The New England Journal of Medicine, ISSN 0028-4793, 02/2016, Volume 374, Issue 6, pp. 511 - 522
Thrombotic thrombocytopenic purpura is often caused by an autoantibody to ADAMTS13, resulting in ultralarge von Willebrand factor, which induces platelet...
ANTIBODIES | CARDIAC INVOLVEMENT | RECOVERY | DIAGNOSIS | MEDICINE, GENERAL & INTERNAL | MANAGEMENT | MYOCARDIAL-INFARCTION | ADAMTS13 ACTIVITY | RITUXIMAB | EXPERIENCE | MICROANGIOPATHIES | Single-Blind Method | Recurrence | Single-Domain Antibodies - therapeutic use | Single-Domain Antibodies - adverse effects | Humans | Middle Aged | Male | ADAMTS13 Protein | Remission Induction | Young Adult | Fibrinolytic Agents - adverse effects | ADAM Proteins - metabolism | von Willebrand Factor - antagonists & inhibitors | Fibrinolytic Agents - therapeutic use | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Aged | Hemorrhage - chemically induced | Purpura, Thrombotic Thrombocytopenic - metabolism | Plasma | Immunoglobulins | Thrombosis | Patients | Bleeding | Morbidity | Immunosuppressive agents | Thrombocytopenic purpura | Thrombotic thrombocytopenic purpura | Ischemia | Blood platelets | Purpura | Von Willebrand factor | Remission | Microvasculature | Blood diseases | Health risk assessment | Platelets | Drug therapy
ANTIBODIES | CARDIAC INVOLVEMENT | RECOVERY | DIAGNOSIS | MEDICINE, GENERAL & INTERNAL | MANAGEMENT | MYOCARDIAL-INFARCTION | ADAMTS13 ACTIVITY | RITUXIMAB | EXPERIENCE | MICROANGIOPATHIES | Single-Blind Method | Recurrence | Single-Domain Antibodies - therapeutic use | Single-Domain Antibodies - adverse effects | Humans | Middle Aged | Male | ADAMTS13 Protein | Remission Induction | Young Adult | Fibrinolytic Agents - adverse effects | ADAM Proteins - metabolism | von Willebrand Factor - antagonists & inhibitors | Fibrinolytic Agents - therapeutic use | Purpura, Thrombotic Thrombocytopenic - drug therapy | Adult | Female | Aged | Hemorrhage - chemically induced | Purpura, Thrombotic Thrombocytopenic - metabolism | Plasma | Immunoglobulins | Thrombosis | Patients | Bleeding | Morbidity | Immunosuppressive agents | Thrombocytopenic purpura | Thrombotic thrombocytopenic purpura | Ischemia | Blood platelets | Purpura | Von Willebrand factor | Remission | Microvasculature | Blood diseases | Health risk assessment | Platelets | Drug therapy
Journal Article
Blood, ISSN 0006-4971, 11/2018, Volume 132, Issue Supplement 1, pp. 3750 - 3750
Abstract INTRODUCTION Immune thrombotic thrombocytopenic purpura (iTTP) is an acute, multisystem thrombotic microangiopathy mediated by immunoglobulin G (IgG)...
Journal Article
Blood, ISSN 0006-4971, 12/2016, Volume 128, Issue 22, pp. 1365 - 1365
Abstract Background: Acute immune-mediated Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening disorder caused by acquired antibodies to the Von...
Journal Article
British Journal of Haematology, ISSN 0007-1048, 03/2019, Volume 184, Issue 5, pp. 858 - 861
Journal Article
British Journal of Haematology, ISSN 0007-1048, 12/2015, Volume 171, Issue 5, pp. 830 - 835
Summary Thrombotic microangiopathies (TMAs) are frequently difficult to differentiate clinically, and measurement of ADAMTS13 (a disintegrin and...
haemolytic uraemic syndrome | thrombotic thrombocytopenic purpura | ADAMTS13 | thrombotic microangiopathies | Haemolytic uraemic syndrome | Thrombotic thrombocytopenic purpura | Thrombotic microangiopathies | ANTIBODIES | HEMOLYTIC-UREMIC SYNDROME | THROMBOCYTOPENIC PURPURA | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | DEFICIENCY | Biomarkers - metabolism | Diagnosis, Differential | Humans | Middle Aged | Child, Preschool | Infant | Male | Purpura, Thrombotic Thrombocytopenic - diagnosis | ADAMTS13 Protein | Young Adult | Creatinine - metabolism | ADAM Proteins - metabolism | Platelet Count | Adolescent | Aged, 80 and over | Adult | Female | Registries | Thrombotic Microangiopathies - diagnosis | Aged | Retrospective Studies | Child | Infant, Newborn
haemolytic uraemic syndrome | thrombotic thrombocytopenic purpura | ADAMTS13 | thrombotic microangiopathies | Haemolytic uraemic syndrome | Thrombotic thrombocytopenic purpura | Thrombotic microangiopathies | ANTIBODIES | HEMOLYTIC-UREMIC SYNDROME | THROMBOCYTOPENIC PURPURA | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | DEFICIENCY | Biomarkers - metabolism | Diagnosis, Differential | Humans | Middle Aged | Child, Preschool | Infant | Male | Purpura, Thrombotic Thrombocytopenic - diagnosis | ADAMTS13 Protein | Young Adult | Creatinine - metabolism | ADAM Proteins - metabolism | Platelet Count | Adolescent | Aged, 80 and over | Adult | Female | Registries | Thrombotic Microangiopathies - diagnosis | Aged | Retrospective Studies | Child | Infant, Newborn
Journal Article
Transfusion, ISSN 0041-1132, 01/2017, Volume 57, Issue 1, pp. 131 - 136
Journal Article
British Journal of Haematology, ISSN 0007-1048, 03/2014, Volume 164, Issue 6, pp. 858 - 866
Summary Complement dysregulation is key in the pathogenesis of atypical Haemolytic Uraemic Syndrome (aHUS), but no clear role for complement has been...
cytokines | ADAMTS13 | Thrombotic Thrombocytopenic Purpura | complement | T cells | Complement | Cytokines | ANTIBODIES | ACTIVATION | MICROANGIOPATHIES | FACTOR-CLEAVING PROTEASE | DEFICIENCY | IMMUNE | ADAMTS-13 | LUPUS | INFLAMMATION | DISEASE | HEMATOLOGY | ADAM Proteins - immunology | Prospective Studies | Humans | Middle Aged | Male | Complement Activation - immunology | Hemolytic-Uremic Syndrome - immunology | Hemolytic-Uremic Syndrome - pathology | Young Adult | Purpura, Thrombotic Thrombocytopenic - pathology | Adolescent | Purpura, Thrombotic Thrombocytopenic - immunology | Adult | Female | Aged | Atypical Hemolytic Uremic Syndrome | Cohort Studies | Cytokines - immunology | Thrombocytopenic purpura | Immunoglobulin G | Platelets, Haemostasis and Thrombosis
cytokines | ADAMTS13 | Thrombotic Thrombocytopenic Purpura | complement | T cells | Complement | Cytokines | ANTIBODIES | ACTIVATION | MICROANGIOPATHIES | FACTOR-CLEAVING PROTEASE | DEFICIENCY | IMMUNE | ADAMTS-13 | LUPUS | INFLAMMATION | DISEASE | HEMATOLOGY | ADAM Proteins - immunology | Prospective Studies | Humans | Middle Aged | Male | Complement Activation - immunology | Hemolytic-Uremic Syndrome - immunology | Hemolytic-Uremic Syndrome - pathology | Young Adult | Purpura, Thrombotic Thrombocytopenic - pathology | Adolescent | Purpura, Thrombotic Thrombocytopenic - immunology | Adult | Female | Aged | Atypical Hemolytic Uremic Syndrome | Cohort Studies | Cytokines - immunology | Thrombocytopenic purpura | Immunoglobulin G | Platelets, Haemostasis and Thrombosis
Journal Article
British Journal of Haematology, ISSN 0007-1048, 05/2017, Volume 177, Issue 3, pp. 475 - 480
Summary Eltrombopag is well established in treatment of severe immune thrombocytopenia (ITP) and is increasingly commonplace in second‐line management. A role...
ITP | eltrombopag | TPO/THPO agonist therapy | LONG-TERM | TRIAL | TPO | CHRONIC IMMUNE THROMBOCYTOPENIA | MANAGEMENT | PURPURA | THPO agonist therapy | HEMATOLOGY | Benzoates - adverse effects | Pyrazoles - therapeutic use | Benzoates - therapeutic use | Humans | Middle Aged | Male | Purpura, Thrombocytopenic, Idiopathic - blood | Purpura, Thrombocytopenic, Idiopathic - drug therapy | Dose-Response Relationship, Drug | Young Adult | Thrombocytopenia - drug therapy | Aged, 80 and over | Adult | Female | Retrospective Studies | Hydrazines - administration & dosage | Pyrazoles - adverse effects | Hydrazines - therapeutic use | Drug Administration Schedule | Treatment Outcome | Thrombocytopenia - blood | Benzoates - administration & dosage | Receptors, Thrombopoietin - agonists | Pyrazoles - administration & dosage | Platelet Count | Adolescent | Aged | Hydrazines - adverse effects | Thrombocytopenia | Therapy | Side effects | Bridging | Complications | Surgery | Exanthema | Diarrhea | Nausea | Patients
ITP | eltrombopag | TPO/THPO agonist therapy | LONG-TERM | TRIAL | TPO | CHRONIC IMMUNE THROMBOCYTOPENIA | MANAGEMENT | PURPURA | THPO agonist therapy | HEMATOLOGY | Benzoates - adverse effects | Pyrazoles - therapeutic use | Benzoates - therapeutic use | Humans | Middle Aged | Male | Purpura, Thrombocytopenic, Idiopathic - blood | Purpura, Thrombocytopenic, Idiopathic - drug therapy | Dose-Response Relationship, Drug | Young Adult | Thrombocytopenia - drug therapy | Aged, 80 and over | Adult | Female | Retrospective Studies | Hydrazines - administration & dosage | Pyrazoles - adverse effects | Hydrazines - therapeutic use | Drug Administration Schedule | Treatment Outcome | Thrombocytopenia - blood | Benzoates - administration & dosage | Receptors, Thrombopoietin - agonists | Pyrazoles - administration & dosage | Platelet Count | Adolescent | Aged | Hydrazines - adverse effects | Thrombocytopenia | Therapy | Side effects | Bridging | Complications | Surgery | Exanthema | Diarrhea | Nausea | Patients
Journal Article
Expert Opinion on Orphan Drugs, ISSN 2167-8707, 10/2018, Volume 6, Issue 10, pp. 577 - 584
Introduction: Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening condition, associated with a low platelet count, hemolytic anemia,...
Caplacizumab | anti ADAMTS 13 auto antibodies | immune TTP | ADAMTS 13 | thrombocytopenia | SAFETY | MICROANGIOPATHIES REFERENCE CENTER | HEMOLYTIC UREMIC SYNDROME | IGG ANTIBODIES | VON-WILLEBRAND-FACTOR | PLASMA-EXCHANGE | PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY | ADAMTS13 | RITUXIMAB | PHARMACOLOGY & PHARMACY | ANTI-ADAMTS13 AUTOANTIBODIES
Caplacizumab | anti ADAMTS 13 auto antibodies | immune TTP | ADAMTS 13 | thrombocytopenia | SAFETY | MICROANGIOPATHIES REFERENCE CENTER | HEMOLYTIC UREMIC SYNDROME | IGG ANTIBODIES | VON-WILLEBRAND-FACTOR | PLASMA-EXCHANGE | PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY | ADAMTS13 | RITUXIMAB | PHARMACOLOGY & PHARMACY | ANTI-ADAMTS13 AUTOANTIBODIES
Journal Article
Annals of the Rheumatic Diseases, ISSN 0003-4967, 03/2014, Volume 73, Issue 3, p. A81
Journal Article
British Journal of Haematology, ISSN 0007-1048, 11/2015, Volume 171, Issue 4, pp. 625 - 630
Summary Severe immune thrombocytopenia purpura (ITP) presents a clinical challenge. Second‐line treatment options are variable without a precise protocol. We...
immune thrombocytopenia purpura | mycophenolate mofetil | platelets | Platelets | Immune thrombocytopenia purpura | Mycophenolate mofetil | CELL-DEPLETING THERAPY | ITP | EFFICACY | PURPURA | RITUXIMAB | ADULTS | HEMATOLOGY | TRANSPLANTATION | Purpura, Thrombocytopenic, Idiopathic - surgery | Comorbidity | Humans | Immunosuppressive Agents - therapeutic use | Middle Aged | Drug Evaluation | Salvage Therapy | Male | Combined Modality Therapy | Virus Diseases - complications | Remission Induction | Purpura, Thrombocytopenic, Idiopathic - drug therapy | Purpura, Thrombocytopenic, Idiopathic - etiology | Splenectomy | Young Adult | Mycophenolic Acid - analogs & derivatives | Mycophenolic Acid - therapeutic use | Immunoglobulins, Intravenous - therapeutic use | Aged, 80 and over | Adult | Female | Aged | Retrospective Studies | Thrombocytopenia | Care and treatment
immune thrombocytopenia purpura | mycophenolate mofetil | platelets | Platelets | Immune thrombocytopenia purpura | Mycophenolate mofetil | CELL-DEPLETING THERAPY | ITP | EFFICACY | PURPURA | RITUXIMAB | ADULTS | HEMATOLOGY | TRANSPLANTATION | Purpura, Thrombocytopenic, Idiopathic - surgery | Comorbidity | Humans | Immunosuppressive Agents - therapeutic use | Middle Aged | Drug Evaluation | Salvage Therapy | Male | Combined Modality Therapy | Virus Diseases - complications | Remission Induction | Purpura, Thrombocytopenic, Idiopathic - drug therapy | Purpura, Thrombocytopenic, Idiopathic - etiology | Splenectomy | Young Adult | Mycophenolic Acid - analogs & derivatives | Mycophenolic Acid - therapeutic use | Immunoglobulins, Intravenous - therapeutic use | Aged, 80 and over | Adult | Female | Aged | Retrospective Studies | Thrombocytopenia | Care and treatment
Journal Article
Blood, ISSN 0006-4971, 07/2017, Volume 130, Issue 4, pp. 466 - 471
Immune-mediated thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by antibodies against ADAMTS13. From the United Kingdom TTP...
AUTOANTIBODIES | DIAGNOSIS | PLASMA-EXCHANGE | EXPERIENCE | IGG ANTIBODIES | MICROANGIOPATHIES | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | REGISTRY | REMISSION | FEATURES | Immunoglobulin G - blood | Autoantibodies - blood | Humans | Middle Aged | Male | Survival Rate | Purpura, Thrombotic Thrombocytopenic - mortality | ADAMTS13 Protein - blood | ADAMTS13 Protein - immunology | Disease-Free Survival | Autoantibodies - immunology | Immunoglobulin G - immunology | Adolescent | Aged, 80 and over | Purpura, Thrombotic Thrombocytopenic - immunology | Adult | Female | Aged | Child | Purpura, Thrombotic Thrombocytopenic - blood
AUTOANTIBODIES | DIAGNOSIS | PLASMA-EXCHANGE | EXPERIENCE | IGG ANTIBODIES | MICROANGIOPATHIES | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | REGISTRY | REMISSION | FEATURES | Immunoglobulin G - blood | Autoantibodies - blood | Humans | Middle Aged | Male | Survival Rate | Purpura, Thrombotic Thrombocytopenic - mortality | ADAMTS13 Protein - blood | ADAMTS13 Protein - immunology | Disease-Free Survival | Autoantibodies - immunology | Immunoglobulin G - immunology | Adolescent | Aged, 80 and over | Purpura, Thrombotic Thrombocytopenic - immunology | Adult | Female | Aged | Child | Purpura, Thrombotic Thrombocytopenic - blood
Journal Article
Transfusion, ISSN 0041-1132, 01/2017, Volume 57, Issue 1, pp. 131 - 136
BACKGROUND Patients presenting with acute episodes of thrombotic microangiopathies (TMAs) require urgent access to plasma exchange (PEX). OctaplasLG, a...
HEMOLYTIC-UREMIC SYNDROME | REDUCTION | THERAPEUTIC APHERESIS | GUIDELINES | THROMBOCYTOPENIC PURPURA | COMPLICATIONS | CONTINUOUS-INFUSION | CRYOSUPERNATANT | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | CALCIUM-GLUCONATE | HIV Infections - prevention & control | Plasma | Prospective Studies | Detergents - chemistry | Solvents - chemistry | Humans | Middle Aged | Male | HIV | Plasma Exchange - methods | Prions | Adolescent | Disinfection - methods | Aged, 80 and over | Thrombotic Microangiopathies - therapy | Adult | Female | Aged | Virus Inactivation | Hepatitis B - prevention & control | Hepatitis B virus | Hepatitis C - prevention & control | Hepacivirus | Acute respiratory distress syndrome | Care and treatment | Biological products | Safety and security measures | Hepatitis C | Thromboembolism | Health aspects | Hepatitis | Human immunodeficiency virus--HIV | Catheters | Index Medicus
HEMOLYTIC-UREMIC SYNDROME | REDUCTION | THERAPEUTIC APHERESIS | GUIDELINES | THROMBOCYTOPENIC PURPURA | COMPLICATIONS | CONTINUOUS-INFUSION | CRYOSUPERNATANT | HEMATOLOGY | FACTOR-CLEAVING PROTEASE | CALCIUM-GLUCONATE | HIV Infections - prevention & control | Plasma | Prospective Studies | Detergents - chemistry | Solvents - chemistry | Humans | Middle Aged | Male | HIV | Plasma Exchange - methods | Prions | Adolescent | Disinfection - methods | Aged, 80 and over | Thrombotic Microangiopathies - therapy | Adult | Female | Aged | Virus Inactivation | Hepatitis B - prevention & control | Hepatitis B virus | Hepatitis C - prevention & control | Hepacivirus | Acute respiratory distress syndrome | Care and treatment | Biological products | Safety and security measures | Hepatitis C | Thromboembolism | Health aspects | Hepatitis | Human immunodeficiency virus--HIV | Catheters | Index Medicus
Journal Article
BLOOD, ISSN 0006-4971, 04/2019, Volume 133, Issue 15, pp. 1644 - 1651
Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra-rare thrombomi-croangiopathy caused by an inherited deficiency of a disintegrin and...
IN-VITRO | MANAGEMENT | CLEAVING PROTEASE ADAMTS13 | IGG ANTIBODIES | MUTATIONS | HEMATOLOGY | VON-WILLEBRAND-FACTOR | FRESH-FROZEN PLASMA | UPSHAW-SCHULMAN SYNDROME | DEFICIENCY | REGISTRY
IN-VITRO | MANAGEMENT | CLEAVING PROTEASE ADAMTS13 | IGG ANTIBODIES | MUTATIONS | HEMATOLOGY | VON-WILLEBRAND-FACTOR | FRESH-FROZEN PLASMA | UPSHAW-SCHULMAN SYNDROME | DEFICIENCY | REGISTRY
Journal Article
The New England Journal of Medicine, ISSN 0028-4793, 02/2016, Volume 374, Issue 6, p. 511
The study examines the potential of caplacizumab, an anti-von Willebrand factor humanized single-variable-domain immunoglobulin, in the treatment of acquired...
Thrombocytopenic purpura | Complications and side effects | Care and treatment | Immunoglobulins | Placebos | Dosage and administration | Research
Thrombocytopenic purpura | Complications and side effects | Care and treatment | Immunoglobulins | Placebos | Dosage and administration | Research
Journal Article
Blood, ISSN 0006-4971, 11/2013, Volume 122, Issue 21, pp. 1073 - 1073
Abstract Increasing understanding of abnormalities within the alternative complement pathway in atypical hemolytic uremic syndrome (aHUS) is changing the way...
Journal Article
Annals of the Rheumatic Diseases, ISSN 0003-4967, 03/2014, Volume 73, Issue Suppl 1, pp. A81 - A82
Background and Aims Thrombotic Thrombocytopenic Purpura (TTP) is due to deficiency or dysfunction of the metalloproteinase ADAMTS13, which cleaves von...
Journal Article
British Journal of Anaesthesia, ISSN 0007-0912, 10/2018, Volume 121, Issue 4, pp. 730 - 738
Cancellation of planned surgery impacts substantially on patients and health systems. This study describes the incidence and reasons for cancellation of...
operating room management | Health Services Research | medical resource utilisation | surgery | NEED | VALIDATION | ANESTHESIOLOGY | CRITICAL-CARE | CANCELLATIONS | ELECTIVE SURGERY
operating room management | Health Services Research | medical resource utilisation | surgery | NEED | VALIDATION | ANESTHESIOLOGY | CRITICAL-CARE | CANCELLATIONS | ELECTIVE SURGERY
Journal Article