A Púrpura Trombocitopênica Trombótica Adquirida é a forma da Púrpura Trombocitopênica Trombótica (PTT) que não é genética (ou seja, não é herdada). Ela é caracterizada por uma contagem muito baixa de plaquetas no sangue, um tipo de anemia chamado anemia hemolítica microangiopática (MAHA) — onde os glóbulos vermelhos são destruídos nos pequenos vasos sanguíneos — e pela falha de um ou mais órgãos, com diferentes níveis de gravidade.
Introdução
O que você precisa saber de cara
A Púrpura Trombocitopênica Trombótica Adquirida é a forma da Púrpura Trombocitopênica Trombótica (PTT) que não é genética (ou seja, não é herdada). Ela é caracterizada por uma contagem muito baixa de plaquetas no sangue, um tipo de anemia chamado anemia hemolítica microangiopática (MAHA) — onde os glóbulos vermelhos são destruídos nos pequenos vasos sanguíneos — e pela falha de um ou mais órgãos, com diferentes níveis de gravidade.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.
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Os sinais que médicos procuram e os exames que confirmam
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Publicações mais relevantes
Systematic Literature Review of the Economic and Quality of Life Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening blood disorder resulting from ADAMTS13 deficiency, caused by mutations in the ADAMTS13 gene in congenital TTP (cTTP), and by neutralizing antibodies against ADAMTS13 in immune-mediated TTP (iTTP). This systematic review summarizes available economic evaluations, healthcare resource utilization (HCRU), cost, utility, and health-related quality of life (HRQoL) literature in TTP, with a focus on cTTP. Given the limited availability of cTTP-specific data, broader TTP evidence was also reviewed to infer potential implications for cTTP where appropriate. Three systematic literature searches were conducted in January 2024 in accordance with PRISMA guidelines, each tailored to identify studies reporting economic evaluations, HCRU/cost data, and utility/HRQoL data respectively. For the economic evaluation and utility/HRQoL reviews, inclusion criteria were broadened to include studies reporting data on iTTP and unspecified TTP from which findings for cTTP were inferred. In total 431, 989, and 849 records were identified for the economic evaluation, HCRU/cost, and utility/HRQoL reviews, respectively. No economic evaluations were identified for treatments in cTTP populations. Eight economic studies (all in iTTP) were included. Overall, 5 studies found caplacizumab to be cost-effective, whereas 3 did not, highlighting variability across model assumptions and healthcare settings. Six HCRU/cost studies reported that cTTP was associated with frequent hospital interactions, missed work time, and high costs. Fourteen studies (1 in cTTP, 10 in iTTP, and 3 in unspecified TTP) were included in the utility/HRQoL review and showed that TTP is associated with reduced utility scores and lower HRQoL, irrespective of TTP subtype. These findings underscore the meaningful patient burden observed across the TTP spectrum and highlight the need for further research to evaluate the cost-effectiveness of emerging therapies in cTTP. However, the predominance of iTTP data reflects ongoing challenges in evidence generation for rare TTP subtypes such as cTTP.
Dynamic Monitoring of ADAMTS-13 Activity for Differential Diagnosis Across the Spectrum of Sepsis-Associated Thrombotic Microangiopathies.
In the ICU, distinguishing immune-mediated thrombotic thrombocytopenic purpura (iTTP) from sepsis-associated thrombotic microangiopathy (TMA) is time-critical. We tested whether serial ADAMTS-13 combined with targeted coagulation and inflammation markers improves iTTP risk stratification in a Sepsis-3 ICU cohort and whether a pragmatic rule-out is feasible. Prospective single-center study of adults meeting Sepsis-3 with thrombocytopenia and schistocytes ≥ 1% or LDH > 2× ULN within 24 h of ICU admission. ADAMTS-13 activity and VWF: Ag were assayed at 0/24/48/72 h alongside a thrombo-inflammatory panel. We derived a Dynamic ADAMTS-13 Index (DAI), a Coagulation Consumption Index (CCI) anchored to ISTH DIC and fibrinogen/antithrombin III, and an Inflammation Index (IL-6/HBP). The prespecified main rule-out required a ≥ 15% ADAMTS-13 rise by 48 h plus low CCI. A prespecified RCV-anchored sensitivity analysis required ≥ 35% relative rise or ≥ 10 absolute % points plus low consumption. For decision-making, pre-treatment (pre-plasma exchange, PEx) analyses are emphasized. Intent-to-diagnose (care-embedded) analyses are exploratory, and internal validation used a bootstrap optimism correction. Of 1,274 screened, 330 were included (iTTP = 34). Discrimination improved from baseline ADAMTS-13 (AUROC 0.78) to DAI (0.93), with smaller gains after adding CCI (0.95) and the Inflammation Index (0.96). With the main rule-out (≥ 15% + low CCI) in the intent-to-diagnose analysis, sensitivity was 97.1%, specificity was 86.1%, and NPV was 99.6%. The RCV-anchored sensitivity analysis preserved 100.0% sensitivity and NPV with 76.0% specificity. A 72-h phenotype (ADAMTS-13 < 10% with high IL-6/HBP) was associated with higher 28-day mortality (adjusted HR 2.6). In Sepsis-3 ICU patients with TMA features, serial ADAMTS-13 testing, along with targeted coagulation/inflammation markers, enhances early iTTP risk stratification and supports a pragmatic rule-out framework. External validation and implementation studies remain essential. These findings also support investment in rapid/automated ADAMTS-13 activity assays and decision-support workflows to enable timely adoption beyond tertiary centers.
A red blood cell-based antigen delivery system to facilitate T cell epitope presentation to promote peripheral tolerance to ADAMT S13 in immune-mediated thrombotic thrombocytopenic purpura.
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and potentially fatal autoimmune disease caused by a severe ADAMTS13 functional deficiency, mediated by autoantibodies targeting ADAMTS13. Despite high survival rates achieved with current treatments, between 20% and 50% of patients experience relapses. Ensuring longer-lasting recovery by restoring immune tolerance toward ADAMTS13 remains a significant unmet need. To address this, we aimed to exploit the tolerogenic nature of the red blood cell (RBC) clearance pathway by using RBCs as antigen carriers. This approach allows for persistent exposure to ADAMTS13-derived peptides, promoting antigen-specific attenuation of autoreactive CD4+ T cells and induction of regulatory T cells. Specifically, we designed a fusion peptide comprising the trans-activator of transcription cell-penetrating peptide and an immunodominant ADAMTS13-derived T cell epitope (FINVAPHAR core amino acid sequence) to enable RBC surface loading. We found that the fusion peptide binds to RBCs in a concentration-dependent manner. To assess whether our approach supports antigen presentation via human leukocyte antigen (HLA) class II molecules, macrophages were incubated with peptide-loaded RBCs, followed by analysis of HLA-DR-bound peptides via mass spectrometry. FINVAPHAR-containing peptides of varying lengths were mainly presented on macrophages from donors carrying the iTTP-associated HLA risk allele DRB1∗11, confirming functional antigen presentation and supporting the biological relevance of our model. Hemoglobin-derived peptides were also abundantly presented, supporting erythrophagocytosis as the entry route of our trans-activator of transcription-FINVAPHAR fusion peptide. Based on our results, we propose RBC-mediated delivery of ADAMTS13-derived peptides as a potential strategy for promoting tolerance in patients with iTTP.
Pregnancy-Associated Thrombotic Thrombocytopenic Purpura: Diagnostic Pitfalls, Therapeutic Strategies, and Emerging Paradigms.
Background: Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening thrombotic microangiopathy (TMA) caused by severe deficiency of the von Willebrand factor-cleaving protease ADAMTS13. Pregnancy is a recognized trigger for both immune-mediated and congenital TTP and is associated with increased maternal and fetal morbidity. Clinical overlap with other pregnancy-associated TMAs, including preeclampsia and Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) syndrome, often delays diagnosis. This review synthesizes current evidence on pathophysiology, diagnostic uncertainty, and gestation-specific management of pregnancy-associated TTP, highlighting differences between immune-mediated and congenital disease. Methods: This is a narrative review. We performed a targeted literature search of PubMed/MEDLINE (from inception to December 2025) to identify English-language publications. The study types included were case reports/series, observational studies, large database studies, randomized trials, reviews, and relevant guidelines addressing TMA in pregnancy, with emphasis on immune-mediated and congenital TTP. Search terms included "pregnancy", "thrombotic thrombocytopenic purpura", "hereditary TTP", "acquired TTP", "ADAMTS13," "thrombotic microangiopathy," "HELLP," "postpartum", and "complement-mediated TMA" alone or in combination. The search was supplemented by manual screening of reference lists and key guidelines. Articles were selected based on relevance to diagnosis and management of pregnancy-associated TTP. Conference abstracts and non-peer-reviewed sources were not routinely included and were considered only when peer-reviewed evidence was limited. Results: Pregnancy-associated TTP remains a major diagnostic challenge due to overlapping clinical and laboratory features with other obstetric thrombotic microangiopathies. Distinguishing immune-mediated from congenital TTP is essential, as management and prognosis differ substantially. Prompt recognition and early initiation of therapeutic plasma exchange, immunosuppression, or prophylactic plasma therapy markedly improve maternal outcomes. Rapid ADAMTS13 testing, structured risk stratification, and multidisciplinary care are central to optimal management. Fetal outcomes are closely linked to gestational age at onset and timeliness of therapy. Conclusions: Early differentiation of TTP from other pregnancy-associated TMAs is critical for maternal and fetal survival. Advances in rapid ADAMTS13 diagnostics and emerging targeted therapies, including caplacizumab and recombinant ADAMTS13, offer opportunities to improve precision management and outcomes in future pregnancies.
Comment on "Fulminant Myocarditis in Immune-Mediated Thrombotic Thrombocytopenic Purpura: A Case Report".
We commented on a recent case report of fulminant myocarditis in immune-mediated thrombotic thrombocytopenic purpura (iTTP). While the authors attributed cardiac dysfunction to myocarditis, we caution that microvascular ischemia remains a plausible mechanism, given the clinical context and nonspecific imaging. Diagnostic clarity is essential because therapeutic strategies may differ from one another. We advocated for a rigorous cardiac evaluation in patients with iTTP to inform appropriate management.
Publicações recentes
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Epidemiology, therapy, and outcome of immune-mediated thrombotic thrombocytopenic purpura at population level in Germany.
Treating Immune-Mediated Thrombotic Thrombocytopenic Purpura with Caplacizumab.
Method for Assessing Inhibition Kinetics of Autoantibodies Against ADAMTS13 in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Microfluidic Shear-Based Assay for Assessing ADAMTS13 Activity Under Flow.
📚 EuropePMC152 artigos no totalmostrando 197
Dynamic Monitoring of ADAMTS-13 Activity for Differential Diagnosis Across the Spectrum of Sepsis-Associated Thrombotic Microangiopathies.
Mediterranean journal of hematology and infectious diseasesSystematic Literature Review of the Economic and Quality of Life Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Journal of blood medicineA red blood cell-based antigen delivery system to facilitate T cell epitope presentation to promote peripheral tolerance to ADAMT S13 in immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisPregnancy-Associated Thrombotic Thrombocytopenic Purpura: Diagnostic Pitfalls, Therapeutic Strategies, and Emerging Paradigms.
BiomedicinesHematologic safety of caplacizumab in immune-mediated thrombotic thrombocytopenic purpura: insights from platelet-related signal detection in the FAERS database.
Thrombosis journal[Immune-mediated thrombotic thrombocytopenic purpura successfully diagnosed and treated through repeated ADAMTS13 inhibitor testing].
[Rinsho ketsueki] The Japanese journal of clinical hematologyMonitoring ADAMTS-13 conformation in immune-mediated thrombotic thrombocytopenic purpura: toward personalized management.
Research and practice in thrombosis and haemostasisEndothelial injury to cognitive decline: a 12-month follow-up using computed tomography perfusion and diffusion magnetic resonance imaging in immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHThrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus after anifrolumab: a possible association.
Archive of clinical casesHealth-related quality of life and mental health in autoimmune thrombotic thrombocytopenic purpura patients in the caplacizumab era.
Research and practice in thrombosis and haemostasisEthnicity affects relapse-free survival in immune-mediated thrombotic thrombocytopenic purpura.
HaematologicaAcute Kidney Injury in Immune-Mediated Thrombotic Thrombocytopenic Purpura - Solving the Puzzle?
Kidney international reportsTherapeutic plasma exchangerelated complications in patients with immune-mediated thrombotic thrombocytopenic purpura.
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British journal of haematologyTailored treatment of acute immune-mediated thrombotic thrombocytopenic purpura.
Hematology. American Society of Hematology. Education ProgramChallenges and opportunities in the long-term management of immune-mediated TTP.
Hematology. American Society of Hematology. Education ProgramEASIX, a new tool to predict response and refractoriness in immune-mediated thrombotic thrombocytopenic purpura.
Frontiers in immunologyImmune-mediated thrombotic thrombocytopenic purpura as a model of systemic microvascular dysfunction: moving from an acute to a chronic disorder.
Journal of thrombosis and haemostasis : JTHNovel cryptic ADAMTS13 epitopes uncover a distinct open ADAMTS13 conformation in immune-mediated TTP.
HaematologicaRecurrent Thrombocytopenia During Caplacizumab Therapy in Acute Immune-Mediated Thrombotic Thrombocytopenic Purpura.
American journal of hematologySuccessful use of recombinant ADAMTS13 in a pregnant patient with immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHAssociation of Cognitive Impairment with Reduced Health-Related Quality of Life and Depression Among Survivors of Thrombotic Thrombocytopenic Purpura.
Hematology reportsStandard cardiovascular risk prediction scores underestimate risk in immune-mediated thrombotic thrombocytopenic purpura survivors.
Research and practice in thrombosis and haemostasisNew Insights Into Renal Involvement During Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Kidney international reportsEvaluation of different platelet-dependent von Willebrand factor activity assays to assess the in vivo inhibitory effect of caplacizumab on the von Willebrand factor-platelet interaction.
Journal of thrombosis and haemostasis : JTHHigh anti-ADAMTS13 IgG Levels after Plasma Exchange Predict Delayed ADAMTS13 Normalization in Immune-mediated Thrombotic Thrombocytopenic Purpura.
Thrombosis and haemostasisPrognostic relevance of early clinical and laboratory findings in immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisTrp1250, Lys1252, and Arg1367 of ADAMTS13 comprise a hot spot for anti-CUB domain antibodies in patients with iTTP.
Blood advancesA peculiar case report of primary iTTP in a previously healthy Saudi man.
Journal of medicine and lifeImmune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome.
Lupus science & medicineFulminant myocarditis in immune-mediated thrombotic thrombocytopenic purpura: A case report.
Acute medicine & surgeryAnti-ADAMTS13 Antibodies Trajectory is Associated With ADAMTS13 Recovery in Immune-Mediated TTP.
American journal of hematology[Plasma exchange-free therapeutic management of immune-mediated thrombotic thrombocytopenic purpura].
[Rinsho ketsueki] The Japanese journal of clinical hematologyNavigating recurrent immune-mediated thrombotic thrombocytopenic purpura (iTTP) in pregnancy: A case report and literature review.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisHow we manage immune-mediated thrombotic thrombocytopenic purpura after rituximab failure or intolerance.
British journal of haematologyAcute stroke in children with immune thrombocytopenia: A systematic review and meta-analysis.
British journal of haematologyCaplacizumab use in immune-mediated thrombotic thrombocytopenic purpura: an international multicentre retrospective Cohort study (The Capla 1000+ project).
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Antibodies (Basel, Switzerland)Challenges in managing iTTP: insights into ADAMTS13 inhibitor boosting during caplacizumab therapy.
Annals of hematologyN-glycan shielded CUB domains of ADAMTS13 prevent binding of C-terminal antibodies in patients with immune-mediated TTP.
Blood advancesGC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura.
Scientific reportsUnraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS.
Blood advancesRituximab in Immune-mediated thrombotic thrombocytopenic purpura.
European journal of internal medicineComplex immunogenicity assessment in caplacizumab-treated patients with immune-mediated thrombotic thrombocytopenic purpura who have received plasma exchange.
Research and practice in thrombosis and haemostasisEfficacy and Safety of Obinutuzumab in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
American journal of hematologyBortezomib for rituximab-refractory immune-mediated thrombotic thrombocytopenic purpura in the caplacizumab era: an Italian multicenter study.
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British journal of haematologyMain features of ischemic stroke in patients with acute immune-mediated thrombotic thrombocytopenic purpura.
Thrombosis researchCaplacizumab as an add-on therapy in a 7-year-old girl with exacerbated immune-mediated thrombotic thrombocytopenic purpura, a case report and literature review.
Frontiers in pediatricsEfficacy and safety of daratumumab in multiresistant immune-mediated thrombotic thrombocytopenic purpura.
British journal of haematologyAssociation of Sjögren's syndrome with immune-mediated thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndrome: A case report.
HeliyonCaplacizumab improves clinical outcomes and is well tolerated across clinically relevant subgroups of patients with immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisA comparative study of anti-ADAMTS-13 antibody dynamics in immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisClinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren's disease.
RMD openDiagnostic Challenges in a Case of Immune-Mediated Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency.
CureusA Systematic Review of the Epidemiology and Disease Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Journal of blood medicineImmune-mediated thrombotic thrombocytopenic purpura with ischaemic cerebral infarction preceding onset of thrombocytopenia and microangiopathic haemolytic anaemia: A case report.
British journal of haematologyFrontline use of rituximab may prevent ADAMTS13 inhibitor boosting during caplacizumab treatment in patients with iTTP: post hoc analysis of a phase 2/3 study in Japan.
Thrombosis journalImmature platelet count responses of pediatric patients with immune-mediated thrombotic thrombocytopenic purpura.
Thrombosis researchEfficacy and relative safety of caplacizumab in immune-mediated thrombotic thrombocytopenic purpura: a systematic review and meta-analysis.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisTailoring caplacizumab administration using ADAMTS13 activity for immune-mediated thrombotic thrombocytopenic purpura.
Blood vessels, thrombosis & hemostasisReal-world insights on the management of immune-mediated thrombotic thrombocytopenic purpura with caplacizumab.
Blood vessels, thrombosis & hemostasisA machine learning approach to predict mortality due to immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisDe-novo immune-mediated thrombotic thrombocytopenic purpura following surgical and non-surgical procedures: A systematic review.
Blood reviewsReal-World Data Analysis of Patients Affected by Immune-Mediated Thrombotic Thrombocytopenic Purpura in Italy.
Journal of clinical medicineDiagnosis of thrombotic thrombocytopenic purpura: easy-to-use fiber optic surface plasmon resonance immunoassays for automated ADAMTS-13 antigen and conformation evaluation.
Journal of thrombosis and haemostasis : JTHOutcomes and Costs in Patients with Immune Thrombotic Thrombocytopenic Purpura Receiving Front-Line Versus Delayed Caplacizumab: A US Hospital Database Study.
Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisTargeting neutrophil extracellular trap accumulation under flow in patients with immune-mediated thrombotic thrombocytopenic purpura.
Blood advancesPeak ADAMTS13 activity to assess ADAMTS13 conformation and risk of relapse in immune-mediated thrombotic thrombocytopenic purpura.
BloodPersistent ADAMTS13 inhibitor delays recovery of ADAMTS13 activity in caplacizumab-treated Japanese patients with iTTP.
Blood advancesTreatment of immune-mediated thrombotic thrombocytopenic purpura without plasma exchange.
HaematologicaMechanism underlying severe deficiency of plasma ADAMTS-13 activity in immune thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHPlasma cell-directed therapy strategies in immune-mediated thrombotic thrombocytopenic purpura (iTTP).
TransfusionImmune-mediated thrombotic thrombocytopenic purpura: don't miss the boat.
Intensive care medicineSuccessful management of refractory immune-mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti-VWF nanobody caplacizumab.
British journal of haematologyADAMTS-13 conformation influences autoimmune recognition in immune thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHAlemtuzumab-induced immune-mediated thrombotic thrombocytopenic purpura: A newly described drug-related autoimmune disease.
British journal of haematologyCognitive decline in thrombotic thrombocytopenic purpura survivors: The role of white matter health as assessed by MRI.
British journal of haematologyA Long-Term Follow-Up Study in Immune-Mediated Thrombotic Thrombocytopenic Purpura: What Are the Outcomes?
Journal of clinical medicineSuccessful Use of Bortezomib in an Adolescent with Refractory TTP.
Case reports in hematologyTrue vs. false immune-mediated thrombotic thrombocytopenic purpura exacerbations: a clinical case in the caplacizumab era.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisOpen ADAMTS-13 conformation index predicts earlier relapse in immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHADAMTS13 and Non-ADAMTS13 Biomarkers in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Journal of clinical medicineTwo pregnant women with immune-mediated thrombotic thrombocytopenic purpura: A case report.
The journal of obstetrics and gynaecology researchAcute Hepatitis E Induced the First Episode of Immune-Mediated Thrombotic Thrombocytopenic Purpura: The First Case Report.
Infection and drug resistanceFive years of caplacizumab - lessons learned and remaining controversies in immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHA novel von Willebrand factor multimer ratio as marker of disease activity in thrombotic thrombocytopenic purpura.
Blood advancesPredictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Journal of neurologySafety and efficacy of caplacizumab retreatment in a real-life monocentric cohort of patients with immune-mediated thrombotic thrombocytopenic purpura.
Thrombosis researchImmature platelet dynamics in management of protracted response to therapy of a young pediatric patient with immune-mediated thrombotic thrombocytopenic purpura.
Thrombosis researchCaplacizumab: A game changer also in pregnancy-associated immune-mediated thrombotic thrombocytopenic purpura?
British journal of haematologyDaratumumab for refractory immune-mediated thrombotic thrombocytopenic purpura.
British journal of haematologyRituximab in the treatment of immune-mediated thrombotic thrombocytopenic purpura.
Blood transfusion = Trasfusione del sangueReversible skin microvascular hyporeactivity in patients with immune-mediated thrombocytopenic thrombotic purpura.
Critical care (London, England)Post-traumatic stress disorder and quality of life alterations in survivors of immune-mediated thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome.
Journal of critical careDistinguishing immune-mediated thrombotic thrombocytopenic purpura from septic disseminated intravascular coagulation using plasma levels of haptoglobin and factor XIII activity.
Research and practice in thrombosis and haemostasisDelayed normalization of ADAMTS13 activity in acute thrombotic thrombocytopenic purpura in the caplacizumab era.
BloodOverlapping ADAMTS13 peptide binding profiles of DRB1∗08:03 and DRB1∗11:01 suggest a common etiology of immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHCovid-19 vaccination in patients with immune-mediated thrombotic thrombocytopenic purpura: a single-referral center experience.
HaematologicaSecondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review.
Rheumatology internationalPredictive Scores for Early Identification of Immune-Mediated Thrombotic Thrombocytopenic Purpura: Room for Improvement?
Kidney international reportsImmune-mediated thrombotic thrombocytopenic purpura plasma induces calcium- and IgG-dependent endothelial activation: correlations with disease severity.
HaematologicaThe efficacy and safety of caplacizumab in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura: an open-label phase 2/3 study.
International journal of hematologyFrom the Discovery of ADAMTS13 to Current Understanding of Its Role in Health and Disease.
Seminars in thrombosis and hemostasisLong-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy.
BloodADAMTS13 conformation and immunoprofiles in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura.
Blood advancesHealth following recovery from immune thrombotic thrombocytopenic purpura: the patient's perspective.
Blood advancesPreemptive cyclosporin A in immune-mediated thrombotic thrombocytopenic purpura.
European journal of haematologyMore on the use of frontline caplacizumab in immune-mediated thrombotic thrombocytopenic purpura.
Blood advancesEpidemiology, treatment patterns, clinical outcomes, and disease burden among patients with immune-mediated thrombotic thrombocytopenic purpura in the United States.
Research and practice in thrombosis and haemostasisClinical features and neurological outcomes in pediatric immune-mediated thrombotic thrombocytopenic purpura: A report from a large pediatric hematology center.
Pediatric blood & cancerLong-term follow-up of patients treated with caplacizumab and safety and efficacy of repeat caplacizumab use: Post-HERCULES study.
Journal of thrombosis and haemostasis : JTHUnmet needs in the management of immune-mediated thrombotic thrombocytopenic purpura and the potential role of caplacizumab in the UK-A modified-Delphi study.
EJHaemMaternal and fetal outcomes of pregnancy occurring after a diagnosis of immune-mediated thrombotic thrombocytopenic purpura.
Annals of hematologyRace, rituximab, and relapse in TTP.
BloodEfficacy and safety of azathioprine during remission of immune-mediated thrombotic thrombocytopenic purpura.
Blood advancesHow I treat immune-mediated thrombotic thrombocytopenic purpura after hospital discharge.
BloodImmune-Mediated Thrombotic Thrombocytopenic Purpura Following mRNA-Based COVID-19 Vaccine BNT162b2: Case Report and Mini-Review of the Literature.
Frontiers in medicineImmune-mediated thrombotic thrombocytopenic purpura in a Jehovah's Witness - Effectiveness of incorporating extracorporeal immunoadsorption to caplacizumab, steroids and rituximab.
Clinical case reportsImmune-mediated thrombotic thrombocytopenic purpura prognosis is affected by blood pressure.
Research and practice in thrombosis and haemostasisNaturally Occurring Anti-Idiotypic Antibodies Portray a Largely Private Repertoire in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Journal of immunology (Baltimore, Md. : 1950)Relapse of immune-mediated thrombotic thrombocytopenic purpura following mRNA COVID-19 vaccination: a prospective cohort study.
Haematologica[Immune-mediated thrombotic thrombocytopenic purpura and susceptible HLA alleles].
[Rinsho ketsueki] The Japanese journal of clinical hematologyRecent advances in the management of immune-mediated thrombotic thrombocytopenic purpura.
Blood researchAnti-CD20 therapeutic options in immune-mediated thrombotic thrombocytopenic purpura.
British journal of haematologySuccessful preventive treatment with cyclosporine in a patient with relapsed/refractory immune-mediated thrombotic thrombocytopenic purpura: a case report and review of the literature.
International journal of hematologyImmune-mediated thrombotic thrombocytopenic purpura following COVID-19 vaccination.
BloodClinical relapse of immune-mediated thrombotic thrombocytopenic purpura following COVID-19 vaccination.
Research and practice in thrombosis and haemostasisAlternate-day dosing of caplacizumab for immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHImmune thrombotic thrombocytopenic purpura: Personalized therapy using ADAMTS-13 activity and autoantibodies.
Research and practice in thrombosis and haemostasisImmune-Mediated Thrombotic Thrombocytopenic Purpura after BNT162b2 Vaccine.
Turkish journal of haematology : official journal of Turkish Society of HaematologyFocus on Key Issues in Immune Thrombotic Thrombocytopenic Purpura: Italian Experience of Six Centers.
Journal of clinical medicineDeleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura.
PloS onePredictors of relapse and preventative strategies in immune thrombotic thrombocytopenic purpura.
Expert review of hematologyA case of "smoldering" immune-mediated thrombotic thrombocytopenic purpura manifesting as recurrent cardioembolic stroke.
Clinical case reportsAnti-cysteine/spacer antibodies that open ADAMTS13 are a common feature in iTTP.
Blood advancesIntracranial hemorrhage in immune thrombotic thrombocytopenic purpura treated with caplacizumab: COMMENT from Eşkazan et al.
Journal of thrombosis and haemostasis : JTHBlood-brain barrier permeability in survivors of immune-mediated thrombotic thrombocytopenic purpura: a pilot study.
Blood advancesAnti-ADAMTS13 autoantibody profiling in patients with immune-mediated thrombotic thrombocytopenic purpura.
Blood advances[Treatment of immune-mediated thrombotic thrombocytopenic purpura: A decisive turning point].
Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguineCardiovascular disease is a leading cause of mortality among TTP survivors in clinical remission.
Blood advancesConformational plasticity of ADAMTS13 in hemostasis and autoimmunity.
The Journal of biological chemistryMajor adverse cardiovascular events in survivors of immune-mediated thrombotic thrombocytopenic purpura.
American journal of hematologyImmune-mediated thrombotic thrombocytopenic purpura following administration of Pfizer-BioNTech COVID-19 vaccine.
HaematologicaStrong association between insufficient plasma exchange and fatal outcomes in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura.
International journal of hematologySurvival after acute episodes of immune-mediated thrombotic thrombocytopenic purpura (iTTP) - cognitive functioning and health-related quality of life impact: a descriptive cross-sectional survey of adults living with iTTP in the United Kingdom.
Hematology (Amsterdam, Netherlands)Do patients with immune-mediated thrombotic thrombocytopenic purpura receiving caplacizumab need antithrombotic therapy?
Expert review of clinical pharmacologyEvaluation of Immuno-Rolling Circle Amplification for Multiplex Detection and Profiling of Antigen-Specific Antibody Isotypes.
Analytical chemistryIncidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura.
Journal of clinical apheresisDetermination of anti-ADAMTS-13 autoantibody titers in ELISA: Influence of ADAMTS-13 presentation and autoantibody detection.
Journal of thrombosis and haemostasis : JTHTreating thrombotic thrombocytopenic purpura without plasma exchange during the COVID-19 pandemic. A case report and a brief literature review.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisImmune-mediated thrombotic thrombocytopenic purpura in childhood treated by caplacizumab, about 3 cases.
Journal of nephrologyIdentification of a novel genetic locus associated with immune-mediated thrombotic thrombocytopenic purpura.
HaematologicaN-glycan-mediated shielding of ADAMTS13 prevents binding of pathogenic autoantibodies in immune-mediated TTP.
BloodRedefining outcomes in immune TTP: an international working group consensus report.
BloodImmature platelet dynamics correlate with ADAMTS13 deficiency and predict therapy response in immune-mediated thrombotic thrombocytopenic purpura.
Thrombosis researchEffective and safe off-label use of caplacizumab treatment in a middle-aged obese male.
Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguineLaboratory surveillance of immune-mediated thrombotic thrombocytopenic purpura.
Hematology. American Society of Hematology. Education ProgramShould all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab?
Journal of thrombosis and haemostasis : JTHIntensive rituximab regimen in immune-mediated thrombotic thrombocytopenic purpura can circumvent unresponsiveness to standard rituximab treatment.
British journal of haematologyExome Sequencing Identifies Abnormalities in Glycosylation and ANKRD36C in Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Thrombosis and haemostasisImmunogenic hotspots in the spacer domain of ADAMTS13 in immune-mediated thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTHA regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP.
BloodReal-world experience with caplacizumab in the management of acute TTP.
BloodElevated plasma levels of syndecan-1 and soluble thrombomodulin predict adverse outcomes in thrombotic thrombocytopenic purpura.
Blood advancesThe HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians.
Journal of clinical medicineLong-term cognitive impairments following recovery in the patients with immune-mediated thrombotic thrombocytopenic purpura.
British journal of haematologyEfficacy of subcutaneous preemptive rituximab in immune-mediated thrombotic thrombocytopenic purpura: Experience from the first 12 cases.
American journal of hematologyAssessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes.
Journal of blood medicineHemophagocytic lymphohistiocytosis in a patient with COVID-19 treated with tocilizumab: a case report.
Journal of medical case reportsCo(III)-NTA Mediated Antigen Immobilization on a Fiber Optic-SPR Biosensor for Detection of Autoantibodies in Autoimmune Diseases: Application in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Analytical chemistryImmune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study.
Orphanet journal of rare diseases[Venous thrombo-embolism during immune-mediated thrombotic thrombocytopenic purpura is prevalent in patients with a prolonged treatment with therapeutic plasma exchange].
La Revue de medecine interneGeneration and validation of small ADAMTS13 fragments for epitope mapping of anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura.
Research and practice in thrombosis and haemostasisClinical outcomes and risk factors for severe COVID-19 in patients with haematological disorders receiving chemo- or immunotherapy.
British journal of haematologyDevelopment and validation of a prediction model (AHC) for early identification of refractory thrombotic thrombocytopenic purpura using nationally representative data.
British journal of haematologyOpen ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura.
BloodLow levels of ADAMTS-13 with high anti-ADAMTS-13 antibodies during remission of immune-mediated thrombotic thrombocytopenic purpura highly predict for disease relapse: A multi-institutional study.
American journal of hematologyImmune-Mediated Thrombotic Thrombocytopenic Purpura: A Narrative Review of Diagnosis and Treatment in Adults.
Seminars in thrombosis and hemostasisHLA loci predisposing to immune TTP in Japanese: potential role of the shared ADAMTS13 peptide bound to different HLA-DR.
BloodAnti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura do not hamper ELISA-based quantification of ADAMTS13 antigen.
Journal of thrombosis and haemostasis : JTHAmotosalen-inactivated fresh frozen plasma is comparable to solvent-detergent inactivated plasma to treat thrombotic thrombocytopenic purpura.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisRisk factors associated with the human leucocyte antigen system in Lebanese patients with immune-mediated thrombotic thrombocytopenic purpura.
Presse medicale (Paris, France : 1983)[Effect of Recombinant Protein in the Spacer Domain on ADAMTS13 Activity].
Zhongguo shi yan xue ye xue za zhiObinutuzumab in two patients suffering from immune-mediated thrombotic thrombocytopenic purpura intolerant to rituximab.
American journal of hematologyCaplacizumab to treat immune-mediated thrombotic thrombocytopenic purpura.
Drugs of today (Barcelona, Spain : 1998)Rituximab Prevents Stroke Recurrences in Atypical Chronic Immune-Mediated Thrombotic Thrombocytopenic Purpura.
TH open : companion journal to thrombosis and haemostasisBethesda Assay for Detecting Inhibitory Anti-ADAMTS13 Antibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
TH open : companion journal to thrombosis and haemostasisHow targeted therapy disrupts the treatment paradigm for acquired TTP: the risks, benefits, and unknowns.
BloodDevelopments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura.
Expert review of hematologyPredictors of relapse and efficacy of rituximab in immune thrombotic thrombocytopenic purpura.
Blood advancesComparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura.
British journal of haematologyGeneration of anti-idiotypic antibodies to detect anti-spacer antibody idiotopes in acute thrombotic thrombocytopenic purpura patients.
HaematologicaTransfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.
Arteriosclerosis, thrombosis, and vascular biologyAnti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Thrombosis and haemostasisPreemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura.
BloodClinical factors and biomarkers predict outcome in patients with immune-mediated thrombotic thrombocytopenic purpura.
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Systematic Literature Review of the Economic and Quality of Life Burden of Congenital and Immune-Mediated Thrombotic Thrombocytopenic Purpura.
- Dynamic Monitoring of ADAMTS-13 Activity for Differential Diagnosis Across the Spectrum of Sepsis-Associated Thrombotic Microangiopathies.
- A red blood cell-based antigen delivery system to facilitate T cell epitope presentation to promote peripheral tolerance to ADAMT S13 in immune-mediated thrombotic thrombocytopenic purpura.
- Pregnancy-Associated Thrombotic Thrombocytopenic Purpura: Diagnostic Pitfalls, Therapeutic Strategies, and Emerging Paradigms.
- Comment on "Fulminant Myocarditis in Immune-Mediated Thrombotic Thrombocytopenic Purpura: A Case Report".
- Establishing reverse chimeric antigen receptor T cells for precise targeting of immunemediated thrombotic thrombocytopenic purpura.
- Epidemiology, therapy, and outcome of immune-mediated thrombotic thrombocytopenic purpura at population level in Germany.
- Treating Immune-Mediated Thrombotic Thrombocytopenic Purpura with Caplacizumab.
- Method for Assessing Inhibition Kinetics of Autoantibodies Against ADAMTS13 in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
- Microfluidic Shear-Based Assay for Assessing ADAMTS13 Activity Under Flow.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:93585(Orphanet)
- MONDO:0019740(MONDO)
- Purpura Trombocitopenica Idiopatica(PCDT · Ministério da Saúde)
- GARD:4607(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788848(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
