ATTP
MCID: IMM236
MIFTS: 23

Immune-Mediated Thrombotic Thrombocytopenic Purpura (ATTP)

Categories: Blood diseases, Immune diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Immune-Mediated Thrombotic Thrombocytopenic Purpura

MalaCards integrated aliases for Immune-Mediated Thrombotic Thrombocytopenic Purpura:

Name: Immune-Mediated Thrombotic Thrombocytopenic Purpura 58
Thrombotic Thrombocytopenic Purpura Due to Anti-Adamts-13 Antibodies 58
Autoimmune Thrombotic Thrombocytopenic Purpura 58
Acquired Thrombotic Thrombocytopenic Purpura 58
Acquired Ttp 58
Attp 58
Ittp 58

Characteristics:

Orphanet epidemiological data:

58
immune-mediated thrombotic thrombocytopenic purpura
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 58  
Rare renal diseases
Rare haematological diseases


External Ids:

MESH via Orphanet 45 C536901
ICD10 via Orphanet 33 M31.1
UMLS via Orphanet 72 C2584778
Orphanet 58 ORPHA93585

Summaries for Immune-Mediated Thrombotic Thrombocytopenic Purpura

MalaCards based summary : Immune-Mediated Thrombotic Thrombocytopenic Purpura, also known as thrombotic thrombocytopenic purpura due to anti-adamts-13 antibodies, is related to thrombotic thrombocytopenic purpura, acquired and thrombotic thrombocytopenic purpura. An important gene associated with Immune-Mediated Thrombotic Thrombocytopenic Purpura is ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13). The drugs Prednisolone acetate and Prednisolone phosphate have been mentioned in the context of this disorder.

Related Diseases for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Diseases related to Immune-Mediated Thrombotic Thrombocytopenic Purpura via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 72)
# Related Disease Score Top Affiliating Genes
1 thrombotic thrombocytopenic purpura, acquired 11.3
2 thrombotic thrombocytopenic purpura 10.9
3 purpura 10.9
4 thrombocytopenia 10.5
5 thrombotic microangiopathy 10.5
6 hemolytic anemia 10.4
7 hemolytic uremic syndrome, atypical 1 10.3
8 thrombotic thrombocytopenic purpura, hereditary 10.3
9 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
10 pre-eclampsia 10.3
11 signet ring cell adenocarcinoma 10.3
12 hemophilia 10.3
13 autoimmune disease 10.2
14 systemic lupus erythematosus 10.2
15 ocular motor apraxia 10.2
16 hemolytic-uremic syndrome 10.2
17 lupus erythematosus 10.2
18 pulmonary embolism 10.2
19 47,xyy 10.2
20 multiple sclerosis 10.1
21 factor viii deficiency 10.1
22 thrombocytopenic purpura, autoimmune 10.1
23 wiskott-aldrich syndrome 10.1
24 hemophilia a 10.1
25 anxiety 10.1
26 inflammatory bowel disease 10.1
27 covid-19 10.1
28 multicentric castleman disease 10.1
29 castleman disease 10.1
30 hepatitis c 10.1
31 juvenile rheumatoid arthritis 10.1
32 arthritis 10.1
33 rheumatoid factor-negative juvenile idiopathic arthritis 10.1
34 depression 10.1
35 aortic aneurysm, familial abdominal, 1 10.1
36 pernicious anemia 10.1
37 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.1
38 hypertensive nephropathy 10.1
39 aplastic anemia 10.1
40 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 10.1
41 cholangiocarcinoma 10.1
42 proteinuria, chronic benign 10.1
43 deficiency anemia 10.1
44 pulmonary hypertension 10.1
45 cardiac arrest 10.1
46 chronic myelomonocytic leukemia 10.1
47 disseminated intravascular coagulation 10.1
48 pyelonephritis 10.1
49 allergic disease 10.1
50 von willebrand's disease 10.1

Graphical network of the top 20 diseases related to Immune-Mediated Thrombotic Thrombocytopenic Purpura:



Diseases related to Immune-Mediated Thrombotic Thrombocytopenic Purpura

Symptoms & Phenotypes for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Drugs & Therapeutics for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Drugs for Immune-Mediated Thrombotic Thrombocytopenic Purpura (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 34)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
2
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
3
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
4
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
5
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
6
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
7
Heparin Approved, Investigational Phase 3 9005-49-6 772 9812414
8
Dalteparin Approved Phase 3 9005-49-6
9
Enoxaparin Approved Phase 3 9005-49-6 772
10
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
11
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
12 Pharmaceutical Solutions Phase 2, Phase 3
13 Protective Agents Phase 2, Phase 3
14 Antineoplastic Agents, Immunological Phase 2, Phase 3
15 Gastrointestinal Agents Phase 2, Phase 3
16 Anti-Inflammatory Agents Phase 2, Phase 3
17 Hormone Antagonists Phase 2, Phase 3
18 glucocorticoids Phase 2, Phase 3
19 Immunologic Factors Phase 2, Phase 3
20 Hormones Phase 2, Phase 3
21 Antirheumatic Agents Phase 2, Phase 3
22 Neuroprotective Agents Phase 2, Phase 3
23 Methylprednisolone Acetate Phase 2, Phase 3
24 Antineoplastic Agents, Hormonal Phase 2, Phase 3
25 Immunosuppressive Agents Phase 2, Phase 3
26 Antiemetics Phase 2, Phase 3
27 Fibrinolytic Agents Phase 3
28 Heparin, Low-Molecular-Weight Phase 3
29 Calcium, Dietary Phase 3
30 Anticoagulants Phase 3
31 calcium heparin Phase 3
32 Thromboplastin Phase 3
33
Calcium Nutraceutical Phase 3 7440-70-2 271
34
Fibrinolysin Investigational Phase 2 9004-09-5

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prospective Follow-up Study for Patients Who Completed Study ALX0681-C301 (HERCULES) to Evaluate Long-term Safety and Efficacy of Caplacizumab (Post-HERCULES) Completed NCT02878603 Phase 3
2 A Phase III Double-blind, Randomized, Parallel Group, Multicenter Placebo-controlled Trial to Study the Efficacy and Safety of Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura Completed NCT02553317 Phase 3
3 An Open-label Multicenter Trial to Study the Efficacy and Safety of Caplacizumab in Japanese Patients With Acquired Thrombotic Thrombocytopenic Purpura Recruiting NCT04074187 Phase 2, Phase 3 Caplacizumab (ALX-0081);Plasma exchange (PE);Corticosteroid treatment (Methylprednisolone or prednisolone);Immunosuppressive treatment (eg, rituximab)
4 Clinical Efficacy of Heparin and Tocilizumab in Patients With Severe COVID-19 Infection: a Randomized Clinical Trial Not yet recruiting NCT04600141 Phase 3 Tocilizumab;Heparin - Therapeutic dosage;Heparin - Prophylactic dosage
5 A Phase II, Single-blind, Randomized, Placebo-controlled Trial to Study the Efficacy and Safety of Anti-von Willebrand Factor Nanobody Administered as Adjunctive Treatment to Patients With Acquired Thrombotic Thrombocytopenic Purpura Completed NCT01151423 Phase 2
6 Adjuvant Low Dose Rituximab for Acquired TTP With Severe ADAMTS13 Deficiency Completed NCT01554514 Phase 2
7 A Phase 2, Multicenter, Randomized, Placebo-Controlled, Double-blind Study in Patients With Acquired Thrombotic Thrombocytopenic Purpura (aTTP) to Evaluate the Pharmacokinetics,Safety and Efficacy of rADAMTS-13 (SHP655) Administered in Addition to Standard Of Care (SoC) Treatment Recruiting NCT03922308 Phase 2 SHP655
8 Efficacy of a Personalized Caplacizumab Regimen Based on ADAMTS13 Activity Monitoring in Adult Acquired Thrombotic Thrombocytopenic Purpura: A Phase II, Multicenter Non-inferiority Single-arm Study. Not yet recruiting NCT04720261 Phase 2 Caplacizumab
9 A Multicenter, Randomized, Double-blind, Placebo-parallel, Phase II Clinical Trial of the Efficacy and Safety of Anfibatide in Treating Patients With Acquired Thrombotic Thrombocytopenic Purpura (TTP) Not yet recruiting NCT04021173 Phase 2 Anfibatide;Placebos
10 Studying the Effectiveness of Remote Training Recruiting NCT03164278

Search NIH Clinical Center for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Genetic Tests for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Anatomical Context for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Publications for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Articles related to Immune-Mediated Thrombotic Thrombocytopenic Purpura:

(show top 50) (show all 51)
# Title Authors PMID Year
1
Effective and safe off-label use of caplacizumab treatment in a middle-aged obese male. 61
33285299 2021
2
Identification of a novel genetic locus associated with immune mediated thrombotic thrombocytopenic purpura. 61
33596643 2021
3
Immature platelet dynamics correlate with ADAMTS13 deficiency and predict therapy response in immune-mediated thrombotic thrombocytopenic purpura. 61
33290885 2021
4
REDEFINING OUTCOMES IN IMMUNE TTP: AN INTERNATIONAL WORKING GROUP CONSENSUS REPORT. 61
33529333 2021
5
A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP. 61
33150928 2021
6
N-glycan mediated shielding of ADAMTS13 prevents binding of pathogenic autoantibodies in immune-mediated TTP. 61
33544829 2021
7
Should all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab? 61
33236389 2021
8
Efficacy of subcutaneous preemptive rituximab in immune-mediated thrombotic thrombocytopenic purpura: Experience from the first 12 cases. 61
33068040 2021
9
Intensive rituximab regimen in immune-mediated thrombotic thrombocytopenic purpura can circumvent unresponsiveness to standard rituximab treatment. 61
33216951 2021
10
Laboratory surveillance of immune-mediated thrombotic thrombocytopenic purpura. 61
33275690 2020
11
[Venous thrombo-embolism during immune-mediated thrombotic thrombocytopenic purpura is prevalent in patients with a prolonged treatment with therapeutic plasma exchange]. 61
32727695 2020
12
Exome Sequencing Identifies Abnormalities in Glycosylation and ANKRD36C in Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura. 61
33184803 2020
13
Elevated plasma levels of syndecan-1 and soluble thrombomodulin predict adverse outcomes in thrombotic thrombocytopenic purpura. 61
33141886 2020
14
Immunogenic hotspots in the spacer domain of ADAMTS13 in immune-mediated thrombotic thrombocytopenic purpura. 61
33171004 2020
15
Real-World Evidence of Caplacizumab Use in the Management of Acute TTP. 61
33150355 2020
16
Clinical outcomes and risk factors for severe COVID-19 in patients with haematological disorders receiving chemo- or immunotherapy. 61
32678948 2020
17
Long-term cognitive impairments following recovery in the patients with immune-mediated thrombotic thrombocytopenic purpura. 61
33090486 2020
18
The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians. 61
33096882 2020
19
Development and validation of a prediction model (AHC) for early identification of refractory thrombotic thrombocytopenic purpura using nationally representative data. 61
32452543 2020
20
Hemophagocytic lymphohistiocytosis in a patient with COVID-19 treated with tocilizumab: a case report. 61
33054818 2020
21
Co(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. 61
32929962 2020
22
Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study. 61
32859237 2020
23
Low 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. 61
32350923 2020
24
Generation and validation of small ADAMTS13 fragments for epitope mapping of anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura. 61
32685903 2020
25
Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura. 61
32356859 2020
26
HLA loci predisposing to immune TTP in Japanese: potential role of the shared ADAMTS13 peptide bound to different HLA-DR. 61
32253422 2020
27
Anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura do not hamper ELISA-based quantification of ADAMTS13 antigen. 61
31989742 2020
28
Immune-Mediated Thrombotic Thrombocytopenic Purpura: A Narrative Review of Diagnosis and Treatment in Adults. 61
32259874 2020
29
Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes. 61
33061729 2020
30
Amotosalen-inactivated fresh frozen plasma is comparable to solvent-detergent inactivated plasma to treat thrombotic thrombocytopenic purpura. 61
31740165 2019
31
[Effect of Recombinant Protein in the Spacer Domain on ADAMTS13 Activity]. 61
31607318 2019
32
Risk factors associated with the human leucocyte antigen system in Lebanese patients with immune-mediated thrombotic thrombocytopenic purpura. 61
31672454 2019
33
Obinutuzumab in two patients suffering from immune-mediated thrombotic thrombocytopenic purpura intolerant to rituximab. 61
31321811 2019
34
How targeted therapy disrupts the treatment paradigm for acquired TTP: the risks, benefits, and unknowns. 61
31217190 2019
35
Caplacizumab to treat immune-mediated thrombotic thrombocytopenic purpura. 61
31250841 2019
36
Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura. 61
31092093 2019
37
Comparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura. 61
30919938 2019
38
Generation of anti-idiotypic antibodies to detect anti-spacer antibody idiotopes in acute thrombotic thrombocytopenic purpura patients. 61
30523052 2019
39
Predictors of relapse and efficacy of rituximab in immune thrombotic thrombocytopenic purpura. 61
31076407 2019
40
Clinical factors and biomarkers predict outcome in patients with immune-mediated thrombotic thrombocytopenic purpura. 61
30171022 2019
41
Transfusion 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. 61
30354235 2018
42
Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura. 61
30201758 2018
43
Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura. 61
30235483 2018
44
Rituximab Prevents Stroke Recurrences in Atypical Chronic Immune-Mediated Thrombotic Thrombocytopenic Purpura. 61
31249968 2018
45
Bethesda Assay for Detecting Inhibitory Anti-ADAMTS13 Antibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura. 61
31249957 2018
46
Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura: interplay between genes and environmental triggers. 61
29674502 2018
47
Dasatinib-induced immune mediated-thrombotic thrombocytopenic purpura. 61
29475747 2018
48
Anti-ADAMTS13 Antibodies and a Novel Heterozygous p.R1177Q Mutation in a Case of Pregnancy-Onset Immune-Mediated Thrombotic Thrombocytopenic Purpura. 61
31249923 2018
49
Cyclosporine or steroids as an adjunct to plasma exchange in the treatment of immune-mediated thrombotic thrombocytopenic purpura. 61
29296854 2017
50
Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura. 61
28576877 2017

Variations for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Expression for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Search GEO for disease gene expression data for Immune-Mediated Thrombotic Thrombocytopenic Purpura.

Pathways for Immune-Mediated Thrombotic Thrombocytopenic Purpura

GO Terms for Immune-Mediated Thrombotic Thrombocytopenic Purpura

Sources for Immune-Mediated Thrombotic Thrombocytopenic Purpura

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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