MCID: THR044
MIFTS: 33

Thrombotic Thrombocytopenic Purpura, Acquired

Categories: Blood diseases, Genetic diseases, Nephrological diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Thrombotic Thrombocytopenic Purpura, Acquired

MalaCards integrated aliases for Thrombotic Thrombocytopenic Purpura, Acquired:

Name: Thrombotic Thrombocytopenic Purpura, Acquired 54 74
Idiopathic Thrombotic Thrombocytopenic Purpura 54 56
Autoimmune Thrombotic Thrombocytopenic Purpura 60 74
Purpura, Thrombotic Thrombocytopenic 54 74
Acquired Thrombotic Thrombocytopenic Purpura 60
Moschowitz Syndrome 54
Acquired Ttp 60
Ttp 54

Characteristics:

Orphanet epidemiological data:

60
acquired thrombotic thrombocytopenic purpura
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:



External Ids:

MESH via Orphanet 46 C536901
ICD10 via Orphanet 35 M31.1
UMLS via Orphanet 75 C2584778
Orphanet 60 ORPHA93585

Summaries for Thrombotic Thrombocytopenic Purpura, Acquired

NIH Rare Diseases : 54 Thrombotic thrombocytopenic purpura (TTP), acquired is a blood disorder characterized by low platelets (i.e., thrombocytopenia), small areas of bleeding under the skin (i.e., purpura), low red blood cell count, and hemolytic anemia. TTP causes blood clots (thrombi) to form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and decrease or stop blood flow to organs such as the brain, kidneys, and heart. Complications may include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems. Hemolytic anemia can lead to paleness, yellowing of the eyes and skin (jaundice), fatigue, shortness of breath, and a rapid heart rate. Acquired TTP usually begins in adulthood, but can affect children. An episode of TTP usually occurs suddenly and lasts for days or weeks, but it may continue for months. Relapses (or flareups) can occur in up to 60 percent of people who have the acquired TTP. Acquired TTP is caused when a person's body mistakingly makes antibodies that block the activity of the ADAMTS13 enzyme. THe ADAMTS13 enzyme normally helps control the activity of certain blood clotting factors. Treatment includes plasma exchange and in some cases may also include corticosteroid therapy or rituximab.

MalaCards based summary : Thrombotic Thrombocytopenic Purpura, Acquired, also known as idiopathic thrombotic thrombocytopenic purpura, is related to thrombotic thrombocytopenic purpura, congenital and thrombotic thrombocytopenic purpura. An important gene associated with Thrombotic Thrombocytopenic Purpura, Acquired is ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13). The drugs Prednisone and rituximab have been mentioned in the context of this disorder. Affiliated tissues include brain, skin and kidney.

Related Diseases for Thrombotic Thrombocytopenic Purpura, Acquired

Diseases in the Thrombotic Thrombocytopenic Purpura family:

Thrombotic Thrombocytopenic Purpura, Congenital Thrombotic Thrombocytopenic Purpura, Acquired

Diseases related to Thrombotic Thrombocytopenic Purpura, Acquired via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 76)
# Related Disease Score Top Affiliating Genes
1 thrombotic thrombocytopenic purpura, congenital 12.3
2 thrombotic thrombocytopenic purpura 10.5
3 hemolytic-uremic syndrome 10.5
4 purpura 10.2
5 systemic lupus erythematosus 10.1
6 thrombocytopenia 10.1
7 pancreatitis 10.1
8 lupus erythematosus 10.1
9 wiskott-aldrich syndrome 10.1
10 hepatitis 10.1
11 inflammatory bowel disease 10.1
12 multicentric castleman disease 10.1
13 castleman disease 10.1
14 hepatitis c 10.1
15 juvenile rheumatoid arthritis 10.1
16 myocardial infarction 10.1
17 hemolytic anemia 10.1
18 pernicious anemia 10.1
19 sjogren syndrome 10.1
20 infantile liver failure syndrome 1 10.1
21 deficiency anemia 10.1
22 chronic myelomonocytic leukemia 10.1
23 placenta disease 10.1
24 pdgfrb-associated chronic eosinophilic leukemia 10.1
25 vitamin e, familial isolated deficiency of 10.0
26 trichotillomania 10.0
27 leukemia 10.0
28 colitis 10.0
29 leptospirosis 10.0
30 human immunodeficiency virus infectious disease 10.0
31 ataxia with vitamin e deficiency 10.0
32 heparin-induced thrombocytopenia 10.0
33 myeloma, multiple 10.0
34 crohn's disease 10.0
35 eclampsia 10.0
36 chickenpox 10.0
37 breast cancer 9.8
38 hepatocellular carcinoma 9.8
39 multiple sclerosis 9.8
40 rheumatoid arthritis 9.8
41 thrombocytopenic purpura, autoimmune 9.8
42 buerger disease 9.8
43 cystic fibrosis 9.8
44 hemolytic uremic syndrome, atypical 1 9.8
45 cervical cancer 9.8
46 salla disease 9.8
47 myelodysplastic syndrome 9.8
48 arthritis 9.8
49 glomerulonephritis 9.8
50 autoimmune pancreatitis 9.8

Graphical network of the top 20 diseases related to Thrombotic Thrombocytopenic Purpura, Acquired:



Diseases related to Thrombotic Thrombocytopenic Purpura, Acquired

Symptoms & Phenotypes for Thrombotic Thrombocytopenic Purpura, Acquired

Drugs & Therapeutics for Thrombotic Thrombocytopenic Purpura, Acquired

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

(show top 50) (show all 60)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 3,Phase 2 53-03-2 5865
2
rituximab Approved Phase 3,Phase 2,Phase 1 174722-31-7 10201696
3
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
4
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 3 7487-88-9 24083
5
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
6 Immunologic Factors Phase 3,Phase 2,Phase 1
7 Antirheumatic Agents Phase 3,Phase 2,Phase 1
8 Antineoplastic Agents, Immunological Phase 3,Phase 2,Phase 1
9 Anti-Inflammatory Agents Phase 3
10 Antineoplastic Agents, Hormonal Phase 3
11 Immunosuppressive Agents Phase 3
12 Dermatologic Agents Phase 3
13 Antifungal Agents Phase 3
14 Calcineurin Inhibitors Phase 3
15 glucocorticoids Phase 3
16 Anti-Infective Agents Phase 3,Early Phase 1
17 Hormone Antagonists Phase 3,Phase 2
18 Cyclosporins Phase 3
19 Hormones Phase 3,Phase 2
20 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
21 Central Nervous System Depressants Phase 3
22 Anti-Arrhythmia Agents Phase 3
23 Anticonvulsants Phase 3
24 calcium channel blockers Phase 3
25 Peripheral Nervous System Agents Phase 3
26 Tocolytic Agents Phase 3
27 Analgesics Phase 3
28 Anesthetics Phase 3
29 Calcium, Dietary Phase 3
30
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
31
Acetaminophen Approved Phase 2 103-90-2 1983
32
Promethazine Approved, Investigational Phase 2 60-87-7 4927
33
Danazol Approved Phase 2 17230-88-5 28417
34 Pharmaceutical Solutions Phase 2
35 Natriuretic Agents Phase 2,Not Applicable
36 Arginine Vasopressin Phase 2
37 arginine Phase 2
38 Hemostatics Phase 2
39 Coagulants Phase 2
40 Vasopressins Phase 2
41 Deamino Arginine Vasopressin Phase 2
42 Immunoglobulin G Phase 1, Phase 2
43 Immunoglobulins Phase 1, Phase 2
44 Antibodies Phase 1, Phase 2
45 Estrogen Antagonists Phase 2
46 Estrogens Phase 2
47 Estrogen Receptor Antagonists Phase 2
48
Acetylcysteine Approved, Investigational Early Phase 1 616-91-1 12035
49
Infliximab Approved 170277-31-3
50 N-monoacetylcystine Early Phase 1

Interventional clinical trials:

(show all 35)
# Name Status NCT ID Phase Drugs
1 Prophylactic Plasma Infusion Therapy for Congenital Thrombotic Thrombocytopenic Purpura Withdrawn NCT01754545 Phase 4 Octaplas infusion and placebo (group 1);Octaplas infusion and placebo (group 2)
2 Phase III Trial With Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura Completed NCT02553317 Phase 3
3 Study of Cyclosporine or Corticosteroids as an Adjunct to Plasma Exchange in Thrombotic Thrombocytopenic Purpura (TTP) Completed NCT00713193 Phase 3 Cyclosporine;Prednisone
4 Follow-up Study for Patients Who Completed Study ALX0681-C301 (Post-HERCULES) Active, not recruiting NCT02878603 Phase 3
5 A Phase 3, Randomized, Controlled Study of Prophylactic and On-demand Treatment of cTTP With BAX 930 (rADAMTS13) Active, not recruiting NCT03393975 Phase 3
6 Magnesium Sulfate in Thrombotic Thrombocytopenic Purpura in Intensive Care Not yet recruiting NCT03237819 Phase 3 Sulfate, Magnesium;Placebo - Concentrate
7 Evaluating the Effectiveness of Adding Rituximab to Standard Treatment for Thrombotic Thrombocytopenic Purpura (TTP) Terminated NCT00799773 Phase 3 Rituximab;Corticosteroids
8 Safety and Efficacy Study to Compare Uniplas With Cryosupernatant Plasma in Thrombotic Thrombocytopenic Purpura (TTP) Terminated NCT00411801 Phase 3
9 The Plasma Large-Volume Exchange RCT Withdrawn NCT01433003 Phase 3
10 The Use of Rituximab in Acute Thrombotic Thrombocytopenic Purpura (TTP) Unknown status NCT00937131 Phase 2 Rituximab
11 Rituximab in Patients With Relapsed or Refractory TTP-HUS Unknown status NCT00531089 Phase 2 Rituximab
12 Rituximab in Adult Acquired Idiopathic Thrombotic Thrombocytopenic Purpura Completed NCT00907751 Phase 2 rituximab
13 Study to Assess Efficacy and Safety of Anti-von Willebrand Factor Nanobody in Patients With Acquired Thrombotic Thrombocytopenic Purpura (TTP) Completed NCT01151423 Phase 2
14 ARC1779 Injection in Patients With Von Willebrand Factor-Related Platelet Function Disorders Completed NCT00632242 Phase 2 ARC1779;ARC1779;ARC1779;ARC1779;ARC1779
15 Low Dose Rituximab in Thrombotic Thrombocytopenic Purpura Active, not recruiting NCT01554514 Phase 2
16 Study of rADAMTS-13 (SHP655) in the Treatment of Participants With Acquired Thrombotic Thrombocytopenic Purpura (aTTP) Not yet recruiting NCT03922308 Phase 2 SHP655
17 IdeS in Asymptomatic Asymptomatic Antibody-Mediated Thrombotic Thrombocytopenic Purpura (TTP) Patients Terminated NCT02854059 Phase 1, Phase 2
18 Safety Study of Danazol With Plasma Exchange and Steroids for the Treatment of Thrombotic Thrombocytopenic Purpura (TTP) Terminated NCT00953771 Phase 2 Danazol
19 Clinical Outcome Study of ARC1779 Injection in Patients With Thrombotic Microangiopathy Terminated NCT00726544 Phase 2 ARC 1779 Placebo;ARC1779 Injection;ARC1779 Injection;ARC1779 Injection
20 Use of Rituximab Treatment in Addition to Standard Care for Newly Presenting Thrombotic Thrombocytopenic Purpura Withdrawn NCT00251277 Phase 1, Phase 2 Rituximab
21 Phase 1 Dose Escalation, Single Dose Study to Assess Safety and Pharmacokinetics of BAX930 in Hereditary Thrombotic Thrombocytopenic Purpura (TTP) Completed NCT02216084 Phase 1 Recombinant ADAMTS13
22 Influence of Extracorporeal Circulation During Cardiac Surgery on the Development of Postoperative Thrombotic Thrombocytopenic Purpura (TTP) Unknown status NCT01300117
23 A Pilot Study of N-acetylcysteine in Thrombotic Thrombocytopenia Purpura Completed NCT01808521 Early Phase 1 N-Acetylcysteine
24 ADAMTS13 in Thrombotic Thrombocytopenic Purpura Completed NCT00426686
25 European Safety Registry in Ulcerative Colitis (P04808) Completed NCT00705484 Standard Therapy
26 Early Predictive Factors of Cardiac and Cerebral Involvement in TMA Completed NCT02134171 Not Applicable
27 The United Kingdom Thrombotic Thrombocytopenic Purpura Registry (UK TTP Registry) Recruiting NCT03832881
28 Prospective Psychometric Evaluation Study of a Patient-reported Outcomes (PRO) Instrument for Congenital Thrombotic Thrombocytopenic Purpura (cTTP, Upshaw-Schulman Syndrome [USS], Hereditary Thrombotic Thrombocytopenic Purpura [hTTP] Recruiting NCT03519672
29 Observational Study of the Use of octaplasLG®. Recruiting NCT03369314 octaplasLG®
30 A Prospective Study on the Long-Term Vascular Burden in TTP Patients Recruiting NCT03187652
31 Octaplas Adult TTP Trial Recruiting NCT01938404 Standard Plasma
32 Genotype and Phenotype Correlation in Hereditary Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome) Recruiting NCT01257269
33 TTP and aHUS in Complicated Pregnancies Recruiting NCT03605511
34 International Registry and Biorepository for TMA(Thrombotic Microangiopathy) Terminated NCT00593229
35 The Role of Microparticles as a Biomarker Withdrawn NCT02626663

Search NIH Clinical Center for Thrombotic Thrombocytopenic Purpura, Acquired

Genetic Tests for Thrombotic Thrombocytopenic Purpura, Acquired

Anatomical Context for Thrombotic Thrombocytopenic Purpura, Acquired

MalaCards organs/tissues related to Thrombotic Thrombocytopenic Purpura, Acquired:

42
Brain, Skin, Kidney, Eye, Heart, B Cells, Endothelial

Publications for Thrombotic Thrombocytopenic Purpura, Acquired

Articles related to Thrombotic Thrombocytopenic Purpura, Acquired:

(show top 50) (show all 65)
# Title Authors Year
1
Acquired autoimmune thrombotic thrombocytopenic purpura. ( 30586046 )
2019
2
ADDITIONAL AUTOIMMUNE DISORDERS IN PATIENTS WITH ACQUIRED AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA. ( 30895642 )
2019
3
Acquired autoimmune thrombotic thrombocytopenic purpura. ( 30531363 )
2019
4
Concentration and Subclass Distribution of Anti-ADAMTS13 IgG Autoantibodies in Different Stages of Acquired Idiopathic Thrombotic Thrombocytopenic Purpura. ( 30061898 )
2018
5
Complement Regulatory Genetic Mutations in the Setting of Autoimmune Thrombotic Thrombocytopenic Purpura: A Case Series. ( 30225434 )
2018
6
Relapse Rate in Survivors of Acute Autoimmune Thrombotic Thrombocytopenic Purpura Treated with or without Rituximab. ( 30235478 )
2018
7
ASFA Category IV becomes Category I: Idiopathic thrombotic thrombocytopenic purpura in a patient with presumed gemcitabine-induced thrombotic microangiopathy. ( 28940604 )
2018
8
Autoimmune thrombotic thrombocytopenic purpura associated with HHV8-related Multicentric Castleman disease. ( 27221364 )
2017
9
Acquired idiopathic thrombotic thrombocytopenic purpura successfully treated with intravenous immunoglobulin and glucocorticoid: A case report. ( 28383422 )
2017
10
Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis. ( 28791286 )
2017
11
The role of human leukocyte antigen DRB1-DQB1 haplotypes in the susceptibility to acquired idiopathic thrombotic thrombocytopenic purpura. ( 27866840 )
2017
12
Treatment of autoimmune thrombotic thrombocytopenic purpura in the more severe forms. ( 28110841 )
2017
13
Idiopathic thrombotic thrombocytopenic purpura: strongest risk factor for relapse from remission is having had a relapse. ( 27514869 )
2016
14
Acquired autoimmune thrombotic thrombocytopenic purpura in a case of severe haemophilia A. ( 27704646 )
2016
15
Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. ( 26608617 )
2016
16
Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story. ( 26293834 )
2015
17
An autopsy case of myocardial infarction due to idiopathic thrombotic thrombocytopenic purpura. ( 26022055 )
2015
18
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. ( 26173755 )
2015
19
Cocaine-induced microangiopathic hemolytic anemia mimicking idiopathic thrombotic thrombocytopenic purpura: a case report and review of the literature. ( 24753113 )
2014
20
Successful treatment of relapsing autoimmune thrombotic thrombocytopenic purpura with rituximab. ( 25521977 )
2014
21
Circulating endothelial cells and progenitors as prognostic factors during autoimmune thrombotic thrombocytopenic purpura: results of a prospective multicenter French study. ( 25088020 )
2014
22
Successful treatment of an elderly frail patient with acquired idiopathic thrombotic thrombocytopenic purpura under close monitoring of ADAMTS13 activity and anti-ADAMTS13 antibody titers. ( 24517874 )
2014
23
Interferon-α is not elevated in idiopathic thrombotic thrombocytopenic purpura patients. ( 24764259 )
2014
24
Seasonal distribution of severe ADAMTS13 deficient idiopathic thrombotic thrombocytopenic purpura. ( 24123112 )
2014
25
Bortezomib induces clinical remission and reduction of ADAMTS13 inhibitory antibodies in relapsed refractory idiopathic thrombotic thrombocytopenic purpura. ( 24345005 )
2014
26
Measurement and prevalence of circulating ADAMTS13-specific immune complexes in autoimmune thrombotic thrombocytopenic purpura. ( 24354764 )
2014
27
A beneficial effect of rituximab on autoimmune thrombotic thrombocytopenic purpura: just a B-cell depletion? ( 24365135 )
2014
28
Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature. ( 23121663 )
2013
29
Cord blood transplantation combined with rituximab for Wiskott-Aldrich syndrome with autoimmune thrombotic thrombocytopenic purpura. ( 23498591 )
2013
30
Treatment of chronic hepatitis C with pegylated interferon-α in a patient with recurrent autoimmune thrombotic thrombocytopenic purpura. ( 23106108 )
2013
31
Health care utilization of patients diagnosed with idiopathic thrombotic thrombocytopenic purpura in a commercially insured population in the United States. ( 22780943 )
2012
32
Autoimmune disorders in patients with idiopathic thrombotic thrombocytopenic purpura. ( 22960981 )
2012
33
Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. ( 22409250 )
2012
34
Case report: use of thienopyridines in a patient with acquired idiopathic thrombotic thrombocytopenic purpura. ( 22450533 )
2012
35
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis. ( 22547089 )
2012
36
Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange. ( 22552883 )
2012
37
Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. ( 22580997 )
2012
38
The role of human leukocyte antigens as predisposing and/or protective factors in patients with idiopathic thrombotic thrombocytopenic purpura. ( 22203269 )
2012
39
ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome. ( 21606162 )
2011
40
B cell activating factor is elevated in acute idiopathic thrombotic thrombocytopenic purpura. ( 21615379 )
2011
41
Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab. ( 20576013 )
2010
42
Successful long-term rituximab maintenance for a relapsing patient with idiopathic thrombotic thrombocytopenic purpura. ( 20609201 )
2010
43
Rituximab for managing relapsing or refractory patients with idiopathic thrombotic thrombocytopenic purpura--haemolytic uraemic syndrome. ( 20671883 )
2010
44
Pathogenesis and treatment of acquired idiopathic thrombotic thrombocytopenic purpura. ( 20807984 )
2010
45
A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13. ( 20121555 )
2010
46
HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians. ( 20141578 )
2010
47
Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. ( 20175870 )
2010
48
Validation of claims-based diagnostic codes for idiopathic thrombotic thrombocytopenic purpura in a commercially-insured population. ( 20352159 )
2010
49
Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura. ( 20378566 )
2010
50
Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab. ( 20422413 )
2010

Variations for Thrombotic Thrombocytopenic Purpura, Acquired

Expression for Thrombotic Thrombocytopenic Purpura, Acquired

Search GEO for disease gene expression data for Thrombotic Thrombocytopenic Purpura, Acquired.

Pathways for Thrombotic Thrombocytopenic Purpura, Acquired

GO Terms for Thrombotic Thrombocytopenic Purpura, Acquired

Sources for Thrombotic Thrombocytopenic Purpura, Acquired

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