In this systematic review, investigators hypothesize that thrombopoietin receptor agonists (TPO-RAs) “influence platelet function, global and secondary hemostasis, and/or fibrinolysis.” After evaluating 12 observational studies comprising 305 patients, they report “[t]here were indications that TPO-RA treatment enhanced platelet function, with respect to platelet-monocyte aggregates, soluble P-selectin, GPVI [glycoprotein VI] expression, and adhesion under flow.”
This review from StatPearls (available on the National Center for Biotechnology Information online bookshelf), updated in late June, provides comprehensive information and clinical pearls related to the autoimmune condition known as Evans syndrome. As the authors explain, patients with Evans syndrome present with two or more cytopenias; these are commonly warm autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia.
This mini-review highlights discussions from the sixth annual meeting on ITP organized by the Intercontinental Cooperative ITP Study Group and held in September 2019 in Locarno, Switzerland. At the 2019 meeting, the authors explain, “scientists and clinicians of pediatric and adult hematology, oncology, immunology, and genetics shared their ideas, experiences, and research findings to critically assess open questions and launch future joint projects” to investigate “out of the box” concepts associated with ITP.
The International ITP Alliance was formed in 2016 by support organizations around the world, to give patients with immune thrombocytopenia a greater voice and promote public awareness of this autoimmune bleeding disorder. This year’s celebration in late September marks its sixth annual global event. The website provides an ITP Awareness Toolkit to help people spread information about ITP on Facebook, Twitter, and other social media. It also offers links to ITP information and resources; patient support organizations, by country; and resources related to COVID-19.
The FWGBD’s mission is to support education and awareness among women, adolescent girls, and their healthcare providers regarding female bleeding disorders, given the challenges that such disorders can pose during the menstrual cycle and at various stages of the female reproductive life cycle (eg, pregnancy, childbirth, menopause). The FWGBD’s website offers particularly comprehensive resources on ITP, including expert interviews, videos, webinars, Q&A, and COVID-19 resources for providers treating patients with ITP.
In this review, researchers from Dijon, France, highlight recent advances in the understanding of ITP pathogenesis that have broadened and deepened the opportunities for therapeutic intervention. Beyond describing multiple potentially promising pathways and targets now under investigation, they caution that “[T]ogether with this therapeutic progress, new questions have arisen, such as the place of each drug in the algorithm of ITP management, the safety of their long-term use and the possibility of synergistic combination. Moreover, the various response rates to all these therapies highlight the crucial need for biomarkers to tailor the treatment to patients and optimize their management.”
The authors of this study state that iWISh “focused on perceptions of 1,507 patients and 472 physicians from 13 countries regarding the diagnostic pathway, frequency and severity of signs and symptoms, and treatment use” related to ITP.
This January 2021 consensus statement was written by clinicians with an interest in coagulation disorders or ITP and was reviewed by members of the UK ITP forum. The ITP Support Association is a UK charity.
The Platelet Disorder Support Association (PDSA) was patient-founded in 1998 for patients with immune thrombocytopenia (ITP) and other platelet disorders. Resources of interest to healthcare providers include a newsroom; the updated international consensus report on management of primary ITP (Provan et al, Nov. 2019); and more than a dozen patient education pamphlets covering a wide range of topics—including the role and function of platelets in ITP, the female lifecycle and ITP, FAQs and lifestyle tips related to ITP, and information about health insurance assistance programs for patients with ITP. The PDSA website also provides up-to-date information about the ITP Natural History Study Patient Registry; the COVID-19 and ITP Research Survey; and relevant medical meetings, including the “ITP Conference 2021,” a virtual event that will occur on July 31 and August 1, 2021.
The Castleman Disease Collaborative Network is a patient advocacy organization founded in 2012 to advance research and treatment. Patients can read patient education materials; donate tissue samples; and enter their medical data into the ACCLERATE patient registry, to inform clinical research (after data de-identification). Resources for clinicians include access to recent studies, treatment toolkits and guidelines, and information about CDCN-funded research. In AIM 2021 (“All-in Movement” 2021), the CDCN Scientific Advisory Board will crowdsource and prioritize questions and ideas from Castleman’s disease community to guide future clinical trials.
These evidence-based guidelines on management of ITP were developed by a multidisciplinary panel formed by ASH in 2015 to evaluate 2011 ITP guidelines and formulate recommendations based on available evidence and recommendations through May 2017. ASH will maintain currency of the guidelines by ongoing review and surveillance.
This review article summarizes current knowledge about this complex disease, highlights research into targeted treatment, and discusses how characteristics of AIHA may vary in older adults, depending on their immunological status.
This review article explores putative mechanisms by which infection and inflammation can trigger and drive hemolysis in cold agglutinin disease, or CAD, and secondary cold agglutinin syndrome, or CAS, as well as recent advances in understanding the pathogenesis of these disorders. Novel targeted treatments such as complement-directed therapies are discussed.
In this multicenter review and consensus opinion, clinicians from the US and UK shed new light on the possible etiology of immune-mediated cytopenias, and offer management strategies for patients with inherited metabolic disorders who develop single or multilineage cytopenias following hematopoietic stem cell transplantation.