keyword
https://read.qxmd.com/read/38385305/treatment-of-immune-mediated-thrombotic-thrombocytopenic-purpura-without-plasma-exchange
#21
JOURNAL ARTICLE
Marco Capecchi, Giada Gazzola, Pasquale Agosti, Pasqualina De Leo, Ilaria Mancini, Barbara Ferrari, Juri Alessandro Giannotta, Andrea Artoni, Flora Peyvandi
Not available.
February 22, 2024: Haematologica
https://read.qxmd.com/read/38360215/mechanism-underlying-severe-deficiency-of-plasma-adamts-13-activity-in-immune-thrombotic-thrombocytopenic-purpura
#22
JOURNAL ARTICLE
X Long Zheng
BACKGROUND: Immune-mediated thrombotic thrombocytopenic purpura is caused by autoantibodies against ADAMTS-13, a plasma enzyme that cleaves von Willebrand factor. However, the mechanism resulting in severe deficiency of plasma ADAMTS-13 activity remains controversial. OBJECTIVES: To determine the mechanism of autoantibody-mediated severe deficiency of plasma ADAMTS13 activity in immune-mediated thrombotic thrombocytopenic purpura. METHODS: Fluorescence resonance energy transfer-VWF73 was used to determine plasma ADAMTS-13 activity...
February 14, 2024: Journal of Thrombosis and Haemostasis: JTH
https://read.qxmd.com/read/38357229/thrombopoietin-receptor-agonists-use-and-risk-of-thrombotic-events-in-patients-with-immune-thrombocytopenic-purpura-a-systematic-review-and-meta%C3%A2-analysis-of-randomized-controlled-trials
#23
JOURNAL ARTICLE
Nan Shen, Jibing Qiao, Yazhou Jiang, Jingjing Yan, Rang Wu, Hanjun Yin, Suyue Zhu, Jianqin Li
Thrombopoietin receptor agonists (TPO-RAs) have a role in second-line immune thrombocytopenic purpura (ITP) treatment, binding to and activating thrombopoietin receptors on megakaryocyte membranes in the bone marrow. This promotes megakaryocyte maturation and increases platelet production. Despite a 2-6% incidence of thrombotic events during TPO-RA treatment, it remains uncertain whether TPO-RAs elevate thrombosis rates. A comprehensive search of electronic databases was conducted using the relevant search criteria...
March 2024: Biomedical Reports
https://read.qxmd.com/read/38353182/should-we-consider-caplacizumab-as-routine-treatment-for-acute-thrombotic-thrombocytopenic-purpura-an-expert-perspective-on-the-pros-and-cons
#24
REVIEW
Inés Gómez Seguí, María Eva Mingot Castellano, Cristina Pascual Izquierdo, Javier de la Rubia
INTRODUCTION: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening disorder. Caplacizumab has been the latest drug incorporated into the initial treatment of acute episodes, allowing for faster platelet recovery and a decrease in refractoriness, exacerbation, thromboembolic events, and mortality. However, caplacizumab is also associated with a bleeding risk and higher treatment costs, which prevent many centers from using it universally. AREAS COVERED: Studies that included iTTP and/or caplacizumab to date were selected for this review using PubMed and MEDLINE platforms...
2024: Expert Review of Hematology
https://read.qxmd.com/read/38311385/-acquired-thrombotic-thrombocytopenic-purpura-during-durvalumab-monotherapy-for-non-small-cell-lung-cancer
#25
JOURNAL ARTICLE
Shotaro Shimada, Kai Kuroiwa, Hinako Narita, Reiko Okamura, Yuka Uesugi, Yohei Sasaki, Megumi Watanuki, Nana Arai, Yukiko Kawaguchi, Shun Fujiwara, Koji Yanagisawa, Norimichi Hattori
Immune checkpoint inhibitor (ICI)-induced thrombocytopenias are rare immune-related adverse events (irAE), but ICI-related thrombotic thrombocytopenic purpura (TTP) is extremely rare. A 79-year-old woman with non-small cell lung cancer received maintenance therapy with the anti-human PD-L1 monoclonal antibody durvalumab. Four weeks after the last infusion, she developed overt TTP. Remission was achieved by plasma exchange and prednisolone, and the patient has now been recurrence-free for over 12 months. To our knowledge, this is the first report of TTP occurring as an irAE of durvalumab...
2024: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://read.qxmd.com/read/38282948/refractory-acquired-amegakaryocytic-thrombocytopenia-with-rapid-progression-to-aplastic-anaemia-in-sle
#26
Bana Hadid, Arif Kodza, Sumatha Channapatna Suresh, Aleksander Feoktistov
Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare cause of thrombocytopenia seen in systemic lupus erythematosus (SLE) that is frequently misdiagnosed as immune thrombocytopenic purpura (ITP). Often patients do not respond to standard ITP treatment. Prompt bone marrow biopsy and further workup should ensue as it is a diagnosis of exclusion. While no standard guidelines exist, the mainstay of treatment is immunosuppressive therapy. Some cases are refractory and should have a follow-up biopsy, typically showing worsening disease...
December 2023: Mediterranean journal of rheumatology
https://read.qxmd.com/read/38282441/a-review-on-romiplostim-mechanism-of-action-and-the-expressive-approach-in-e-coli
#27
REVIEW
Masoud Hashemzaei, Mohammad Bagher Ghoshoon, Mehrnaz Jamshidi, Fatemeh Moradbeygi, Ahmad Hashemzehi
Immune thrombocytopenic purpura (ITP) is an autoimmune disorder determined by immune-mediated platelet demolition and reduction of platelet production. Romiplostim is a new thrombopoiesis motivating peptibody that binds and stimulates the human thrombopoietin receptor the patent of which was registered in 2008. It is used to treat thrombocytopenia in patients with chronic immune thrombocytopenic purpura. Romiplostim is a 60 kDa peptibody designed to inhibit cross-reacting immune responses. It consists of four high-affinity TPO-receptor binding domains for the Mpl receptor and one human IgG1 Fc domain...
2024: Recent Patents on Biotechnology
https://read.qxmd.com/read/38281080/a-review-of-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura-past-present-and-future
#28
REVIEW
Dinah V Parums
The clinical association of purpura, arthralgia, and arthritis was first described in 1837 in a publication by Johann Lukas Schönlein, a German physician. In 1874, Eduard Henoch, a student of Schönlein, reported cases of children with purpura, abdominal pain, bloody diarrhea, and joint pain. IgA vasculitis, or Henoch-Schönlein purpura, is a systemic hypersensitivity vasculitis caused by the deposition of immune complexes in small blood vessels, including the renal glomeruli and mesangium. In the skin, the presentation is with non-thrombocytopenic purpura or urticaria...
January 28, 2024: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://read.qxmd.com/read/38268581/knowledge-map-of-thrombopoietin-receptor-agonists-a-bibliometric-analysis
#29
JOURNAL ARTICLE
Rong Hu, Songbin Guo, Min Liu
Thrombopoietin receptor agonists (TPO-RAs) have been widely used to treat thrombocytopenia, however, a scientometric profile of TPO-RAs research is lacking. Methods : This study uses VOSviewer, CiteSpace, and R software to provide an overview of current research, highlight study hotspots, and predict future research directions of TPO-RAs. Results: One thousand seven hundred and nineteen relevant studies from 1993 to 2022 with 43962 citations were identified from the Web of Science Core Collection. Over three decades, the USA has been leading TPO-RAs publications...
January 15, 2024: Heliyon
https://read.qxmd.com/read/38241567/recent-advances-in-the-management-of-immune-thrombocytopenic-purpura-itp-a-comprehensive-review
#30
REVIEW
Mohammed Ali Madkhali
Autoimmune disorders place a substantial burden on the healthcare system all over the world affecting almost 3% to 8% of the population. Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura, is a blood disorder in which the body immune system destroys platelets, leading to low platelet counts in the blood (peripheral blood platelet count < 150 × 109/L). Although the pathophysiology of ITP is not fully understood, it is believed to result from a complex interplay between hereditary and environmental variables...
January 19, 2024: Medicine (Baltimore)
https://read.qxmd.com/read/38237147/adamts13-recovery-in-acute-thrombotic-thrombocytopenic-purpura-after-caplacizumab-therapy-the-spanish-registry
#31
JOURNAL ARTICLE
Maria Eva Mingot-Castellano, Faustino García-Candel, Jorge Martinez Nieto, José García-Arroba Peinado, Javier de la Rubia Comos, Ines Gomez-Segui, María-Liz Paciello-Coronel, David Valcarcel, Moraima Jiménez, Joan Cid, Miquel Lozano, José-María García-Gala, Sonia Angós-Vazquez, Miriam Vara Pampliega, Luisa Guerra Dominguez, Laura-Francisca Ávila-Idrovo, Ana Oliva Hernandez, Saioa Zalba, Inmaculada Tallón Ruiz, Sandra Ortega Sánchez, Rosa Goterris, Maria Gemma Moreno Jimenez, Lourdes Domínguez-Acosta, María Araiz-Ramírez, Luis M Hernández-Mateo, Elena Flores Ballester, Julio Del Rio-Garma, Cristina Pascual Izquierdo
Caplacizumab prevents the interaction between von Willebrand factor and platelets, and is used to treat immune thrombotic thrombocytopenic purpura (iTTP). Its administration has been associated with a delay in ADAMTS13 activity restoration after plasma exchange (PEX) suspension. We analyzed the outcomes of 113 iTTP episodes, 75 of which were treated with caplacizumab, in 108 patients from the Spanish Registry of Thrombotic Thrombocytopenic Purpura. Caplacizumab shortened the time to platelet count normalization and reduced PEX requirement, exacerbations and relapses...
January 18, 2024: Blood
https://read.qxmd.com/read/38235916/plasma-cell-directed-therapy-strategies-in-immune-mediated-thrombotic-thrombocytopenic-purpura-ittp
#32
REVIEW
Püsem Patır, Nurtaç Önkibar, Sedef Subari, Ahmet Emre Eşkazan
No abstract text is available yet for this article.
January 18, 2024: Transfusion
https://read.qxmd.com/read/38234934/how-should-complicated-cases-of-thrombotic-thrombocytopenic-purpura-with-positive-coombs-test-be-treated
#33
Moutaz Ghrewati, Anas Mahmoud, Tala Beliani, Karam Zakharia, Mehandar Kumar
Thrombocytopenia with concomitant anemia is a serious condition with a high mortality risk. Destruction of platelets, i.e., thrombocytopenia, can be secondary to either auto-antibodies (immune-mediated) or mechanical destruction (non-immune-mediated). The Coombs test is a widespread tool to differentiate between the two categories, resulting in different specific treatment approaches for each diagnosis. A peripheral blood smear can also help make the diagnosis; for instance, in cases of mechanical destruction such as thrombotic thrombocytopenic purpura (TTP), the red blood cell (RBC) shape looks fragmented, forming schistocytes...
December 2023: Curēus
https://read.qxmd.com/read/38227934/antinuclear-antibodies-associated-auto-immune-cytopenia-in-childhood-is-a-risk-factor-for-systemic-lupus-erythematosus
#34
JOURNAL ARTICLE
Jérome Granel, Helder Fernandes, Brigitte Bader-Meunier, Amandine Guth, Olivier Richer, Pascal Pillet, Guy Leverger, Stéphane Ducassou, Mony Fahd, Marlène Pasquet, Nathalie Garnier, Vincent Barlogis, Corinne Guitton, Eric Jeziorski, Caroline Thomas, Sophie Bayart, Nathalie Cheikh, Catherine Paillard, Wadih Abou Chahla, Pascal Chastagner, Bénédicte Neven, Frédéric Millot, Julien Lejeune, Valérie Li Thiao Te, Corinne Armari-Alla, Claire Briandet, Liana Carausu, Marianna Deparis, Christophe Piguet, Joy Benadiba, Aude Marie-Cardine, Jean-Louis Louis Stephan, Isabelle Pellier, Claire Pluchart, Eric Doré, Katell Michaux, Sebastien Heritier, Thierry M Leblanc, Nathalie Aladjidi
Autoimmune cytopenia (AIC) in children may be associated with positive antinuclear antibodies (ANA) and may progress to systemic lupus erythematosus (SLE). We evaluated the risk of progression to SLE of childhood-onset ANA-associated AIC. In the French national prospective OBS'CEREVANCE cohort, the long-term outcome of children with ANA-associated AIC (ANA titer ≥ 1/160) and the subgroup of children who developed SLE were described. ANA were positive in 355/1803 (20%) children with AIC. With a median follow-up of 5...
January 16, 2024: Blood
https://read.qxmd.com/read/38205735/a-prospective-cohort-study-to-identify-clinical-diagnostic-and-prognostic-markers-of-primary-immune-thrombocytopenia-in-dogs
#35
JOURNAL ARTICLE
Marjory B Brooks, Robert Goggs, Amelia H Frye, Jessica Armato, Marnin Forman, Julia Hertl, Michael Koch, John P Loftus, John Lucy, Brandi Mattison, Julia Merriam, Sarah Shropshire, Laura Van Vertloo, Austin Viall, Dana N LeVine
BACKGROUND: Primary immune thrombocytopenia (pITP) in dogs presents a diagnostic challenge, and clinical markers of severity are lacking. OBJECTIVES: Identify clinicopathologic features that differentiate pITP from secondary ITP (sITP) and markers related to bleeding severity, transfusion, and survival of dogs with pITP. ANIMALS: Ninety-eight thrombocytopenic dogs (58 pITP and 40 sITP). METHODS: Client-owned dogs with platelet counts <50 000/μL were enrolled in a prospective, multi-institution cohort study...
January 11, 2024: Journal of Veterinary Internal Medicine
https://read.qxmd.com/read/38193904/immune-mediated-thrombotic-thrombocytopenic-purpura-don-t-miss-the-boat
#36
JOURNAL ARTICLE
Adrien Picod, Lara Zafrani, Elie Azoulay
No abstract text is available yet for this article.
January 9, 2024: Intensive Care Medicine
https://read.qxmd.com/read/38174163/a-successfully-treated-covid-19-vaccine-induced-immune-thrombocytopenic-purpura
#37
Miguel A Rodriguez Guerra, Siddharth Chinta, Ana P Urena Neme, Sorab Gupta, Gabriella Roa Gomez
Immune Thrombocytopenic Purpura (ITP) is a life-threatening condition where an accurate initial assessment is essential to be able to offer the proper therapy in a timely matter to improve the outcome of the patient. Here, we present a case of ITP secondary to the coronavirus disease 2019 (COVID-19) vaccine (BioNTech, Pfizer vaccine). A 61-year-old obese African American female presented to the emergency room (ER) from a clinic with a platelet count of 11k/ul 21 days after she received the second dose of the BioNTech, Pfizer vaccine...
December 2023: Curēus
https://read.qxmd.com/read/38168727/immune-mediated-thrombocytopenia-and-il-6-mediated-thrombocytosis-observed-in-idiopathic-multicentric-castleman-disease
#38
JOURNAL ARTICLE
Ayelet I Rubenstein, Sheila K Pierson, Saishravan Shyamsundar, Mateo Sarmiento Bustamante, Michael V Gonzalez, Ira D Milller, Joshua D Brandstadter, Melanie D Mumau, David C Fajgenbaum
Idiopathic multicentric Castleman disease (iMCD) is a rare haematological disorder characterized by generalized lymphadenopathy with atypical histopathological features and systemic inflammation caused by a cytokine storm involving interleukin-6 (IL-6). Three clinical subtypes are recognized: thrombocytopenia, anasarca, fever, renal dysfunction, organomegaly (iMCD-TAFRO); idiopathic plasmacytic lymphadenopathy (iMCD-IPL), involving thrombocytosis and hypergammaglobulinaemia; and iMCD-not otherwise specified (iMCD-NOS), which includes patients who do not meet criteria for the other subtypes...
January 2, 2024: British Journal of Haematology
https://read.qxmd.com/read/38168722/successful-management-of-refractory-immune-mediated-thrombotic-thrombocytopenic-purpura-during-pregnancy-and-delivery-using-the-anti-vwf-nanobody-caplacizumab
#39
JOURNAL ARTICLE
Roman R Schimmer, Tabea Sutter, Adrian Bachofner, Elisabetta Ranieri, Ann-Kathrin Rodewald, Johanna A Kremer Hovinga, Nina Kimmich, Alice Trinchero, Jan-Dirk Studt
Pregnancy is a potential trigger of acute thrombotic thrombocytopenic purpura (TTP). The management of pregnancy-associated immune-mediated TTP (iTTP) can be challenging, especially when it is refractory to standard treatment. Caplacizumab, a nanobody to von Willebrand factor (VWF) blocking its A1 domain, is a valuable new therapeutic option. Its use is, however, not approved during pregnancy and breastfeeding. We describe the successful off-label administration of caplacizumab during pregnancy and delivery in a patient with refractory iTTP...
January 2, 2024: British Journal of Haematology
https://read.qxmd.com/read/38160729/adamts-13-conformation-influences-autoimmune-recognition-in-immune-thrombotic-thrombocytopenic-purpura
#40
JOURNAL ARTICLE
Mary I Underwood, Mari R Thomas, Marie A Scully, James T B Crawley
BACKGROUND: Patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP) have anti-ADAMTS-13 immunoglobulin G (IgG) autoantibodies that enhance ADAMTS-13 clearance and/or inhibit its function. ADAMTS-13 normally circulates in a closed conformation, which is manifested by the interaction of the CUB domains with the central spacer domain. Disruption of the spacer-CUB interaction opens ADAMTS-13, which augments its proteolytic function but may also expose cryptic autoimmune epitopes that promote further autoantibody recognition...
December 30, 2023: Journal of Thrombosis and Haemostasis: JTH
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