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Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

2021

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTT…

medicine.medical_specialtyThrombotic microangiopathyTTPdiagnosisThrombotic thrombocytopenic purpuralcsh:MedicineReview030204 cardiovascular system & hematologycaplacizumabGastroenterology03 medical and health sciences0302 clinical medicineVon Willebrand factorhemic and lymphatic diseasesInternal medicinefollow-upmedicinethrombotic thrombocytopenic purpuratreatmentbiologybusiness.industrylcsh:RGeneral MedicineMicroangiopathic hemolytic anemiamedicine.diseaseADAMTS13ADAMTS13biology.proteinRituximabFresh frozen plasmaCaplacizumabbusiness030215 immunologymedicine.drugJournal of Clinical Medicine
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Algoritmo diagnóstico-terapéutico de las microangiopatías trombóticas. A propósito de 2 casos

2022

Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).

medicine.medical_specialtyThrombotic microangiopathyTherapeutic algorithmThrombotic thrombocytopenic purpura030204 cardiovascular system & hematologyurologic and male genital diseasesCritical Care and Intensive Care MedicineGastroenterology03 medical and health sciences0302 clinical medicinehemic and lymphatic diseasesInternal medicinemedicineThrombotic Microangiopathiesneoplasmsbusiness.industryOrgan dysfunction030208 emergency & critical care medicineEculizumabmedicine.diseaseADAMTS13Anesthesiology and Pain MedicineHaemolytic-uraemic syndromemedicine.symptombusinessmedicine.drugRevista Española de Anestesiología y Reanimación
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Comparison of Percutaneous Coronary Intervention (With Drug-Eluting Stents) Versus Coronary Artery Bypass Grafting in Women With Severe Narrowing of …

2014

Women typically present with coronary artery disease later than men with more unfavorable clinical and anatomic characteristics. It is unknown whether differences exist in women undergoing treatment for unprotected left main coronary artery (ULMCA) disease. Our aim was to evaluate long-term clinical outcomes in women treated with percutaneous coronary intervention (PCI) with drug-eluting stents versus coronary artery bypass grafting (CABG). All consecutive women from the Drug-Eluting stent for LefT main coronary Artery disease registry with ULMCA disease were analyzed. A propensity matching was performed to adjust for baseline differences. In total, 817 women were included: 489 (59.8%) unde…

medicine.medical_specialtyTime Factorsmedicine.medical_treatmentCoronary AngiographySeverity of Illness IndexCoronary artery diseasePercutaneous Coronary InterventionRisk FactorsInternal medicinemedicineHumansMyocardial infarctionRegistriesCoronary Artery BypassPropensity ScoreAgedRetrospective Studiesbusiness.industryCoronary StenosisPercutaneous coronary interventionStentDrug-Eluting StentsOdds ratiomedicine.diseaseCoronary VesselsSurgerymedicine.anatomical_structureTreatment OutcomeDrug-eluting stentConventional PCICardiologyFemaleCardiology and Cardiovascular MedicinebusinessArteryFollow-Up Studies
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DOP41 Efficacy and safety of open-label treatment with tofacitinib 10 mg twice daily in patients with ulcerative colitis with clinical response, but …

2019

medicine.medical_specialtyTofacitinibOctave (poetry)business.industryOPEN LABEL TREATMENTGastroenterologyGeneral Medicinemedicine.diseaseUlcerative colitisMaintenance therapyInternal medicinemedicineIn patientbusinessJournal of Crohn's and Colitis
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Improved Safety with the Use of Subcutaneous Bortezomib in Combination with Panobinostat and Dexamethasone: Preliminary Data from a Panobinostat Glob…

2016

Abstract Introduction: Panobinostat (PAN) is a potent pan-deacetylase inhibitor that targets multiple myeloma (MM) cells via its epigenetic effects as well as its effect on the aggresome. In the PANORAMA 1 phase 3 trial, the combination of PAN, bortezomib (BTZ), and dexamethasone (Dex; PAN+BTZ+Dex) significantly increased progression-free survival compared with placebo plus BTZ and Dex, leading to approval in Europe of the combination for the treatment of patients with MM who have received ≥ 2 prior regimens, including BTZ and an immunomodulatory agent. The purpose of this expanded treatment protocol (ETP) is to further evaluate safety and to provide panobinostat prior to commercial availab…

medicine.medical_specialtyTreatment protocolBortezomibbusiness.industryImmunologyDisease progressionCell BiologyHematologyNeutropeniamedicine.diseaseBiochemistryOlder populationSurgerychemistry.chemical_compoundchemistryTolerabilityInternal medicinePanobinostatmedicinebusinessDexamethasonemedicine.drugBlood
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Comparative effects of UVA and UVB irradiation on the immune system of fish.

2000

Aquatic organisms can be harmed by the current levels of solar ultraviolet radiation. We have recently shown that exposure of fish to UVB irradiation alters the functioning of the fish immune system, but the effects of UVA radiation are unknown. The present study continues this work by characterizing UVA irradiation-induced immunological changes in fish. Roach, a cyprinid fish, were exposed to a single dose of either UVA (3.6 J/cm2) or UVB (0.5 J/cm2) irradiation. Both irradiations suppressed transiently mitogen-stimulated proliferation of blood lymphocytes. UVA, but not UVB, decreased hematocrit, plasma protein, and plasma immunoglobulin levels and increased the proportions of blood cells …

medicine.medical_specialtyUltraviolet RaysBiophysicsCyprinidaeImmunoglobulinsHematocritLymphocyte ActivationLeukocyte CountImmune systemInternal medicinemedicineAnimalsRadiology Nuclear Medicine and imagingLymphocytesRadiationHematologyintegumentary systemRadiological and Ultrasound Technologybiologymedicine.diagnostic_testGranulocytosisBlood Proteinsmedicine.diseaseMolecular biologyBlood proteinsBlood chemistryHematocritImmunologybiology.proteinErythrocyte Countsense organsAntibodyLymphocytopeniaJournal of photochemistry and photobiology. B, Biology
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Thrombotic thrombocytopenic purpura (TTP) leading to pseudotumour's autoimmune pancreatitis (AIP): A case report

2012

International audience; Introduction: Autoimmune pancreatitis is an idiopathic inflammatory disease that produces pancreatic masses and ductal strictures. This benign disease can be associated with extrapancreatic manifestations including cholangitis, sialadenitis, inflammatory bowel disease or retroperitoneal fibrosis, mediastinal adenopathy, interstitial nephritis mainly due to immunoglobulin G4 (Ig G4), and occasional association with other auto-immune diseases. Observation: We report a 57-year-old woman who developed thrombotic thrombocytopenic purpura (UP) and pseudo-tumour's seronegative autoimmune pancreatitis (ATP) type 1. The patient was initially treated with pulse corticosteroids…

medicine.medical_specialtyVON-WILLEBRAND-FACTOREndocrinology Diabetes and Metabolismmedicine.medical_treatmentInterstitial nephritisAnti-Inflammatory AgentsThrombotic thrombocytopenic purpuraRetroperitoneal fibrosisGastroenterologyInflammatory bowel diseaseDISEASEAutoimmune DiseasesAntibodies Monoclonal Murine-Derived03 medical and health sciences0302 clinical medicineThrombotic thrombocytopenic purpuraInternal medicine[SDV.IDA]Life Sciences [q-bio]/Food engineeringmedicineHumans[SPI.GPROC]Engineering Sciences [physics]/Chemical and Process EngineeringSYSTEMIC-LUPUS-ERYTHEMATOSUSAutoimmune pancreatitisAutoimmune pancreatitisPurpura Thrombotic ThrombocytopenicHepatologybusiness.industryENTITYGastroenterologyMiddle Agedmedicine.diseaseSialadenitis3. Good healthPancreatitis030220 oncology & carcinogenesisImmunologyFemale030211 gastroenterology & hepatologyRituximabPlasmapheresismedicine.symptombusinessRituximabmedicine.drug
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Long-Term Outcomes with Subcutaneous C1-Inhibitor Replacement Therapy for Prevention of Hereditary Angioedema Attacks: An Open-Label Extension Study …

2018

medicine.medical_specialtybiologybusiness.industryC1 Esterase Inhibitor ProteinExtension studymedicine.diseaseC1-inhibitorInternal medicineHereditary angioedemabiology.proteinLong term outcomesMedicineOpen labelbusinessSSRN Electronic Journal
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Patterns of changes of anti‐ADAMTS13 after plasma exchange

2006

An enzyme-linked immunosorbent assay (ELISA) has recently been developed to detect antibodies against the von Willebrand factor-cleaving protease ADAMTS13 in patients with thrombotic thrombocytopenic purpura (TTP). The ELISA is based on incubation of plasma with immobilized recombinant ADAMTS13 followed by visualization of IgG and IgM antibodies by means of secondary enzyme-labeled antibodies [1]. In a recent study, anti-ADAMTS13 IgG antibodies were detected in most patients with TTP (97%) characterized by severe ADAMTS13 deficiency (< 10% of normal) [1]. The ELISA was more sensitive than the standard inhibitor assay based upon ADAMTS13 neutralizing activity, which gave positive results in …

medicine.medical_specialtybiologybusiness.industryThrombotic thrombocytopenic purpuraAntibody titerHematologymedicine.diseaseGastroenterologyADAMTS13Immunoglobulin GTiterhemic and lymphatic diseasesInternal medicineImmunologymedicinebiology.proteinPlateletAntibodybusinessIncubationJournal of Thrombosis and Haemostasis
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Human Papillomavirus (HPV) Detection in Keratotic Variants of Oral Lichen Planus: Negative Results in a Preliminary Open Study

2017

Background During the last few decades, several researches have been identified HPV genomic sequences in Head Neck Squamous Cell Carcinomas (HNSCC) and in Oral Potentially Malignant Disorders (OPMD) hypothesizing a possible viral etiological role. However, the reported prevalence of HPV-DNA (varying from 0% to 100%) in these lesions result still very discordant. Objective Human papillomavirus (HPV) infection in Oral Lichen Planus (OLP) has been largely investigated but not so extensively with respect to its morphological/histopathological features. The possible influence of hyper-keratosis/parakeratosis in HPV detection for keratotic variants of OLP is here proposed and investigated. Materi…

medicine.medical_specialtybusiness.industry030206 dentistryGeneral MedicineHpv detectionmedicine.diseaseDermatologyOpen study03 medical and health sciences0302 clinical medicine030220 oncology & carcinogenesisMedicineOral lichen planusHPV Keratotic Variants Oral lichen planusHuman papillomavirusbusinessARC Journal of Dental Science
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