acquired Thrombotic thrombocytopenic purpura

A blood disorder characterized by low PLATELETS (THROMBOCYTOPENIA), small areas of bleeding under the skin (PURPURA), low red blood cell count, and HEMOLYTIC ANEMIA, causing blood clots to form in small blood vessels throughout the body.

Also Known As:

Networked: 337 relevant articles (23 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombotic Thrombocytopenic Purpura
2.	Acute Disease
3.	COVID-19
4.	Thrombotic Microangiopathies
5.	Hemolytic Anemia

Experts

1.	Matsumoto, Masanori: 16 articles (06/2022 - 09/2002)
2.	Coppo, Paul: 16 articles (01/2021 - 03/2010)
3.	Peyvandi, Flora: 14 articles (01/2021 - 08/2005)
4.	Cataland, Spero R: 13 articles (01/2019 - 09/2005)
5.	Kremer Hovinga, Johanna A: 12 articles (01/2021 - 01/2004)
6.	George, James N: 11 articles (01/2022 - 11/2007)
7.	Veyradier, Agnès: 11 articles (11/2020 - 01/2012)
8.	Voorberg, Jan: 10 articles (11/2020 - 09/2006)
9.	Wu, Haifeng M: 10 articles (01/2015 - 09/2005)
10.	Fujimura, Yoshihiro: 9 articles (08/2016 - 03/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to acquired Thrombotic thrombocytopenic purpura:

1.	thiamine triphosphorate (TTP)IBA 01/01/2019 - "In patients treated for acquired thrombotic thrombocytopenic purpura (TTP), a persistently severe ADAMTS13 deficiency (<10%) in remission is associated with more relapses. " 07/10/2014 - "In acquired thrombotic thrombocytopenic purpura (TTP), the persistence of severe ADAMTS13 deficiency (<10%) during remission is associated with more relapse. " 04/17/2014 - "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. " 01/01/2023 - "The outcomes and characteristics of acquired thrombotic thrombocytopenic purpura (TTP) in adolescents is poorly understood due to an absence of studies focused on this population. " 02/01/2021 - "The aim of this study was to explore the clinical characteristics and treatment of acquired thrombotic thrombocytopenic purpura (TTP). "
2.	Rituximab (Mabthera)FDA Link 01/01/2019 - "Comparison of the Long-Term Remission of Rituximab and Conventional Treatment for Acquired Thrombotic Thrombocytopenic Purpura: A Systematic Review and Meta-Analysis." 06/01/2017 - "Objective To investigate the efficacy and safety of rituximab (RTX) as first-line treatment of acquired thrombotic thrombocytopenic purpura (aTTP). " 06/01/2017 - "Rituximab as first-line treatment for acquired thrombotic thrombocytopenic purpura." 07/10/2014 - "Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura." 10/01/2012 - "Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis."
3.	caplacizumabIBA 01/01/2020 - "Introduction of the nanobody caplacizumab was shown to be effective in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in the acute setting. " 01/01/2023 - "Commentary on: Odetola et al. Safe and effective use of caplacizumab in pregnancy-related acquired thrombotic thrombocytopenic purpura. " 01/01/2023 - "A safe and effective use of caplacizumab in pregnancy-related acquired thrombotic thrombocytopenic purpura." 06/01/2023 - "Caplacizumab was licensed for acquired thrombotic thrombocytopenic purpura (aTTP) based on prospective controlled trials. " 01/01/2021 - "The efficacy and safety of caplacizumab in individuals with acquired thrombotic thrombocytopenic purpura (aTTP) have been established in the phase 2 TITAN and phase 3 HERCULES trials. "
4.	von Willebrand FactorIBA 06/01/2012 - "The TITAN trial--assessing the efficacy and safety of an anti-von Willebrand factor Nanobody in patients with acquired thrombotic thrombocytopenic purpura." 09/01/2011 - "Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission." 06/01/2012 - "The Phase II TITAN trial is designed to assess the efficacy and safety of an anti-von Willebrand factor (vWF) Nanobody in patients with acquired thrombotic thrombocytopenic purpura (TTP). " 01/01/2023 - "[Caplacizumab in acquired thrombotic thrombocytopenic purpura: Dose adjustment based on von Willebrand factor level]." 09/01/2019 - "Loss of von Willebrand factor high-molecular-weight multimers at acute phase is associated with detectable anti-ADAMTS13 IgG and neurological symptoms in acquired thrombotic thrombocytopenic purpura."
5.	AntibodiesIBA 02/01/2008 - "ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission." 04/01/2021 - "In agreement with this observation, all the incubation methods permitted the accurate detection and quantification of inhibitory anti-ADAMTS13 antibodies in the samples from patients with acquired thrombotic thrombocytopenic purpura. " 03/17/2018 - "Antibodies anti-ADAMTS13 were positive, establishing the diagnosis of acquired thrombotic thrombocytopenic purpura. " 01/01/2016 - "Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura." 04/01/2014 - "Here, we analyzed a large cohort of patients with acquired thrombotic thrombocytopenic purpura for the presence of free and complexed anti-ADAMTS13 antibodies. "
6.	Peptide Hydrolases (Proteases)FDA Link 02/01/2008 - "Survivors of an acute episode of acquired thrombotic thrombocytopenic purpura with severely reduced levels of ADAMTS13 and/or with anti-ADAMTS13 antibodies during remission have an approximately three-fold greater likelihood of developing another episode of thrombotic thrombocytopenic purpura than patients with higher protease activity and no antibody." 09/01/2005 - "The demonstration that a severe deficiency of von Willebrand factor-cleaving protease activity in acquired thrombotic thrombocytopenic purpura is due to an autoantibody inhibitor of the von Willebrand factor-cleaving protease argues for targeting the antibody inhibitor of the von Willebrand factor-cleaving protease with immunosuppressive therapy as a means of increasing protease activity and inducing a sustained remission of the disease. " 12/01/2013 - "Acquired thrombotic thrombocytopenic purpura (TTP) is caused by a deficiency of von Willebrand factor-cleaving protease (ADAMTS13) and is often associated with the presence of an antibody inhibiting the activity of the protease. " 02/01/2017 - "The acquired form of idiopathic thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease, in which the underlying ADAMTS13-deficiency is caused by inhibitory autoantibodies against the protease. " 01/01/2016 - "Acquired thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease in which ultralarge von Willebrand factor (UL-VWF) multimers accumulate as a result of autoantibody inhibition of the VWF protease, ADAMTS13. "
7.	AutoantibodiesIBA 11/01/2020 - "Modifying ADAMTS13 to modulate binding of pathogenic autoantibodies of patients with acquired thrombotic thrombocytopenic purpura." 07/01/2020 - "Acquired thrombotic thrombocytopenic purpura is an autoimmune disorder caused by an inhibitory autoantibody against ADAMTS-13. " 01/01/2018 - "Background Acquired thrombotic thrombocytopenic purpura (aTTP) is an autoimmune disorder characterized by absent ADAMTS-13 activity and the presence of anti-ADAMTS-13 autoantibodies. " 01/01/2017 - "Acquired thrombotic thrombocytopenic purpura (aTTP) is caused by a deficiency of ADAMTS13 activity due to neutralizing auto-autoantibodies (inhibitors) against ADAMTS13. " 11/01/2016 - "Acquired thrombotic thrombocytopenic purpura is primarily caused by the deficiency of plasma ADAMTS13 activity resulting from autoantibodies against ADAMTS13. "
8.	ADAMTS13 ProteinIBA 04/01/2002 - "Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura." 06/01/1998 - "Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura." 09/01/2005 - "In the future, a better understanding of the relation between the von Willebrand factor-cleaving protease activity, the nature and titer of von Willebrand factor-cleaving protease inhibitors, and the risk of relapse of thrombotic thrombocytopenic purpura will be important to further validate the strategy of targeting the von Willebrand factor-cleaving protease inhibitory antibody as an effective means of treating acquired thrombotic thrombocytopenic purpura. " 05/01/2006 - "Unlike acquired thrombotic thrombocytopenic purpura or haemolytic uraemic syndrome, which are often intractable, thrombotic microangiopathy in patients with Upshaw-Schulman syndrome (USS)--a congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS13) activity--responds very well to plasma infusion and does not even require plasma exchange. " 04/01/2021 - "Acquired thrombotic thrombocytopenic purpura is a rare disease associated with the production of autoantibodies against the VWF-cleaving protease ADAMTS13. "
9.	Cyclosporine (Ciclosporin)FDA LinkGeneric 07/01/2013 - "Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature." 12/01/2008 - "Several reports have been published regarding the use of cyclosporine (CSA) in the treatment of idiopathic thrombotic thrombocytopenic purpura (TTP). " 11/01/2007 - "We hypothesized that cyclosporin (CSA) as adjunct to plasma exchange (PE) improves the efficacy of PE in idiopathic thrombotic thrombocytopenic purpura (TTP) via suppression of the antibody inhibitor of ADAMTS13. " 12/01/2008 - "Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura."
10.	Adrenal Cortex Hormones (Corticosteroids)IBA 07/01/2021 - "We present a 60-year-old female who was admitted due to an exacerbation of acquired thrombotic thrombocytopenic purpura requiring high doses of corticosteroids. " 10/01/2016 - "Therapeutic plasma exchange (TPE) has dramatically improved the prognosis of acquired thrombotic thrombocytopenic purpura (TTP), and TPE and corticosteroids are the mainstays of treatment of acquired TTP. " 01/01/2009 - "Although there appears to be a consistent treatment approach to idiopathic thrombotic thrombocytopenic purpura (TTP) using plasma exchange, corticosteroids and rituximab, the treatment strategies for TA-TMA are perplexing, in part, because the literature regarding this complex condition does not provide true consensus for incidence, aetiology, diagnostic criteria, classification and optimal therapy. "

Therapies and Procedures

1.	Plasma Exchange 10/01/2015 - "Plasma exchange (PE) is the first-line treatment for primary acquired thrombotic thrombocytopenic purpura (aTTP) with severe deficiency of ADAMTS13 activity (ADAMTS13:AC). " 10/01/2021 - "Consensus guidelines recommend that therapeutic plasma exchange (TPE) should be started within 4 to 8 hours after the diagnosis of suspected acquired thrombotic thrombocytopenic purpura (aTTP). " 10/01/2021 - "Benchmarking the centralized urgent plasma exchange service for patients admitted with a diagnosis of suspected acquired thrombotic thrombocytopenic purpura at a large healthcare system." 12/01/2018 - "Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange." 10/01/2015 - "Type of plasma preparation used for plasma exchange and clinical outcome of adult patients with acquired idiopathic thrombotic thrombocytopenic purpura: a French retrospective multicenter cohort study."
2.	Therapeutics 11/01/2015 - "Potential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic Purpura." 09/01/2005 - "This review focuses on recent advances in the use of immune-based therapy to treat patients with refractory and relapsing acquired thrombotic thrombocytopenic purpura. " 01/01/1984 - "[Aggressive therapy in a case of Moschowitz syndrome]." 10/01/2015 - "Poor responder to plasma exchange therapy in acquired thrombotic thrombocytopenic purpura is associated with ADAMTS13 inhibitor boosting: visualization of an ADAMTS13 inhibitor complex and its proteolytic clearance from plasma." 01/01/2015 - "Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura."
3.	Plasmapheresis 12/01/2020 - "Double filtration plasmapheresis (DFPP) could be an alternative method to simple plasma exchange plasmapheresis in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP). " 04/01/2006 - "In the past two decades, in addition to idiopathic thrombotic thrombocytopenic purpura (TTP) more cases of secondary TTP have been identified, perhaps due to an enthusiasm for cure potential with an availability of exchange-plasmapheresis. " 01/01/2015 - "We here describe a patient with an idiopathic thrombotic thrombocytopenic purpura (TTP) secondary to an ADAMTS13 inhibitor that continued to be dependent on plasmapheresis until the patient was treated with rituximab. "
4.	Liver Transplantation 01/01/2023 - "Hereditary thrombotic thrombocytopenic purpura acquired through liver transplantation: A case report." 05/01/2015 - "Successful liver transplantation in a child with acute-on-chronic liver failure and acquired thrombotic thrombocytopenic purpura."
5.	Splenectomy 03/01/2004 - "Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura." 11/01/2002 - "Splenectomy: the last option of immunosuppressive therapy in patients with chronic or relapsing idiopathic thrombotic thrombocytopenic purpura?"