MCID: THR044
MIFTS: 34

Thrombotic Thrombocytopenic Purpura, Acquired

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

Aliases & Classifications for Thrombotic Thrombocytopenic Purpura, Acquired

MalaCards integrated aliases for Thrombotic Thrombocytopenic Purpura, Acquired:

Name: Thrombotic Thrombocytopenic Purpura, Acquired 53 73
Idiopathic Thrombotic Thrombocytopenic Purpura 53 55
Autoimmune Thrombotic Thrombocytopenic Purpura 59 73
Purpura, Thrombotic Thrombocytopenic 53 73
Acquired Thrombotic Thrombocytopenic Purpura 59
Moschowitz Syndrome 53
Acquired Ttp 59
Ttp 53

Characteristics:

Orphanet epidemiological data:

59
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:

Orphanet 59 ORPHA93585
ICD10 via Orphanet 34 M31.1
MESH via Orphanet 45 C536901
UMLS via Orphanet 74 C2584778

Summaries for Thrombotic Thrombocytopenic Purpura, Acquired

NIH Rare Diseases : 53 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 and purpura. An important gene associated with Thrombotic Thrombocytopenic Purpura, Acquired is ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13). The drugs rituximab and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include brain, skin and eye.

Related Diseases for Thrombotic Thrombocytopenic Purpura, Acquired

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 52)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 3,Phase 2,Phase 1 174722-31-7 10201696
2
Prednisone Approved, Vet_approved Phase 3,Phase 2 53-03-2 5865
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 Antirheumatic Agents Phase 3,Phase 2,Phase 1
6 glucocorticoids Phase 3
7 Cyclosporins Phase 3
8 Hormone Antagonists Phase 3,Phase 2
9 Dermatologic Agents Phase 3
10 Hormones Phase 3,Phase 2
11 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
12 Immunosuppressive Agents Phase 3
13 Anti-Infective Agents Phase 3,Early Phase 1
14 Antifungal Agents Phase 3
15 Anti-Inflammatory Agents Phase 3
16 Calcineurin Inhibitors Phase 3
17 Antineoplastic Agents, Hormonal Phase 3
18 Analgesics Phase 3
19 calcium channel blockers Phase 3
20 Central Nervous System Depressants Phase 3
21 Tocolytic Agents Phase 3
22 Anesthetics Phase 3
23 Anti-Arrhythmia Agents Phase 3
24 Peripheral Nervous System Agents Phase 3
25 Anticonvulsants Phase 3
26 Calcium, Dietary Phase 3
27
Acetaminophen Approved Phase 2 103-90-2 1983
28
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
29
Promethazine Approved, Investigational Phase 2 60-87-7 4927
30
Danazol Approved Phase 2 17230-88-5 28417
31 Pharmaceutical Solutions Phase 2
32 Natriuretic Agents Phase 2,Not Applicable
33 Coagulants Phase 2
34 Hemostatics Phase 2
35 Deamino Arginine Vasopressin Phase 2
36 Vasopressins Phase 2
37 Arginine Vasopressin Phase 2
38 Estrogen Antagonists Phase 2
39 Estrogens Phase 2
40 Immunoglobulin G Phase 1, Phase 2
41 Antibodies Phase 1, Phase 2
42 Immunoglobulins Phase 1, Phase 2
43 arginine Nutraceutical Phase 2
44
Acetylcysteine Approved, Investigational Early Phase 1 616-91-1 12035
45 Respiratory System Agents Early Phase 1
46 Natriuretic Peptide, Brain Not Applicable
47 N-monoacetylcystine Early Phase 1
48 Expectorants Early Phase 1
49 Antidotes Early Phase 1
50 Antioxidants Early Phase 1

Interventional clinical trials:

(show all 31)
# 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 Recruiting NCT01554514 Phase 2
16 Safety Study of Danazol With Plasma Exchange and Steroids for the Treatment of Thrombotic Thrombocytopenic Purpura (TTP) Recruiting NCT00953771 Phase 2 Danazol
17 IdeS in Asymptomatic Asymptomatic Antibody-Mediated Thrombotic Thrombocytopenic Purpura (TTP) Patients Terminated NCT02854059 Phase 1, Phase 2
18 Clinical Outcome Study of ARC1779 Injection in Patients With Thrombotic Microangiopathy Terminated NCT00726544 Phase 2 ARC 1779 Placebo;ARC1779 Injection;ARC1779 Injection;ARC1779 Injection
19 Use of Rituximab Treatment in Addition to Standard Care for Newly Presenting Thrombotic Thrombocytopenic Purpura Withdrawn NCT00251277 Phase 1, Phase 2 Rituximab
20 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
21 Influence of Extracorporeal Circulation During Cardiac Surgery on the Development of Postoperative Thrombotic Thrombocytopenic Purpura (TTP) Unknown status NCT01300117
22 A Pilot Study of N-acetylcysteine in Thrombotic Thrombocytopenia Purpura Completed NCT01808521 Early Phase 1 N-Acetylcysteine
23 ADAMTS13 in Thrombotic Thrombocytopenic Purpura Completed NCT00426686
24 Early Predictive Factors of Cardiac and Cerebral Involvement in TMA Completed NCT02134171 Not Applicable
25 Observational Study of the Use of octaplasLG®. Recruiting NCT03369314 octaplasLG®
26 A Prospective Study on the Long-Term Vascular Burden in TTP Patients Recruiting NCT03187652
27 Octaplas Adult TTP Trial Recruiting NCT01938404 Standard Plasma
28 Genotype and Phenotype Correlation in Hereditary Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome) Recruiting NCT01257269
29 The Role of Microparticles as a Biomarker Enrolling by invitation NCT02626663
30 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] Not yet recruiting NCT03519672
31 International Registry and Biorepository for TMA(Thrombotic Microangiopathy) Terminated NCT00593229

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:

41
Brain, Skin, Eye, Kidney, Heart, B Cells, Lung

Publications for Thrombotic Thrombocytopenic Purpura, Acquired

Articles related to Thrombotic Thrombocytopenic Purpura, Acquired:

(show all 34)
# Title Authors Year
1
ASFA Category IV becomes Category I: Idiopathic thrombotic thrombocytopenic purpura in a patient with presumed gemcitabine-induced thrombotic microangiopathy. ( 28940604 )
2017
2
The role of human leukocyte antigen DRB1-DQB1 haplotypes in the susceptibility to acquired idiopathic thrombotic thrombocytopenic purpura. ( 27866840 )
2017
3
Idiopathic thrombotic thrombocytopenic purpura: strongest risk factor for relapse from remission is having had a relapse. ( 27514869 )
2016
4
Bortezomib induces clinical remission and reduction of ADAMTS13 inhibitory antibodies in relapsed refractory idiopathic thrombotic thrombocytopenic purpura. ( 24345005 )
2013
5
Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature. ( 23121663 )
2013
6
Seasonal distribution of severe ADAMTS13 deficient idiopathic thrombotic thrombocytopenic purpura. ( 24123112 )
2013
7
Health care utilization of patients diagnosed with idiopathic thrombotic thrombocytopenic purpura in a commercially insured population in the United States. ( 22780943 )
2012
8
Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. ( 22409250 )
2012
9
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
10
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis. ( 22547089 )
2012
11
Case report: use of thienopyridines in a patient with acquired idiopathic thrombotic thrombocytopenic purpura. ( 22450533 )
2012
12
Autoimmune disorders in patients with idiopathic thrombotic thrombocytopenic purpura. ( 22960981 )
2012
13
The role of human leukocyte antigens as predisposing and/or protective factors in patients with idiopathic thrombotic thrombocytopenic purpura. ( 22203269 )
2012
14
ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome. ( 21606162 )
2011
15
B cell activating factor is elevated in acute idiopathic thrombotic thrombocytopenic purpura. ( 21615379 )
2011
16
Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab. ( 20576013 )
2010
17
Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab. ( 20422413 )
2010
18
High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. ( 20033409 )
2010
19
Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic link. ( 19922436 )
2010
20
Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura. ( 20378566 )
2010
21
Successful long-term rituximab maintenance for a relapsing patient with idiopathic thrombotic thrombocytopenic purpura. ( 20609201 )
2010
22
HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians. ( 20141578 )
2010
23
A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13. ( 20121555 )
2010
24
Rituximab for managing relapsing or refractory patients with idiopathic thrombotic thrombocytopenic purpura--haemolytic uraemic syndrome. ( 20671883 )
2010
25
Fulminant thrombotic microangiopathy as a clinical presentation of an occult signet-ring cell carcinoma of the lung and misdiagnosed as idiopathic thrombotic thrombocytopenic purpura. ( 20464054 )
2010
26
Rituximab pharmacokinetics during the management of acute idiopathic thrombotic thrombocytopenic purpura. ( 20175870 )
2010
27
Pathogenesis and treatment of acquired idiopathic thrombotic thrombocytopenic purpura. ( 20807984 )
2010
28
Validation of claims-based diagnostic codes for idiopathic thrombotic thrombocytopenic purpura in a commercially-insured population. ( 20352159 )
2010
29
Effect of blood group on idiopathic thrombotic thrombocytopenic purpura. ( 19591220 )
2009
30
Demographic and ADAMTS13 biomarker data as predictors of early recurrences of idiopathic thrombotic thrombocytopenic purpura. ( 19674080 )
2009
31
Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment. ( 19205654 )
2009
32
Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura. ( 18821711 )
2008
33
New-onset and idiopathic thrombotic thrombocytopenic purpura: incidence, diagnostic validity, and potential risk factors. ( 16795056 )
2006
34
Recurrent idiopathic thrombotic thrombocytopenic purpura: a role for vaccination in disease relapse? ( 16931205 )
2006

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

Biological processes related to Thrombotic Thrombocytopenic Purpura, Acquired according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 9.16 ADAMTS13 VWF
2 platelet activation GO:0030168 8.96 ADAMTS13 VWF
3 hemostasis GO:0007599 8.62 ADAMTS13 VWF

Molecular functions related to Thrombotic Thrombocytopenic Purpura, Acquired according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 8.62 ADAMTS13 VWF

Sources for Thrombotic Thrombocytopenic Purpura, Acquired

3 CDC
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