Article
Hematology
Wobke E. M. van Dijk, Geke C. Poolen, Albert Huisman, Harry R. Koene, Rob Fijnheer, Noortje Thielen, Esther R. van Bladel, Karin P. M. van Galen, Roger E. G. Schutgens, Rolf T. Urbanus
Summary: This study found that patients with ITP were in a hypercoagulable state, with preactivated platelets and increased thrombin generation potential. Eltrombopag treatment further increased plasma thrombin generation potential but had no significant impact on other hemostatic parameters.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Review
Hematology
Ilene Ceil Weitz, Howard Allen Liebman
Summary: Immune thrombocytopenia (ITP) is a disorder characterized by low platelets due to increased clearance and decreased platelet production. The interaction between platelet autoantibodies and the complement system plays an important role in ITP. A recent clinical trial has shown the efficacy of complement inhibitors in refractory ITP patients.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Review
Cell Biology
Simone Birocchi, Gian Marco Podda, Marco Manzoni, Giovanni Casazza, Marco Cattaneo
Summary: In patients with immune thrombocytopenia, thrombopoietin receptor agonists (TPO-RA) such as Romiplostim, Eltrombopag, and Avatrombopag have shown to be effective and safe, with lower treatment failure rates and less severe bleeding compared to control groups. Remission rates after discontinuation of treatment were around 18%, and adverse events were rare and similar in both TPO-RA treated patients and controls. The all-cause mortality was significantly lower in patients treated with TPO-RA.
Article
Hematology
Etienne Crickx, Mikael Ebbo, Etienne Riviere, Odile Souchaud-Debouverie, Louis Terriou, Sylvain Audia, Marc Ruivard, Bouchra Asli, Jean-Pierre Marolleau, Nadine Meaux-Ruault, Mathieu Gerfaud-Valentin, Philippe Audeguy, Mohamed Hamidou, Selim Corm, Xavier Delbrel, Jean Fontan, Delphine Lebon, Christelle Mausservey, Guillaume Moulis, Nicolas Limal, Marc Michel, Bertrand Godeau, Matthieu Mahevas
Summary: Combining drugs could effectively treat multirefractory ITP, with 77% of patients achieving a response. The combination regimen included a thrombopoietin receptor agonist and an immunosuppressive drug, and the median duration of treatment was 12 months. Severe adverse events related to ITP treatment were observed in 31% of patients.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Medicine, General & Internal
R. Aggarwal, C. Charles-Schoeman, J. Schessl, Z. Bata-Csorgo, M. M. Dimachkie, Z. Griger, S. Moiseev, C. Oddis, E. Schiopu, J. Vencovsky, I Beckmann, E. Clodi, O. Bugrova, K. Danko, F. Ernste, N. A. Goyal, M. Heuer, M. Hudson, Y. M. Hussain, C. Karam, N. Magnolo, R. Nelson, N. Pozur, L. Prystupa, M. Sardy, G. Valenzuela, A. J. van Der Kooi, T. Vu, M. Worm, T. Levine
Summary: This study evaluated the use of IVIG for the treatment of dermatomyositis in a randomized, placebo-controlled trial. The results showed that IVIG treatment resulted in a significant increase in the percentage of patients with at least minimal improvement in disease activity compared to placebo. However, IVIG treatment was associated with adverse events, including thromboembolism.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Article
Hematology
Chandana Mareddy, Manas Kalra, Anupam Sachdeva
Summary: We evaluated the response, efficacy, and safety of a generic form of romiplostim manufactured in India for treating children with persistent/chronic immune thrombocytopenia (ITP). The study showed that the generic romiplostim is well tolerated and efficacious in children with persistent/chronic ITP.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Pharmacology & Pharmacy
Kensaku Nakai, Takuya Misugi, Kohei Kitada, Yasushi Kurihara, Mie Tahara, Akihiro Hamuro, Akemi Nakano, Masayasu Koyama, Yukimi Kira, Daisuke Tachibana
Summary: This study examined the effects of romiplostim, a form of thrombopoietin receptor agonist, on pregnant mice. The results showed that romiplostim increased platelet and megakaryocyte counts in pregnant mice and fetuses at doses above 10 mu g/kg. However, the clinically used dose for ITP patients did not have any adverse effects on pregnant mice.
Article
Chemistry, Analytical
Xiaoying Wang, Tao Gao, Jin Zhu, Shipeng Long, Songyan Zhao, Li Yuan, Zhaoxia Wang
Summary: Hematogenous metastasis is the main cause of cancer-related deaths, and platelets play a crucial role in promoting this process. A new channeled and conductive 3D electrode was constructed to capture and detect platelet interacted circulating tumor cells (CTCs). The modified antibody allowed for the capture of platelet-interacted CTC hybrids, while the conductive layer facilitated electron transfer for sensitive electrochemical detection. This work provides an efficient method for simultaneous capture and detection of invasive CTCs, with the potential to monitor hematogenous metastasis accurately.
ANALYTICAL CHEMISTRY
(2023)
Article
Medicine, General & Internal
Tamer Hassan, Doaa Abdel Rahman, Nermin Raafat, Manar Fathy, Mohamed Shehab, Ahmed Hosny, Reham Fawzy, Marwa Zakaria
Summary: Concepts regarding the mechanisms of thrombocytopenia in ITP have shifted, and it is now believed that impaired platelet production, T-cell-mediated effects, and disturbed cytokine profiles play a role. IL-27 plays an important role in the pathogenesis of childhood ITP and can be used as a predictor of disease occurrence and response to treatment.
Review
Chemistry, Medicinal
Maria Eva Mingot-Castellano, Jose Maria Bastida, Gonzalo Caballero-Navarro, Laura Entrena Urena, Tomas Jose Gonzalez-Lopez, Jose Ramon Gonzalez-Porras, Nora Butta, Mariana Canaro, Reyes Jimenez-Barcenas, Maria Del Carmen Gomez Del Castillo Solano, Blanca Sanchez-Gonzalez, Cristina Pascual-Izquierdo
Summary: Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by low platelet counts and bleeding risk. Current treatments have limitations, and therefore a thorough review of novel therapies is necessary. The pathophysiology of ITP is complex, but it also presents an opportunity to uncover new treatment targets.
Editorial Material
Hematology
James B. B. Bussel
Summary: The use of intravenous anti-D for treating ITP has been largely abandoned due to the risk of intravascular hemolysis. Intramuscular delivery of anti-D could be a safer approach and needs further evaluation. IV anti-D was widely used for ITP treatment in the United States in the 1990s until the discovery of its risk for intravascular hemolysis. As a result, it is now rarely used due to the availability of alternative treatments. IM anti-D, on the other hand, does not carry the risk of intravascular hemolysis and should be reconsidered as an option for certain adult patients with ITP.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Critical Care Medicine
George A. Alba, Andriy O. Samokhin, Rui-Sheng Wang, Ying-Yi Zhang, Bradley M. Wertheim, Elena Arons, Edward A. Greenfield, Martina H. Lundberg Slingsby, Julia R. Ceglowski, Kathleen J. Haley, Frederick P. Bowman, Yen-Rei Yu, John C. Haney, George Eng, Richard N. Mitchell, Anthony Sheets, Sara O. Vargas, Sachiko Seo, Richard N. Channick, Peter J. Leary, Sudarshan Rajagopal, Joseph Loscalzo, Elisabeth M. Battinelli, Bradley A. Maron
Summary: The study identified the modifiable protein-protein interaction between NEDD9 and P-selectin as a potential target to inhibit platelet-pulmonary endothelial adhesion and thromboembolic vascular remodeling under hypoxia conditions. Custom anti-NEDD9 antibody effectively inhibited platelet adhesion to pulmonary artery endothelial cells and reduced chronic pulmonary thromboembolic remodeling in vivo.Therefore, the NEDD9-P-selectin interaction may have therapeutic implications for patients with disorders characterized by increased hypoxia signaling pathways, such as CTEPH.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Cell Biology
Tyler E. James, Leslie J. Martin, Theodore E. Warkentin, Mark A. Crowther
Summary: Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by sudden multiorgan dysfunction caused by thrombosis from antibodies. Treatment includes anticoagulation, corticosteroids, TPE, and IVIG. This case report is the first to document initial failure of IVIG treatment in a CAPS patient with subsequent success with TPE.
Article
Hematology
Lydie Crescence, Roxane Darbousset, Eva Caroff, Francis Hubler, Markus A. Riederer, Laurence Panicot-Dubois, Christophe Dubois
Summary: Through real-time intravital microscopy in mice, it was found that compared to clopidogrel and ticagrelor, Selatogrel has fewer platelet thrombus formations, better stability of hemostatic seals, and does not interfere with endothelial activation, neutrophil function, and fibrin-mediated stabilization of hemostatic seals.
THROMBOSIS RESEARCH
(2021)
Article
Immunology
Pedro Rosa-Guerrero, Antonio Trujillo-Aguilera, Juan Molina, Ana Navas, Cristina Lopez-Martin, Aurora Jurado, Alberto Rodriguez-Benot, Alvaro Torres-De-Rueda
Summary: Transplanted populations under chronic immunosuppression are more severely impacted by Covid-19 pneumonia caused by SARS-CoV-2. Treatment poses a major challenge for physicians, but the combination of high-dose IVIG and steroid pulses has shown success in reducing inflammation levels, improving symptoms, and gradually restoring allograft function in a severe case.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Hematology
John Grainger, James Bussel, Michael Tarantino, Nichola Cooper, Donald Beam, Jenny Despotovic, Alexey Maschan, Kejia Wang, Melissa Eisen, Charles Bowers
Summary: This study investigated the long-term efficacy and safety of romiplostim in children with immune thrombocytopenia (ITP) for at least 6 months. The results showed that romiplostim can maintain platelet counts within the desired range and achieve platelet responses for a significant proportion of the treatment period. The use of romiplostim in the long term resulted in sustained platelet responses and demonstrated a consistent safety profile.
Article
Hematology
Andrew J. Doyle, Matthew J. Stubbs, Tina Dutt, Will Lester, Will Thomas, Joost van Veen, Joannes Hermans, Tanya Cranfield, Quentin A. Hill, Amanda Clark, Catherine Bagot, Steven Austin, John -Paul Westwood, Mari Thomas, Marie Scully
Summary: Relapse is a risk for patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP) after acute treatment, but the identification of patients at risk and relapse patterns is not well established. This study in the UK followed iTTP patients for over 3 years to identify characteristics for relapse, assess relapse rates and patterns, and evaluate response to anti-CD20 therapy for ADAMTS13 relapses. Results showed a relapse rate of 40% after 5 years, with no difference observed at 10-year follow-up. Black Caribbean ethnicity increased the risk of relapse. Early relapses occurred in 6% of patients, with subsequent relapses within 2 years. ADAMTS13 relapses accounted for nearly 60% of all relapses, and treatment reduced the risk of progression to clinical relapses. Regular monitoring and preemptive rituximab led to lower rates of clinical relapses in later diagnosed iTTP cases. Anti-CD20 therapy was effective for ADAMTS13 relapses and maintained treatment response after subsequent episodes.
Article
Cardiac & Cardiovascular Systems
Ingrid Bekono-Nessah, Alex Rosenburg, Christopher T. Bowles, Fernando Riesgo-Gil, Ulrich Stock, Richard R. Szydlo, Mike Laffan, Deepa J. Arachchillage
Summary: Major bleeding and thrombosis significantly increase the 1-year mortality in HVAD patients. Optimal perioperative hemostasis and anticoagulation remain crucial for HVAD patients.
Article
Hematology
Hithin Noble, Christina Crossette-Thambiah, Zain Odho, Nilanthi Karawitage, Karen Logan, Charis Pericleous, Mike Laffan, Deepa J. Arachchillage
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2023)
Article
Hematology
Alexander Roeth, Catherine M. Broome, Wilma Barcellini, Bernd Jilma, Quentin A. Hill, David Cella, Tor Henrik Anderson Tvedt, Masaki Yamaguchi, Michelle Lee, Frank Shafer, Marek Wardecki, Xiaoyu Jiang, Parija Patel, Florence Joly, Ilene C. Weitz
Summary: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia that leads to fatigue and reduced quality of life. In the Phase 3 CADENZA trial, treatment with the complement inhibitor sutimlimab showed rapid improvement in hemolysis, increased hemoglobin levels, and alleviated fatigue in CAD patients. Patient-reported outcomes also demonstrated significant benefits from sutimlimab treatment.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2023)
Review
Cell Biology
Nichola Cooper, Waleed Ghanima, Quentin A. Hill, Phillip Lr Nicolson, Vadim Markovtsov, Craig Kessler
Summary: Spleen tyrosine kinase (SYK) is a critical regulatory molecule in autoimmune diseases, and SYK inhibitors, such as fostamatinib, have shown promising results in the treatment of autoimmune thrombocytopenia (ITP) and other diseases. SYK inhibitors have the potential to target multiple signaling pathways and may be effective in diseases associated with inflammation, autoimmunity, and blood cancers, including COVID-19.
Article
Hematology
Deepa Arachchillage, Indika Rajakaruna, Zain Odho, Mike Makris, Mike Laffan
Summary: The controversy of post-discharge thromboprophylaxis in COVID-19 patients remains. This observational study across 26 NHS Trusts aimed to determine the impact of thromboprophylaxis on hospital acquired thrombosis (HAT) in discharged patients. The study found no difference in HAT between patients discharged with and without thromboprophylaxis, but increasing age and smoking significantly increased the risk of HAT.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Letter
Hematology
Waleed Ghanima, Quentin A. Hill, David J. Kuter
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Hematology
Florence Joly, Pronabesh DasMahapatra, Dana B. DiBenedetti, Katherine Kosa, Quentin A. Hill
Summary: This study aimed to identify important symptoms and impacts related to CAD and develop a CAD-specific patient-reported outcome measure. Through concept elicitation and cognitive debriefing interviews, the researchers identified 11 final items for the CAD-Symptoms and Impact Questionnaire.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2023)
Article
Hematology
Mike Laffan, Gary Benson, Cathy Farrelly, Keith Gomez, April Jones, Rhona Maclean, James O'Donnell, Michelle Lavin
Summary: Von Willebrand Disease (VWD) is a common inherited bleeding disorder, but awareness and understanding of the disease lags behind other bleeding disorders, causing delays in diagnosis and treatment. Updated national guidelines are needed to improve the management of VWD and provide equitable care for both men and women. Through a survey and analysis conducted by a panel of experts, eight recommendations were derived to enhance the detection and management of VWD.
Review
Hematology
Karina Yazdanbakhsh, Drew Provan, John W. Semple
Summary: Immune thrombocytopenia (ITP) is a disease characterized by abnormal immune response against platelets, affecting their destruction and production. Abnormal T-cells and myeloid-derived suppressive cells (MDSCs) play important roles in ITP pathogenesis. Different treatments have diverse effects on T-cells and MDSCs in ITP. Identifying factors and mechanisms for predicting treatment response remains challenging.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Medicine, General & Internal
Tomas Jose Gonzalez-Lopez, Drew Provan
Summary: TPO-RAs have shown high efficacy rates (59-88%), durable responses (up to three years), and a satisfactory safety profile in clinical trials. Some studies have reported the possibility of successfully discontinuing TPO-RAs without further treatment. We propose a protocol for successfully tapering and discontinuing TPO-RAs in daily clinical practice.
MEDICINA-LITHUANIA
(2023)
Article
Hematology
Deepa J. Arachchillage, Anna Weatherill, Indika Rajakaruna, Mihaela Gaspar, Zain Odho, Graziella Isgro, Lenka Cagova, Lucy Fleming, Stephane Ledot, Mike Laffan, Richard Szydlo, Rachel Jooste, Ian Scott, Alain Vuylsteke, Hakeem Yusuff
Summary: Thrombosis and major bleeding are common complications in V-V ECMO patients and significantly increase mortality. Age and elevated creatinine level are associated with increased mortality, while the type of thrombosis has different effects on mortality.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Meeting Abstract
Hematology
Deepa J. Arachchillage, Indika Rajakaruna, Mike Laffan, Richard Szydlo, Zain Odho, Ian Scott, Stephane Ledot, Alain Vuylsteke, Hakeem Yusuff
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Meeting Abstract
Hematology
Giulia Simini, Saravanan Vinayagam, Nilanthi Karawitage, Golzar Mobayen, Anupama Krishnamoorthi, Andrew James Innes, Michael Laffan, Deepa Jayakody Arachchillage
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
