January 2010 and September 2021. The Janusmed database was used for the identification of pDDIs. A total of 2694 pDDIs were identified in 189 patients with a median treatment duration of 22 days. These included 323 (12%) clinically relevant drug combinations that are best avoided, corresponding to a median rate of 0.05 per day. There was no difference in the number of pDDIs between COVID-19- and non-COVID-19-associated ARDS. In patients treated with ECMO, the rate of the most severely graded pDDIs per day was significantly higher compared with those who did not require ECMO. PDDIs occur frequently in patients with ARDS. On average, each patient may encounter at least one clinically relevant drug combination that should be avoided during their intensive care unit stay.
Language	English
Publishing date	2024-02-21
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527217-2
ISSN	1999-4923
ISSN	1999-4923
DOI	10.3390/pharmaceutics16030303
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Article ; Online: Capillaroscopic differences between primary Raynaud phenomenon and healthy controls indicate potential microangiopathic involvement in benign vasospasms.

Brunner-Ziegler, Sophie / Dassler, Eva / Müller, Markus / Pratscher, Marco / Forstner, Nikolaus Franz-Ferdinand Maria / Koppensteiner, Renate / Schlager, Oliver / Jilma, Bernd

Vascular medicine (London, England)

2024 Volume 29, Issue 2, Page(s) 200–207

Abstract: Background: For primary Raynaud phenomenon (PRP), an otherwise unexplained vasospastic disposition is assumed. To test the hypothesis of an additional involvement of distinct ultrastructural microvascular alterations, we compared the nailfold capillary ... ...

Abstract	Background: For primary Raynaud phenomenon (PRP), an otherwise unexplained vasospastic disposition is assumed. To test the hypothesis of an additional involvement of distinct ultrastructural microvascular alterations, we compared the nailfold capillary pattern of patients with PRP and healthy controls. Methods: A total of 120 patients with PRP (with a median duration of vasospastic symptoms of 60 [IQR: 3-120] months) were compared against 125 controls. In both groups, nailfold capillaroscopy was performed to record the presence of dilatations, capillary edema, tortuous capillaries, ramifications, hemorrhages, and reduced capillary density and to determine a semiquantitative rating score. Further, the capacity of finger skin rewarming was investigated by performing infrared thermography in combination with cold provocation. Results: Unspecific morphologic alterations were found in both, PRP, such as controls, whereby the risk for PRP was four times as high in the presence of capillary dilations (CI: 2.3-7.6) and five times as high if capillary density was reduced (CI: 1.9-13.5). Capillary density correlated with thermoregulatory capacity in both hands in the PRP group, but not in controls. In addition, a negative correlation between the microangiopathy score and the percentage degree of rewarming in both hands was found for patients with PRP only. Conclusion: We found specific differences within the microvascular architecture between patients with PRP and controls. As a conclusion, PRP may not be an entirely benign vasospastic phenomenon, but might be associated with subtle microcirculatory vasculopathy. In addition, we suggest that the implementation of a scoring system might serve as guidance in the diagnostic process at least of patients with long-standing PRP.
MeSH term(s)	Humans ; Microscopic Angioscopy ; Capillaries ; Microcirculation ; Raynaud Disease/diagnosis ; Vascular Diseases
Language	English
Publishing date	2024-02-09
Publishing country	England
Document type	Journal Article
ZDB-ID	1311628-9
ISSN	1477-0377 ; 1358-863X
ISSN (online)	1477-0377
ISSN	1358-863X
DOI	10.1177/1358863X231223523
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