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Jian Chen, Michael Losos, Shangbin Yang, Junan Li, Haifeng Wu, Spero Cataland
BACKGROUND: It has been shown that platelet transfusion carries a higher incidence of transfusion-related adverse events than any other blood components, and prolonged platelet storage is associated with more transfusion reactions, most of which are considered to be inflammatory responses. However, the role of complement, which has very important proinflammatory activities, in the pathogenesis of platelet-related adverse events has not been fully understood. STUDY DESIGN AND METHODS: Three units of platelets collected by apheresis were stored on a platelet rotator with the temperature controlled between 22 and 24°C...
September 2017: Transfusion
F Peyvandi, M Scully, J A Kremer Hovinga, P Knöbl, S Cataland, K De Beuf, F Callewaert, H De Winter, R K Zeldin
Essentials Acquired thrombotic thrombocytopenic purpura (aTTP) is linked with significant morbidity/mortality. Caplacizumab's effect on major thromboembolic (TE) events, exacerbations and death was studied. Fewer caplacizumab-treated patients had a major TE event, an exacerbation, or died versus placebo. Caplacizumab has the potential to reduce the acute morbidity and mortality associated with aTTP. SUMMARY: Background Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy...
July 2017: Journal of Thrombosis and Haemostasis: JTH
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with nondeficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
May 25, 2017: Blood
Shruti Chaturvedi, Olalekan Oluwole, Spero Cataland, Keith R McCrae
INTRODUCTION: Survivors of thrombotic thrombocytopenic purpura (TTP) have high rates of chronic morbidities including neurocognitive complications and depression. There is limited information regarding the psychological consequences of TTP. We conducted this cross sectional study to estimate the prevalence of symptoms of PTSD and depression in survivors of TTP. METHODS: An online survey tool comprising demographic and clinical information and two validated self-administered questionnaires, the PTSD checklist for DSM-5 (PCL-5) and Beck Depression Inventory-II (BDI-II), was distributed to individuals with TTP...
March 2017: Thrombosis Research
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates...
February 2017: Journal of Thrombosis and Haemostasis: JTH
Jeffrey L Turner, Joshua Reardon, Tanios Bekaii-Saab, Spero R Cataland, Matthew J Arango
No abstract text is available yet for this article.
September 20, 2016: Clinical Colorectal Cancer
A Kopić, K Benamara, C Piskernik, B Plaimauer, F Horling, G Höbarth, T Ruthsatz, B Dietrich, E-M Muchitsch, F Scheiflinger, M Turecek, W Höllriegl
UNLABELLED: Essentials ADAMTS-13-deficiency is a cause of thrombotic thrombocytopenic purpura (TTP). Preclinical safety of recombinant human ADAMTS-13 (BAX930) was shown in animal models. Preclinical efficacy of BAX930 was shown in a mouse model of TTP. BAX930 showed advantageous efficacy over fresh frozen plasma, the current standard of care. Click to hear Dr Cataland and Prof. Lämmle present a seminar on Thrombotic Thrombocytopenic Purpura (TTP): new Insights in Pathogenesis and Treatment Modalities...
July 2016: Journal of Thrombosis and Haemostasis: JTH
Katerina Pavenski, Spero Cataland, Johanna Kremer Hovinga, Mari Thomas, Karen Vanhoorelbeke
Answering TTP was established to engage the thrombotic thrombocytopenic purpura (TTP) community to further the common goals of patient support, education of patients and medical professionals, and the funding of research to improve the treatment and care of TTP patients. Answering TTP convened a dinner symposium on 23 June 2015 in Toronto, Canada during the International Society of Thrombosis and Haemostasis (ISTH) Congress to bring TTP clinicians and research together to highlight the key clinical and research questions in the field...
August 2016: Expert Review of Hematology
S Vasu, H Wu, A Satoskar, M Puto, J Roddy, W Blum, R Klisovic, L Andritsos, C Hofmeister, D M Benson, Y Efebera, S Jaglowski, S Penza, D Cohen, S Devine, S Cataland
No abstract text is available yet for this article.
September 2016: Bone Marrow Transplantation
Fadi Fakhouri, Maryvonne Hourmant, Josep M Campistol, Spero R Cataland, Mario Espinosa, A Osama Gaber, Jan Menne, Enrico E Minetti, François Provôt, Eric Rondeau, Piero Ruggenenti, Laurent E Weekers, Masayo Ogawa, Camille L Bedrosian, Christophe M Legendre
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare genetic life-threatening disease of chronic uncontrolled complement activation leading to thrombotic microangiopathy (TMA) and severe end-organ damage. Eculizumab, a terminal complement inhibitor approved for aHUS treatment, was reported to improve hematologic and renal parameters in 2 prior prospective phase 2 studies. This is the largest prospective study of eculizumab in aHUS to date, conducted in an adult population. STUDY DESIGN: Open-label single-arm phase 2 trial...
July 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Flora Peyvandi, Marie Scully, Johanna A Kremer Hovinga, Spero Cataland, Paul Knöbl, Haifeng Wu, Andrea Artoni, John-Paul Westwood, Magnus Mansouri Taleghani, Bernd Jilma, Filip Callewaert, Hans Ulrichts, Christian Duby, Dominique Tersago
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is caused by aggregation of platelets on ultralarge von Willebrand factor multimers. This microvascular thrombosis causes multiorgan ischemia with potentially life-threatening complications. Daily plasma exchange and immunosuppressive therapies induce remission, but mortality and morbidity due to microthrombosis remain high. METHODS: Caplacizumab, an anti-von Willebrand factor humanized single-variable-domain immunoglobulin (Nanobody), inhibits the interaction between ultralarge von Willebrand factor multimers and platelets...
February 11, 2016: New England Journal of Medicine
John Kincaid, Spero Cataland, Johan Vande Walle, Yahsou Delmas, Gianluigi Ardissino, Jimmy Wang
No abstract text is available yet for this article.
December 2015: Critical Care Medicine
John L Vaughn, Jared M Moore, Spero R Cataland
Complement-mediated hemolytic uremic syndrome (otherwise known as atypical HUS) is a rare disorder of uncontrolled complement activation that may be associated with heart failure. We report the case of a 49-year-old female with no history of heart disease who presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given her normal ADAMSTS13 activity, evidence of increased complement activation, and renal biopsy showing evidence of thrombotic microangiopathy, she was diagnosed with complement-mediated HUS...
2015: Case Reports in Hematology
S R Cataland, H M Wu
Advances in our understanding of the pathophysiology of both congenital and acquired thrombotic thrombocytopenic purpura (TTP) have led to both an increased understanding of the disease and novel approaches to therapy. The efficacy of rituximab in acquired TTP has led to consideration of rituximab as a prophylactic therapy to prevent relapse of TTP. Novel therapies that target the A1 domain of von Willebrand factor (VWF) to block the formation of microthrombotic disease have also entered clinical study and have demonstrated promise as potential therapeutic options...
June 2015: Journal of Thrombosis and Haemostasis: JTH
Jennifer N Saultz, Haifeng M Wu, Spero Cataland
Thrombotic thrombocytopenia purpura (TTP) and atypical hemolytic uremic syndromes (aHUS) are distinct clinical disorders characterized by hemolytic anemia, thrombocytopenia, microthrombi, and end organ damage. TTP is characterized by a low ADAMTS13 activity level at diagnosis of <10 % ADAMTS13 activity, while aHUS is characterized as having >10 % ADAMTS13 activity. Despite clinical remission, survivors of thrombotic microangiopathy suffer significant comorbidity and decreased quality of life (QOL) than their healthy counterparts...
September 2015: Annals of Hematology
Shangbin Yang, Michael McGookey, Yi Wang, Spero R Cataland, Haifeng M Wu
OBJECTIVES: Recent studies have shown that complement hyperactivation contributes to development of thrombotic microangiopathy. The evaluation of complement biomarkers is known to be influenced by inappropriate specimen handling. However, there has been no study fully addressing this topic. METHODS: Blood from each donor was subjected to 62 different handling conditions prior to complement assays. RESULTS: Complement biomarkers (C4d/C3a/factor Bb/C5a/C5b-9) are stable at room temperature (RT) for up to 4 hours in whole blood containing citrate or EDTA...
April 2015: American Journal of Clinical Pathology
Nan Wu, Jia Liu, Shangbin Yang, Eric T Kellett, Spero R Cataland, Hong Li, Haifeng M Wu
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) requires immediate treatment with plasma exchange (PE) to prevent disease mortality and/or morbidity. Frequently, PE is initiated before blood sample is collected to confirm ADAMTS13 deficiency. However, the effect of PE treatments on the evaluation of ADAMTS13 is uncertain. Moreover, the pertinence of ADAMTS13 activity during PE therapy to prediction of treatment outcomes is unclear. Thus, clarification of the diagnostic and prognostic values of ADAMTS13 activity obtained during PE treatment is an unmet clinical need...
January 2015: Transfusion
Spero R Cataland, V Michael Holers, Susan Geyer, Shangbin Yang, Haifeng M Wu
Atypical hemolytic uremic syndrome (aHUS) is characterized by dysregulated complement activity, the development of a thrombotic microangiopathy (TMA), and widespread end organ injury. aHUS remains a clinical diagnosis without an objective laboratory test to confirm the diagnosis. We performed a retrospective analysis of 103 patients enrolled in the Ohio State University TTP/aHUS Registry presenting with an acute TMA. Nineteen patients were clinically categorized as aHUS based on the following criteria: (1) platelet count <100 × 10(9)/L, (2) serum creatinine >2...
June 12, 2014: Blood
Spero R Cataland, Haifeng M Wu
Published data demonstrating the efficacy of complement inhibition therapy in patients with atypical hemolytic uremic syndrome (aHUS) are remarkable in contrast to the historically poor long-term prognosis for aHUS patients treated with plasma-based therapy. Although both aHUS and acquired thrombotic thrombocytopenic purpura (TTP) remain clinical diagnoses, an increased understanding of both conditions has improved our ability to differentiate aHUS from acquired TTP. These same data have also demonstrated the importance of a more rapid identification and diagnosis of aHUS as the recovery of end-organ injury present appears to be related to the time to initiate therapy with eculizumab...
April 17, 2014: Blood
Spero R Cataland, Haifeng M Wu
Historically, attempts were made to differentiate acquired thrombotic thrombocytopenic purpura (TTP) from atypical hemolytic uremic syndrome (aHUS) based upon the age at presentation and the presence of neurologic or renal injury. Although these means of differentiating acquired TTP from aHUS have now been demonstrated to be inaccurate, there were no clinical consequences as the treatment for both disorders remained plasma exchange therapy (PEX). With the regulatory approval and remarkable efficacy of eculizumab (Soliris) for the treatment of aHUS, the accurate and timely differentiation of acquired TTP from aHUS now has real clinical consequences...
March 2014: Blood Reviews
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