TTP
MCID: THR005
MIFTS: 59

Thrombotic Thrombocytopenic Purpura (TTP)

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

Aliases & Classifications for Thrombotic Thrombocytopenic Purpura

MalaCards integrated aliases for Thrombotic Thrombocytopenic Purpura:

Name: Thrombotic Thrombocytopenic Purpura 12 25 37 29 6 15 63
Purpura, Thrombotic Thrombocytopenic 75 25 44 72
Microangiopathic Hemolytic Anemia 25 72
Congenital Thrombotic Thrombocytopenic Purpura 72
Familial Thrombotic Thrombocytopenia Purpura 25
Familial Thrombotic Thrombocytopenic Purpura 72
Thrombotic Microangiopathy, Familial 25
Purpura Thrombotic Thrombocytopenic 55
Moschcowitz's Syndrome 12
Moschkowitz Disease 25
Ttp 25

Classifications:



External Ids:

Disease Ontology 12 DOID:10772
KEGG 37 H00225
MeSH 44 D011697
NCIt 50 C78797
SNOMED-CT 68 78129009
ICD10 33 M31.1
UMLS 72 C0034155 C0221021 C1268935 more

Summaries for Thrombotic Thrombocytopenic Purpura

Genetics Home Reference : 25 Thrombotic thrombocytopenic purpura is a rare disorder that 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 restrict blood flow to organs such as the brain, kidneys, and heart. Resulting complications can include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems. Blood clots normally form to prevent excess blood loss at the site of an injury. In people with thrombotic thrombocytopenic purpura, clots develop in blood vessels even in the absence of injury. Blood clots are formed from clumps of cell fragments called platelets, which circulate in the blood and assist with clotting. Because a large number of platelets are used to make clots in people with thrombotic thrombocytopenic purpura, fewer platelets are available in the bloodstream. A reduced level of circulating platelets is known as thrombocytopenia. Thrombocytopenia can lead to small areas of bleeding just under the surface of the skin, resulting in purplish spots called purpura. This disorder also causes red blood cells to break down (undergo hemolysis) prematurely. As blood squeezes past clots within blood vessels, red blood cells can break apart. A condition called hemolytic anemia occurs when red blood cells are destroyed faster than the body can replace them. This type of anemia leads to paleness, yellowing of the eyes and skin (jaundice), fatigue, shortness of breath, and a rapid heart rate. There are two major forms of thrombotic thrombocytopenic purpura, an acquired (noninherited) form and a familial form. The acquired form usually appears in late childhood or adulthood. Affected individuals may have a single episode of signs and symptoms, or they may recur over time. The familial form of this disorder is much rarer and typically appears in infancy or early childhood. In people with the familial form, signs and symptoms often recur on a regular basis.

MalaCards based summary : Thrombotic Thrombocytopenic Purpura, also known as purpura, thrombotic thrombocytopenic, is related to thrombotic thrombocytopenic purpura, congenital and thrombocytopenia, and has symptoms including tremor and fever. An important gene associated with Thrombotic Thrombocytopenic Purpura is ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and O-linked glycosylation. The drugs Magnesium Sulfate and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include kidney, brain and heart, and related phenotypes are dyspnea and generalized muscle weakness

KEGG : 37
Thrombotic thrombocytopenic purpura (TTP) is caused by mutation in the ADAMTS13 gene and characterized by microangiopathic hemolytic anemia and thrombocytopenia. The idiopathic forms were found to have acquired autoantibody inhibitors of the ADAMTS13 metalloprotease.

PubMed Health : 63 About thrombotic thrombocytopenic purpura: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. In TTP, blood clots form in small blood vessels throughout the body. The clots can limit or block the flow of oxygen-rich blood to the body's organs, such as the brain, kidneys, and heart. As a result, serious health problems can develop. The increased clotting that occurs in TTP also uses up platelets (PLATE-lets) in the blood. Platelets are blood cell fragments that help form blood clots. These cell fragments stick together to seal small cuts and breaks on blood vessel walls and stop bleeding. With fewer platelets available in the blood, bleeding problems can occur. People who have TTP may bleed inside their bodies, underneath the skin, or from the surface of the skin. When cut or injured, they also may bleed longer than normal. "Thrombotic" (throm-BOT-ik) refers to the blood clots that form. "Thrombocytopenic" (throm-bo-cy-toe-PEE-nick) means the blood has a lower than normal number of platelets. "Purpura" (PURR-purr-ah) refers to purple bruises caused by bleeding under the skin. Bleeding under the skin also can cause tiny red or purple dots on the skin. These pinpoint-sized dots are called petechiae (peh-TEE-kee-ay). Petechiae may look like a rash.

Wikipedia : 75 Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in... more...

Related Diseases for Thrombotic Thrombocytopenic Purpura

Diseases in the Thrombotic Thrombocytopenic Purpura family:

Thrombotic Thrombocytopenic Purpura, Congenital Thrombotic Thrombocytopenic Purpura, Acquired

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

(show top 50) (show all 622)
# Related Disease Score Top Affiliating Genes
1 thrombotic thrombocytopenic purpura, congenital 35.4 CFH ADAMTS13
2 thrombocytopenia 31.8 F3 APOH ADAMTS13
3 purpura 31.6 VWF THBD F3 APOH ADAMTS13
4 disseminated intravascular coagulation 31.4 THBD F3 ADAMTS13
5 hemophilia 31.0 VWF F3
6 platelet aggregation, spontaneous 31.0 VWF SELP
7 spotted fever 31.0 VWF THBD
8 cerebrovascular disease 30.9 VWF SELP SELE
9 coronary thrombosis 30.9 VWF F3
10 complement factor h deficiency 30.8 CFH CD46
11 babesiosis 30.8 THBS1 ADAMTSL1
12 pulmonary hypertension 30.8 VWF THBD F3
13 mixed connective tissue disease 30.8 APOH ADAMTS13
14 hemophilia a 30.7 VWF F3
15 raynaud phenomenon 30.6 VWF THBD APOH
16 intermediate coronary syndrome 30.6 VWF SELP F3
17 korean hemorrhagic fever 30.6 VWF F3
18 polyarteritis nodosa 30.5 SELE APOH
19 acute myocardial infarction 30.5 VWF SELP F3
20 marantic endocarditis 30.5 THBD F3
21 von willebrand's disease 30.4 VWF SELP F3 ADAMTS13
22 vasculitis 30.4 VWF THBD SELE APOH
23 pulmonary embolism 30.3 VWF F3 APOH
24 placental abruption 30.3 THBD APOH
25 retinal vascular occlusion 30.3 SELP APOH
26 hemolytic uremic syndrome, atypical 1 30.2 VWF THBD CFH CD46 ADAMTS13
27 hepatic veno-occlusive disease 30.2 THBD F3
28 portal vein thrombosis 30.1 SELP APOH
29 diabetic angiopathy 30.1 VWF THBD SELE
30 hemorrhagic disease 30.1 VWF THBD F3 ADAMTS13
31 essential thrombocythemia 30.0 VWF THBD SELP F3
32 afibrinogenemia 30.0 VWF F3 CD46
33 branch retinal artery occlusion 30.0 F3 APOH
34 buerger disease 29.9 SELE APOH
35 systemic lupus erythematosus 29.9 VWF THBD SELE F3 APOH ADAMTS13
36 myocardial infarction 29.9 VWF THBD SELP F3 ADAMTS13
37 protein s deficiency 29.8 F3 APOH
38 arteries, anomalies of 29.8 VWF SELP SELE F3
39 membranoproliferative glomerulonephritis 29.7 CFH CD46
40 thrombophilia 29.7 VWF THBD F3 APOH
41 thrombophilia due to thrombin defect 29.6 VWF THBD F3 APOH
42 pre-eclampsia 29.4 VWF THBD SELP SELE F3 APOH
43 thrombosis 29.4 VWF THBD SELP F3 APOH ADAMTS13
44 hypertension, essential 29.4 VWF THBD SELP
45 blood coagulation disease 29.3 VWF THBD SELP F3 ADAMTS13
46 hemolytic-uremic syndrome 29.2 VWF THBD F3 CFH CD46 CABIN1
47 peripheral vascular disease 29.1 VWF THBD SELP SELE F3
48 stroke, ischemic 29.0 VWF THBD SELP F3 APOH ADAMTS13
49 vascular disease 29.0 VWF THBD SELP SELE F3 APOH
50 catastrophic antiphospholipid syndrome 28.8 F3 CFH APOH ADAMTS13

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



Diseases related to Thrombotic Thrombocytopenic Purpura

Symptoms & Phenotypes for Thrombotic Thrombocytopenic Purpura

Human phenotypes related to Thrombotic Thrombocytopenic Purpura:

32 (show all 20)
# Description HPO Frequency HPO Source Accession
1 dyspnea 32 hallmark (90%) HP:0002094
2 generalized muscle weakness 32 hallmark (90%) HP:0003324
3 thrombocytopenia 32 hallmark (90%) HP:0001873
4 reticulocytosis 32 hallmark (90%) HP:0001923
5 microangiopathic hemolytic anemia 32 hallmark (90%) HP:0001937
6 seizures 32 frequent (33%) HP:0001250
7 abdominal pain 32 frequent (33%) HP:0002027
8 confusion 32 frequent (33%) HP:0001289
9 headache 32 frequent (33%) HP:0002315
10 coma 32 frequent (33%) HP:0001259
11 diarrhea 32 frequent (33%) HP:0002014
12 stroke 32 frequent (33%) HP:0001297
13 abnormal lactate dehydrogenase activity 32 frequent (33%) HP:0045040
14 proteinuria 32 occasional (7.5%) HP:0000093
15 fever 32 occasional (7.5%) HP:0001945
16 arrhythmia 32 occasional (7.5%) HP:0011675
17 myocardial infarction 32 occasional (7.5%) HP:0001658
18 hematuria 32 occasional (7.5%) HP:0000790
19 acute kidney injury 32 very rare (1%) HP:0001919
20 decreased serum creatinine 32 very rare (1%) HP:0012101

UMLS symptoms related to Thrombotic Thrombocytopenic Purpura:


tremor, fever

MGI Mouse Phenotypes related to Thrombotic Thrombocytopenic Purpura:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 CD46 CFH F3 SELE SELP THBD
2 hematopoietic system MP:0005397 9.97 ADAMTS13 CABIN1 CFH F3 SELE SELP
3 homeostasis/metabolism MP:0005376 9.91 ADAMTS13 APOH CFH F3 SELE SELP
4 immune system MP:0005387 9.81 ADAMTS13 CABIN1 CFH F3 SELE SELP
5 mortality/aging MP:0010768 9.65 ADAMTS13 APOH CABIN1 CFH F3 SELE
6 vision/eye MP:0005391 9.17 ADAMTS13 CD46 CFH F3 SELE SELP

Drugs & Therapeutics for Thrombotic Thrombocytopenic Purpura

PubMed Health treatment related to Thrombotic Thrombocytopenic Purpura: 63

Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause lasting damage, such as brain damage or a stroke, if it's not treated right away. In most cases, TTP occurs suddenly and lasts for days or weeks, but it can go on for months. Relapses (flareups) can occur in up to 60 percent of people who have acquired TTP. Flareups also occur in most people who have inherited TTP. Plasma treatments are the most common way to treat TTP. Other treatments include medicines and surgery. Treatments are done in a hospital.

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

(show top 50) (show all 112)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 3 7487-88-9 24083
2
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
3
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
4 glucocorticoids Phase 3
5 Anti-Inflammatory Agents Phase 3
6 Cyclosporins Phase 3
7 Dermatologic Agents Phase 3
8 Hormones Phase 3
9 Analgesics Phase 3
10 Tocolytic Agents Phase 3
11 Central Nervous System Depressants Phase 3
12 Anti-Arrhythmia Agents Phase 3
13 Anesthetics Phase 3
14 Peripheral Nervous System Agents Phase 3
15 Anticonvulsants Phase 3
16 Calcium, Dietary Phase 3
17 calcium channel blockers Phase 3
18
Acetaminophen Approved Phase 2 103-90-2 1983
19
Promethazine Approved, Investigational Phase 2 60-87-7 4927
20
Diphenhydramine Approved, Investigational Phase 2 58-73-1, 147-24-0 3100
21
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
22
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
23
Defibrotide Approved, Investigational Phase 2 83712-60-1
24
Danazol Approved Phase 2 17230-88-5 28417
25
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
26
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
27
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492 6473866
28
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616 46835353
29
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
30
Busulfan Approved, Investigational Phase 2 55-98-1 2478
31
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
32
Vidarabine Approved, Investigational Phase 2 24356-66-9 21704 32326
33
Camptothecin Experimental Phase 2 7689-03-4
34
Fibrinolysin Investigational Phase 2 9004-09-5
35 Natriuretic Agents Phase 2
36 Hemostatics Phase 2
37 Coagulants Phase 2
38 arginine Phase 2
39 Deamino Arginine Vasopressin Phase 2
40 Vasopressins Phase 2
41 Arginine Vasopressin Phase 2
42 Angiogenesis Inhibitors Phase 2
43 topoisomerase I inhibitors Phase 2
44 Angiogenesis Modulating Agents Phase 2
45 Topoisomerase Inhibitors Phase 2
46 Fibrinolytic Agents Phase 2
47 Platelet Aggregation Inhibitors Phase 2
48 Anticoagulants Phase 2
49 Complement System Proteins Phase 2
50 Estrogens Phase 2

Interventional clinical trials:

(show top 50) (show all 53)
# Name Status NCT ID Phase Drugs
1 Influence of Plasma Exchange Therapy on the Pharmacokinetics of Rituximab in Patients Treated for Autoimmune Disorders. Completed NCT00820469 Phase 4 Rituximab 375;rituximab 1000
2 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)
3 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
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 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 Participants With Severe Congenital Thrombotic Thrombocytopenic Purpura (cTTP) (Upshaw-Schulman Syndrome) Recruiting NCT03393975 Phase 3
6 Prospective Follow-up Study for Patients Who Completed Study ALX0681-C301 (HERCULES) to Evaluate Long-term Safety and Efficacy of Caplacizumab (Post-HERCULES) Active, not recruiting NCT02878603 Phase 3
7 Interest of Magnesium Sulfate in Thrombotic Thrombocytopenic Purpura in Intensive Care: Multicentric Randomized Controlled Trial Not yet recruiting NCT03237819 Phase 3 Sulfate, Magnesium;Placebo - Concentrate
8 A Blinded Non-inferiority Study to Compare Uniplas With Cryosupernatant Plasma in Thrombotic Thrombocytopenic Purpura (TTP) Terminated NCT00411801 Phase 3
9 STAR - Study of TTP and Rituximab, A Randomized Clinical Trial Terminated NCT00799773 Phase 3 Rituximab;Corticosteroids
10 The Plasma Large-Volume Exchange Randomized Controlled Trial (PLEX-RCT) Withdrawn NCT01433003 Phase 3
11 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
12 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
13 A Single-center Clinical Trial of Bortezomib in Management of Immune Thrombocytopenia (ITP) With High Anti-platelet Antibodies Level Unknown status NCT03013114 Phase 2 Bortezomib
14 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
15 Association of Rituximab to Plasma Exchange in Adult Acquired Idiopathic Thrombotic Thrombocytopenic Purpura Completed NCT00907751 Phase 2 rituximab
16 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
17 An Open-label Safety Study of S-888711 in Adult Subjects With Relapsed Persistent or Chronic Immune Thrombocytopenia With or Without Prior Splenectomy Completed NCT01129024 Phase 2 S-888711 0.5 mg tablet
18 A Phase II Trial of Combination Therapy With Thalidomide and CPT-11 in Patients With Recurrent Anaplastic Gliomas or Glioblastoma Multiforme Completed NCT00412542 Phase 2 Thalidomide;CPT-11
19 A Pilot Trial of Using Pre-Transplant Risk Stratification and Prophylactic Defibrotide to Prevent Serious Thrombotic Microangiopathy in High-Risk Hematopoietic Stem Cell Transplant Patients Recruiting NCT03384693 Phase 2 Defibrotide
20 Adjuvant Low Dose Rituximab for Acquired TTP With Severe ADAMTS13 Deficiency Active, not recruiting NCT01554514 Phase 2
21 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 Not yet recruiting NCT03922308 Phase 2 SHP655
22 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
23 A Phase II, Randomized, Double-blind, Placebo-controlled Trial to Evaluate the Safety and Efficacy of Cemdisiran (ALN-CC5) Following Withdrawal of Chronic Eculizumab Therapy in Patients With Atypical HUS at High Risk of Recurrence Not yet recruiting NCT03999840 Phase 2 cemdisiran;Placebos
24 Phase II Study of Danazol With Plasma Exchange and Steroids for the Treatment of Thrombotic Thrombocytopenic Purpura Terminated NCT00953771 Phase 2 Danazol
25 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 1, Phase 2
26 A Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study to Investigate the Efficacy and Safety of S-888711 Tablets Administered Once-daily for 42 Days to Adult Subjects With Relapsed Persistent or Chronic Immune Thrombocytopenia With or Without Prior Splenectomy Terminated NCT01054443 Phase 2 placebo to S-888711;S-888711;S-888711;S-888711
27 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
28 A Pilot Study Using High Dose Busulfan and Bortezomib as Part of Allogeneic Transplant Conditioning Regimen for High Risk Multiple Myeloma Patients. Terminated NCT01534143 Phase 2 tacrolimus;sirolimus;fludarabine phosphate;busulfan;bortezomib
29 Use of Rituximab Treatment in Addition to Standard Care for Newly Presenting Thrombotic Thrombocytopenic Purpura Withdrawn NCT00251277 Phase 1, Phase 2 Rituximab
30 CHIMERIC MONOCLONAL CD-20 ANTIBODY (RITUXIMAB) FOR STEROID REFRACTORY ACUTE GRAFT VERSUS HOST DISEASE (SR-AGVHD): A PILOT STUDY Withdrawn NCT00578591 Phase 2 Rituximab
31 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
32 Influence of Extracorporeal Circulation During Cardiac Surgery on the Development of Postoperative TTP Unknown status NCT01300117
33 Levels of Von Willebrand Factor Multimers and VWF-Cleaving Protease (ADAMTS-13) in Preterm and Neonate Unknown status NCT00701610
34 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
35 A Pilot Study of N-acetylcysteine in Suspected Thrombotic Thrombocytopenia Purpura Completed NCT01808521 Early Phase 1 N-Acetylcysteine
36 Identification of Early Predictive Factors of Cardiac and Cerebral Involvement in Thrombotic Microangiopathies Completed NCT02134171
37 ADAMTS13-related Prognostic Factors in Adult and Pediatric Thrombotic Thrombocytopenic Purpura Completed NCT00426686
38 Association Between the Quantitative Assessment of Schistocytes in Peripheral Blood Smear and Prognosis of Patient Initially Diagnosed as HELLP Syndrome. Completed NCT03246542
39 Assessment of Immature Platelet Fraction in Pregnancy-Associated Thrombotic Microangiopathy Completed NCT03232359
40 Safety of Tacrolimus And Methotrexate (MTX) Versus Tacrolimus And Mycophenolate Mofetil (MMF) As Graft Versus Host Disease Prophylaxis In Allogeneic Hematopoietic Cell Transplants (HCT) Completed NCT00360685 Tac+MTX;TAC + MMF
41 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 Recruiting NCT01938404 Standard Plasma
42 Thrombotic Thrombocytopenic Purpura Registry - A Prospective Observational Study for Patients Suffering From Hereditary Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome) Recruiting NCT01257269
43 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 Recruiting NCT03605511
44 The United Kingdom Thrombotic Thrombocytopenic Purpura Registry Recruiting NCT03832881
45 Observational, Real-life Study of the Use of octaplasLG®. Recruiting NCT03369314 octaplasLG®
46 A Prospective Study on the Long-Term Vascular Burden in TTP Patients Recruiting NCT03187652
47 Azithromycin in Post Diarrheal Haemolytic and Uremic Syndrome Recruiting NCT02336516 Azithromycin;Placebo (glucose solution 10%)
48 Usefulness of a Diagnostic Algorithm to Diagnose Thrombotic Microangiopathies in Pregnancy Active, not recruiting NCT03580941
49 Serum Androgen Levels as a Marker for the Severity of Preeclampsia Active, not recruiting NCT03744988
50 Outcame of Cases With Hemolytic Uremic Syndrome Attending Assiut University Child Hospital Not yet recruiting NCT03690024

Search NIH Clinical Center for Thrombotic Thrombocytopenic Purpura

Cochrane evidence based reviews: purpura, thrombotic thrombocytopenic

Genetic Tests for Thrombotic Thrombocytopenic Purpura

Genetic tests related to Thrombotic Thrombocytopenic Purpura:

# Genetic test Affiliating Genes
1 Thrombotic Thrombocytopenic Purpura 29

Anatomical Context for Thrombotic Thrombocytopenic Purpura

MalaCards organs/tissues related to Thrombotic Thrombocytopenic Purpura:

41
Kidney, Brain, Heart, Skin, Bone, Endothelial, Bone Marrow

Publications for Thrombotic Thrombocytopenic Purpura

Articles related to Thrombotic Thrombocytopenic Purpura:

(show top 50) (show all 4409)
# Title Authors PMID Year
1
Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays. 9 38
20083997 2010
2
Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor. 9 38
20075158 2010
3
Von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. 9 38
20391298 2010
4
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura. 9 38
19847791 2010
5
Mutation of the H-bond acceptor S119 in the ADAMTS13 metalloprotease domain reduces secretion and substrate turnover in a patient with congenital thrombotic thrombocytopenic purpura. 9 38
19786614 2009
6
Adamalysins in biology and disease. 9 38
19662664 2009
7
Reduced ADAMTS13 activity in delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage. 9 38
19584892 2009
8
Novel evaluation method for densitometric curves of von Willebrand factor multimers and a new parameter (M(MW)) to describe the degree of multimersation. 9 38
19652894 2009
9
Detection of intracellular ADAMTS13, a secreted zinc-metalloprotease, via flow cytometry. 9 38
19526483 2009
10
Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. 9 38
19541819 2009
11
Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. 9 38
19654870 2009
12
The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission. 9 38
19548905 2009
13
VH1-69 germline encoded antibodies directed towards ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura. 9 38
19054323 2009
14
Thrombotic microangiopathies: towards a pathophysiology-based classification. 9 38
19275576 2009
15
The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura. 9 38
19116307 2009
16
ADAMTS13 binds to CD36: a potential mechanism for platelet and endothelial localization of ADAMTS13. 9 38
19389207 2009
17
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura. 9 38
19180123 2009
18
Correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes. 9 38
18957966 2009
19
Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children. 9 38
18574602 2009
20
Effect of blood group on idiopathic thrombotic thrombocytopenic purpura. 9 38
19591220 2009
21
Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report. 9 38
18989146 2008
22
[Genetic polymorphism of von Willebrand factor (VWF)-cleaving protease, ADAMTS13]. 9 38
19069166 2008
23
Von Willebrand factor: another janus-faced hemostasis protein. 9 38
19085767 2008
24
Rapid quantitative assay of ADAMTS13 activity on an automated coagulation analyzer: clinical applications and comparison with immunoblot method. 9 38
18528825 2008
25
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. 9 38
18574040 2008
26
The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction. 9 38
18341632 2008
27
[Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Current perspectives on EHEC, complement mutations and ADAMTS13]. 9 38
18561752 2008
28
Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension. 9 38
18332284 2008
29
Prevalence of the ADAMTS-13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpura. 9 38
18031293 2008
30
ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. 9 38
18223285 2008
31
Linear relationship between ADAMTS13 activity and platelet dynamics even before severe thrombocytopenia. 9 38
18988930 2008
32
Pathogenesis of thrombotic microangiopathies. 9 38
18215115 2008
33
ADAMTS13 related markers and von Willebrand factor in plasma from patients with thrombotic microangiopathy (TMA). 9 38
17900666 2008
34
Thrombotic microangiopathy in a 17-year-old patient: TTP, HUS or a bit of both? 9 38
18184524 2007
35
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. 9 38
17764538 2007
36
Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. 9 38
17910638 2007
37
von Willebrand factor, von Willebrand factor-cleaving protease, and shear stress. 9 38
17979694 2007
38
Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. 9 38
17627784 2007
39
Molecular characterization of four ADAMTS13 mutations responsible for congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome). 9 38
17849048 2007
40
An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers. 9 38
17426255 2007
41
Coagulation parameters of thawed fresh-frozen plasma during storage at different temperatures. 9 38
17561859 2007
42
ADAMTS13 assays and ADAMTS13-deficient mice. 9 38
17414219 2007
43
ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura. 9 38
17187257 2007
44
Targeting the inhibitor of ADAMTS13 in thrombotic thrombocytopenic purpura. 9 38
17309338 2007
45
The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpura. 9 38
17367414 2007
46
Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology. 9 38
17058049 2007
47
Decreased ADAMTS13 activity in plasma from patients with thrombotic thrombocytopenic purpura. 9 38
16797677 2007
48
Comparison and stability of ADAMTS13 activity in therapeutic plasma products. 9 38
17207240 2007
49
Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine and corticosteroids in a patient with systemic lupus erythematosus and antibodies to ADAMTS13. 9 38
17603247 2007
50
The role of von Willebrand factor in hemorrhagic and thrombotic disorders. 9 38
17364690 2007

Variations for Thrombotic Thrombocytopenic Purpura

ClinVar genetic disease variations for Thrombotic Thrombocytopenic Purpura:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 ADAMTS13 NM_139025.4(ADAMTS13): c.3541G> A (p.Gly1181Arg) single nucleotide variant Pathogenic rs192619276 9:136320698-136320698 9:133455576-133455576
2 ADAMTS13 NM_139025.4(ADAMTS13): c.581G> T (p.Gly194Val) single nucleotide variant Pathogenic rs1554785242 9:136291360-136291360 9:133426240-133426240
3 ADAMTS13 NM_139025.4(ADAMTS13): c.2209T> C (p.Cys737Arg) single nucleotide variant Pathogenic rs1554791280 9:136307839-136307839 9:133442718-133442718

Expression for Thrombotic Thrombocytopenic Purpura

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

Pathways for Thrombotic Thrombocytopenic Purpura

Pathways related to Thrombotic Thrombocytopenic Purpura according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.58 VWF THBS1 THBD SELP SELE F3
2
Show member pathways
11.92 THBS1 ADAMTSL1 ADAMTS13
3
Show member pathways
11.88 THBD SELP SELE
4
Show member pathways
11.81 VWF THBD F3
5
Show member pathways
11.74 THBS1 ADAMTSL1 ADAMTS13
6 11.54 THBD SELE F3
7 11.45 VWF THBS1 F3
8
Show member pathways
11.31 THBS1 ADAMTSL1 ADAMTS13
9 11.16 VWF THBD F3 CFH CD46
10 11.02 THBS1 SELP SELE

GO Terms for Thrombotic Thrombocytopenic Purpura

Cellular components related to Thrombotic Thrombocytopenic Purpura according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.87 VWF THBS1 F3 CFH APOH ADAMTSL1
2 extracellular space GO:0005615 9.56 THBS1 THBD SELP SELE F3 CFH
3 collagen-containing extracellular matrix GO:0062023 9.46 VWF THBS1 F3 APOH
4 platelet alpha granule GO:0031091 9.26 VWF THBS1
5 cell surface GO:0009986 9.1 THBS1 THBD F3 CD46 APOH ADAMTS13

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.85 VWF THBS1 SELP SELE
2 response to lipopolysaccharide GO:0032496 9.67 THBD SELP SELE
3 blood coagulation GO:0007596 9.56 VWF THBD F3 ADAMTS13
4 negative regulation of endothelial cell proliferation GO:0001937 9.54 THBS1 APOH
5 response to tumor necrosis factor GO:0034612 9.52 SELE ADAMTS13
6 leukocyte cell-cell adhesion GO:0007159 9.51 SELP SELE
7 negative regulation of endothelial cell migration GO:0010596 9.48 THBS1 APOH
8 leukocyte tethering or rolling GO:0050901 9.46 SELP SELE
9 platelet degranulation GO:0002576 9.46 VWF THBS1 SELP APOH
10 blood coagulation, intrinsic pathway GO:0007597 9.43 VWF APOH
11 negative regulation of blood coagulation GO:0030195 9.4 THBD APOH
12 positive regulation of blood coagulation GO:0030194 9.37 THBS1 APOH
13 positive regulation of transforming growth factor beta production GO:0071636 9.32 THBS1 CD46
14 negative regulation of fibrinolysis GO:0051918 9.13 THBS1 THBD APOH
15 hemostasis GO:0007599 8.92 VWF THBD F3 ADAMTS13

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

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 9.43 VWF THBS1 ADAMTS13
2 sialic acid binding GO:0033691 9.16 SELP SELE
3 oligosaccharide binding GO:0070492 8.96 SELP SELE
4 heparin binding GO:0008201 8.92 THBS1 SELP CFH APOH

Sources for Thrombotic Thrombocytopenic Purpura

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
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57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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