TTP
MCID: THR044
MIFTS: 27

Thrombotic Thrombocytopenic Purpura, Acquired (TTP)

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 20 70
Idiopathic Thrombotic Thrombocytopenic Purpura 20 54
Purpura, Thrombotic Thrombocytopenic 20 70
Autoimmune Thrombotic Thrombocytopenic Purpura 70
Moschowitz Syndrome 20
Ttp 20

Classifications:



External Ids:

UMLS 70 C0034155 C2584777 C2584778

Summaries for Thrombotic Thrombocytopenic Purpura, Acquired

GARD : 20 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, hereditary 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 Magnesium Sulfate and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and brain.

Related Diseases for Thrombotic Thrombocytopenic Purpura, Acquired

Diseases in the Thrombotic Thrombocytopenic Purpura family:

Thrombotic Thrombocytopenic Purpura, Hereditary 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 158)
# Related Disease Score Top Affiliating Genes
1 thrombotic thrombocytopenic purpura, hereditary 11.8
2 thrombotic thrombocytopenic purpura 11.6
3 immune-mediated thrombotic thrombocytopenic purpura 11.0
4 purpura 11.0
5 thrombocytopenia 10.6
6 hemolytic anemia 10.6
7 hemolytic-uremic syndrome 10.6
8 thrombotic microangiopathy 10.5
9 systemic lupus erythematosus 10.2
10 celiac disease 1 10.1
11 myocardial infarction 10.1
12 signet ring cell adenocarcinoma 10.1
13 thyroiditis 10.1
14 ataxia with vitamin 3 deficiency 10.1
15 lupus erythematosus 10.1
16 ataxia and polyneuropathy, adult-onset 10.0
17 stroke, ischemic 10.0
18 exanthem 10.0
19 rapidly involuting congenital hemangioma 10.0
20 disseminated intravascular coagulation 10.0
21 thrombocytopenia due to platelet alloimmunization 10.0
22 antiphospholipid syndrome 10.0
23 acute kidney failure 10.0
24 pancreatitis 10.0
25 heparin-induced thrombocytopenia 10.0
26 raynaud phenomenon 10.0
27 hepatocellular carcinoma 10.0
28 thrombocytopenic purpura, autoimmune 10.0
29 ocular motor apraxia 10.0
30 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
31 proteinuria, chronic benign 10.0
32 aphasia 10.0
33 covid-19 10.0
34 dermatomyositis 10.0
35 pancytopenia 10.0
36 eclampsia 10.0
37 glomerulonephritis 10.0
38 vasculitis 10.0
39 ataxia with vitamin e deficiency 10.0
40 autoimmune disease 9.9
41 lung cancer 9.9
42 hemolytic uremic syndrome, atypical 1 9.9
43 body mass index quantitative trait locus 1 9.9
44 hemolytic uremic syndrome, atypical 2 9.9
45 inflammatory bowel disease 9.9
46 colitis 9.9
47 thrombosis 9.9
48 mild cognitive impairment 9.9
49 nephrotic syndrome 9.9
50 hellp syndrome 9.9

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 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 3 7487-88-9 24083
2
Clotrimazole Approved, Vet_approved Phase 3 23593-75-1 2812
3
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
4
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
5
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
6
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
7
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
8
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
9
Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
10
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
11
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
12 Anti-Arrhythmia Agents Phase 3
13 Anesthetics Phase 3
14 Anticonvulsants Phase 3
15 Tocolytic Agents Phase 3
16 Calcium, Dietary Phase 3
17 calcium channel blockers Phase 3
18 Analgesics Phase 3
19 Anti-Infective Agents Phase 3
20 Cyclosporins Phase 3
21 Antifungal Agents Phase 3
22 Calcineurin Inhibitors Phase 3
23 Dermatologic Agents Phase 3
24 Antineoplastic Agents, Immunological Phase 2, Phase 3
25 Antirheumatic Agents Phase 2, Phase 3
26 Immunologic Factors Phase 2, Phase 3
27 Protective Agents Phase 2, Phase 3
28 Immunosuppressive Agents Phase 2, Phase 3
29 Hormones Phase 2, Phase 3
30 glucocorticoids Phase 2, Phase 3
31 Hormone Antagonists Phase 2, Phase 3
32 Anti-Inflammatory Agents Phase 2, Phase 3
33 Antineoplastic Agents, Hormonal Phase 2, Phase 3
34 Gastrointestinal Agents Phase 2, Phase 3
35 Antiemetics Phase 2, Phase 3
36 Methylprednisolone Acetate Phase 2, Phase 3
37 Neuroprotective Agents Phase 2, Phase 3
38
Calcium Nutraceutical Phase 3 7440-70-2 271
39
Acetaminophen Approved Phase 2 103-90-2 1983
40
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
41
Promethazine Approved, Investigational Phase 2 60-87-7 4927
42
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
43
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
44
Danazol Approved Phase 2 17230-88-5 28417
45
Fibrinolysin Investigational Phase 2 9004-09-5
46 Deamino Arginine Vasopressin Phase 2
47 Fibrinolytic Agents Phase 2
48 Immunoglobulins Phase 2
49 Antibodies Phase 2
50 Estrogen Receptor Antagonists Phase 2

Interventional clinical trials:

(show all 39)
# 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 Interest of Magnesium Sulfate in Thrombotic Thrombocytopenic Purpura in Intensive Care: Multicentric Randomized Controlled Trial Unknown status NCT03237819 Phase 3 Sulfate, Magnesium;Placebo - Concentrate
3 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
4 A Multi-Center, Randomized Study of Cyclosporine or Corticosteroids as an Adjunct to Plasma Exchange in the Initial Therapy of Thrombotic Thrombocytopenic Purpura (TTP) Completed NCT00713193 Phase 3 Cyclosporine;Prednisone
5 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
6 A Phase 3, Prospective, Randomized, Controlled, Open-label, Multicenter, 2 Period Crossover Study With a Single Arm Continuation Evaluating the Safety And Efficacy of BAX 930 (rADAMTS13) in the Prophylactic And On-demand Treatment of Subjects With Severe Congenital Thrombotic Thrombocytopenic Purpura (cTTP, Upshaw-Schulman Syndrome [USS], Hereditary Thrombotic Thrombocytopenic Purpura [hTTP]) Recruiting NCT03393975 Phase 3
7 An Open-label Multicenter Trial to Study the Efficacy and Safety of Caplacizumab in Japanese Patients With Acquired Thrombotic Thrombocytopenic Purpura Active, not recruiting NCT04074187 Phase 2, Phase 3 Caplacizumab (ALX-0081);Plasma exchange (PE);Corticosteroid treatment (Methylprednisolone or prednisolone);Immunosuppressive treatment (eg, rituximab)
8 A Phase 3b, Prospective, Open-label, Multicenter, Single Treatment Arm, Continuation Study of the Safety and Efficacy of TAK-755 (rADAMTS-13, Also Known as BAX 930/SHP655) in the Prophylactic and On-demand Treatment of Subjects With Severe Congenital Thrombotic Thrombocytopenic Purpura (cTTP; Upshaw-Schulman Syndrome, or Hereditary Thrombotic Thrombocytopenic Purpura) Not yet recruiting NCT04683003 Phase 3
9 STAR - Study of TTP and Rituximab, A Randomized Clinical Trial Terminated NCT00799773 Phase 3 Rituximab;Corticosteroids
10 A Blinded Non-inferiority Study to Compare Uniplas With Cryosupernatant Plasma in Thrombotic Thrombocytopenic Purpura (TTP) Terminated NCT00411801 Phase 3
11 The Plasma Large-Volume Exchange Randomized Controlled Trial (PLEX-RCT) Withdrawn NCT01433003 Phase 3
12 A Phase II Study Evaluating the Efficacy of Rituximab in the Management of Patients With Relapsed/Refractory Thrombotic Thrombocytopenic Purpura (TTP) - Hemolytic Uremic Syndrome (HUS) Unknown status NCT00531089 Phase 2 Rituximab
13 A Study to Assess the Safety, Efficacy and Tolerability of Rituximab (Mabthera) in Combination With Plasma Exchange (PEX) in Patients With Acute Thrombotic Thrombocytopenic Purpura (TTP) Unknown status NCT00937131 Phase 2 Rituximab
14 Association of Rituximab to Plasma Exchange in Adult Acquired Idiopathic Thrombotic Thrombocytopenic Purpura Completed NCT00907751 Phase 2 rituximab
15 A Phase 2 Pilot Study of the Safety, Pharmacokinetics, and Pharmacodynamics of ARC1779 Injection in Patients With Von Willebrand Factor-Related Platelet Function Disorders Completed NCT00632242 Phase 2 ARC1779;ARC1779;ARC1779;ARC1779;ARC1779
16 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
17 Adjuvant Low Dose Rituximab for Acquired TTP With Severe ADAMTS13 Deficiency Completed NCT01554514 Phase 2
18 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
19 Romiplostim in Combination With Low-dose Rituximab and High-dose Dexamethasone as Frontline Treatment for Immune Thrombocytopenia Recruiting NCT04588194 Phase 2 Romiplostim;Rituximab;Dexamethasone
20 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
21 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
22 A Phase II Pilot Study to Evaluate the Safety, Tolerability, Efficacy, Pharmacodynamics and Pharmacokinetics of IdeS in Asymptomatic Antibody-Mediated Thrombotic Thrombocytopenic Purpura (TTP) Patients With Low ADAMTS13 Activity Terminated NCT02854059 Phase 2
23 A Randomized, Double-blind, Placebo Controlled, Clinical Outcome Study of ARC1779 Injection in Patients With Thrombotic Microangiopathy Terminated NCT00726544 Phase 2 ARC 1779 Placebo;ARC1779 Injection;ARC1779 Injection;ARC1779 Injection
24 Phase II Study of Danazol With Plasma Exchange and Steroids for the Treatment of Thrombotic Thrombocytopenic Purpura Terminated NCT00953771 Phase 2 Danazol
25 Use of Rituximab Treatment in Addition to Standard Care for Newly Presenting Thrombotic Thrombocytopenic Purpura Withdrawn NCT00251277 Phase 1, Phase 2 Rituximab
26 BAX930 (rADAMTS13): A Phase 1 Prospective, Uncontrolled, Open-Label, Multicenter, Dose-Escalation Study Evaluating the Safety and Pharmacokinetics in Hereditary Thrombotic Thrombocytopenic Purpura (TTP) Completed NCT02216084 Phase 1 Recombinant ADAMTS13
27 A Observational Study to Determine the Prevalence of Pregnancy-related Thrombotic Thrombocytopenic Purpura and Atypical Haemolytic Uraemic Syndrome in Women Affected by Specific Obstetric Complications Unknown status NCT03605511
28 A Pilot Study of N-acetylcysteine in Suspected Thrombotic Thrombocytopenia Purpura Completed NCT01808521 Early Phase 1 N-Acetylcysteine
29 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] Completed NCT03519672
30 Identification of Early Predictive Factors of Cardiac and Cerebral Involvement in Thrombotic Microangiopathies Completed NCT02134171
31 Observational, Real-life Study of the Use of octaplasLG®. Completed NCT03369314 octaplasLG®
32 ADAMTS13-related Prognostic Factors in Adult and Pediatric Thrombotic Thrombocytopenic Purpura Completed NCT00426686
33 Thrombotic Thrombocytopenic Purpura Registry - A Prospective Observational Study for Patients Suffering From Hereditary Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome) Recruiting NCT01257269
34 The United Kingdom Thrombotic Thrombocytopenic Purpura Registry Recruiting NCT03832881
35 A Multi-Site Tissue Repository Providing Annotated Biospecimens for Approved Investigator-Directed Biomedical Research Initiatives Recruiting NCT01931644
36 A Prospective Study on the Long-Term Vascular Burden in Thrombotic Thrombocytopenic Purpura Patients Recruiting NCT03187652
37 An Observational Study of All Forms of Thrombotic Microangiopathy in Pediatric Patients Terminated NCT00593229
38 Post-Marketing Requirement Study to Evaluate the Safety and Efficacy of Octaplas™ in Patients With Thrombotic Thrombocytopenic Purpura With Special Emphasis on Monitoring the Occurrence of Thromboembolic Events Terminated NCT01938404 Standard Plasma
39 The Role of Microparticles as a Biomarker in Distinguishing Between Thrombotic Thrombocytopenic Purpura (TTP) and Atypical Hemolytic Uremic Syndrome (aHUS) 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:

40
Heart, Lung, Brain, Spinal Cord, Kidney, Thyroid, Placenta

Publications for Thrombotic Thrombocytopenic Purpura, Acquired

Articles related to Thrombotic Thrombocytopenic Purpura, Acquired:

(show all 26)
# Title Authors PMID Year
1
ADAMTS13 and anti-ADAMTS13 autoantibodies in thrombotic thrombocytopenic purpura - current perspectives and new treatment strategies. 20
26581428 2016
2
Update on Thrombocytopenia in Pregnancy. 61
33348401 2020
3
The Association Between Human Leukocyte Antigens and ITP, TTP, and HIT. 61
30543580 2019
4
Multifocal serous detachments as the presenting sign of thrombotic thrombocytopenic purpura. 61
25372532 2014
5
Hepatitis B virus reactivation with rituximab-containing regimen. 61
24303089 2013
6
Thrombotic thrombocytopenic purpura: a hematological emergency. 61
22445678 2012
7
Emergency department management of hematologic and oncologic complications in the patient infected with HIV. 61
20413015 2010
8
A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13. 54
20121555 2010
9
Demographic and ADAMTS13 biomarker data as predictors of early recurrences of idiopathic thrombotic thrombocytopenic purpura. 54
19674080 2009
10
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. 54
19652889 2009
11
ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy. 61
19041237 2009
12
Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura. 54
18821711 2008
13
Translational applications of antibody phage display. 61
18773308 2008
14
Evidence based therapeutic apheresis in autoimmune and other hemolytic anemias. 54
17898570 2007
15
Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. 54
17910638 2007
16
Rituximab in the treatment of adult acquired hemophilia A: a systematic review. 61
17236786 2007
17
The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders. 61
16894452 2006
18
Rituximab can be combined with daily plasma exchange to achieve effective B-cell depletion and clinical improvement in acute autoimmune TTP. 54
16627268 2006
19
Novel, semi-automated, 60-min-assay to determine von Willebrand factor cleaving activity of ADAMTS-13. 54
16430944 2006
20
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement. 54
15232311 2004
21
von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience. 54
14727262 2004
22
Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. 61
12966129 2003
23
Systemic vasculitis in a kidney transplant population. 61
9158021 1997
24
[Plasma- and lymphapheresis in autoimmune diseases]. 61
8686387 1996
25
[In the treatment of hematopoietic disease with glucocorticosteroids]. 61
8164372 1994
26
Polycythemia, idiopathic thrombocytopenic purpura, and thrombotic thrombocytopenic purpura. 61
1564600 1992

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

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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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