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Thrombotic Thrombocytopenic Purpura

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An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE.

Also Known As:

Purpura, Thrombotic Thrombocytopenic; Congenital Thrombotic Thrombocytopenic Purpura; Familial Thrombotic Thrombocytopenic Purpura; Moschcowitz Disease; Schulman-Upshaw Syndrome; Disease, Moschcowitz; Disease, Moschkowitz; Purpuras, Thrombotic Thrombocytopenic; Purpuras, Thrombotic Thrombopenic; Schulman Upshaw Syndrome; Syndrome, Schulman-Upshaw; Thrombocytopenic Purpura, Thrombotic; Thrombocytopenic Purpuras, Thrombotic; Thrombopenic Purpura, Thrombotic; Thrombopenic Purpuras, Thrombotic; Thrombotic Thrombocytopenic Purpuras; Thrombotic Thrombopenic Purpura; Thrombotic Thrombopenic Purpuras; Moschkowitz Disease; Purpura, Thrombotic Thrombopenic; Thrombotic Thrombocytopenic Purpura, Congenital; Thrombotic Thrombocytopenic Purpura, Familial

Networked: 2396 relevant articles (126 outcomes, 74 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemolytic-Uremic Syndrome
2.	Thrombocytopenia (Thrombopenia)
3.	Thrombotic Microangiopathies
4.	Anemia
5.	Myasthenia Gravis

Experts

1.	George, James N: 56 articles (12/2015 - 01/2002)
2.	Fujimura, Yoshihiro: 43 articles (07/2015 - 05/2002)
3.	Matsumoto, Masanori: 40 articles (10/2015 - 05/2002)
4.	Lämmle, Bernhard: 37 articles (02/2015 - 01/2002)
5.	Vesely, Sara K: 33 articles (05/2015 - 01/2003)
6.	Kremer Hovinga, Johanna A: 33 articles (02/2015 - 08/2003)
7.	Tsai, Han-Mou: 31 articles (10/2014 - 01/2002)
8.	Veyradier, Agnès: 29 articles (12/2015 - 04/2002)
9.	Terrell, Deirdra R: 27 articles (05/2015 - 01/2003)
10.	Coppo, Paul: 21 articles (12/2015 - 05/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Thrombotic Thrombocytopenic Purpura:

1.	thymidine 5'-triphosphate (TTP)IBA 11/09/1991 - "A controlled multicentre trial was conducted in France over a period of five years in 40 patients with thrombotic thrombocytopenic purpura (TTP) to try to determine the best treatment of this disease. " 06/01/2003 - "Thrombotic thrombocytopenic purpura (TTP) is a serious hematologic disorder with a high rate of morbidity and mortality when it fails to go into remission. " 05/01/1990 - "The observation of two clinical cases make possible an evaluation of the potential therapeutic activity of platelet function inhibitors in thrombotic thrombocytopenic purpura (TTP). " 01/01/2006 - "New developments in the understanding of thrombotic thrombocytopenic purpura (TTP) provide opportunities for improved patient care. " 07/01/1993 - "In this study we evaluated the effect of serum collected from seven thrombotic thrombocytopenic purpura (TTP) patients, either in the acute phase of the disease or in clinical remission, on the in vitro growth of bone marrow haematopoietic progenitor cells, obtained from the same TTP patients in clinical remission and from normal donors. "
2.	rituximab (Mabthera)FDA Link 04/01/2008 - "Successful remission of thrombotic thrombocytopenic purpura with rituximab in a patient with undifferentiated connective tissue disorder." 04/01/2007 - "This report suggests that rituximab can provide a good outcome of the dramatic central nervous system involvement in patients with thrombotic thrombocytopenic purpura." 02/01/2009 - "A Canadian phase II study evaluating the efficacy of rituximab in the management of patients with relapsed/refractory thrombotic thrombocytopenic purpura." 04/01/2011 - "Thrombotic thrombocytopenic purpura with severe neurological impairment: remission after Rituximab." 06/01/2005 - "Rituximab provided long-term remission in a patient with refractory relapsing thrombotic thrombocytopenic purpura."
3.	Vincristine (Oncovin)FDA LinkGeneric 08/01/1992 - "[Thrombotic thrombocytopenic purpura achieving complete remission by slow infusion of vincristine]." 11/01/1992 - "[Vincristine played a pivotal role in hematological remission of thrombotic thrombocytopenic purpura (T.T.P.)]." 01/01/2002 - "In this study, vincristine (VCR) was given as salvage treatment in 12 episodes of recurrent thrombotic thrombocytopenic purpura in seven patients, concomitantly with short-term plasma infusion. " 07/01/2011 - "Thrombotic thrombocytopenic purpura treated with vincristine in a Jehovah's witness." 12/01/2007 - "[Successful treatment of thrombotic thrombocytopenic purpura with vincristine report of one case]."
4.	von Willebrand FactorIBA 06/01/2009 - "The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission." 01/01/1992 - "Chronic relapsing thrombotic thrombocytopenic purpura in infants with large von Willebrand factor multimers during remission." 11/04/2010 - "Recent studies demonstrate that Stx also induces von Willebrand factor (VWF) secretion by human endothelial cells and causes thrombotic thrombocytopenic purpura, a disease with similarities to D+HUS, in Adamts13(-/-) mice. " 01/01/2006 - "Recent studies demonstrate that severe deficiency of the von Willebrand factor cleaving metalloprotease, ADAMTS 13, causes thrombotic thrombocytopenic purpura. " 11/30/2005 - "[A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]."
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 10/01/2003 - "Corticosteroids in the treatment of thrombotic thrombocytopenic purpura." 07/01/1997 - "In the conclusion we emphasize the importance of therapeutic replacement of plasma with the infusion of freshly frozen plasma, antithrombocytic therapy and the application of corticosteroids in the treatment of thrombotic thrombocytopenic purpura." 08/01/2007 - "[Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus successfully treated with plasma exchange and corticosteroids]." 01/01/1988 - "Thrombotic thrombocytopenic purpura in an asplenic patient with hereditary spherocytosis: failure of plasmapheresis, antiplatelet therapy, and corticosteroids." 01/01/2011 - "We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. "
6.	SteroidsIBA 01/01/1959 - "Thrombotic thrombocytopenic purpura; two patients with remission associated with the use of large amounts of steroids." 01/01/1996 - "This study is a presentation of a case of a 43 year old woman suffering from thrombotic thrombocytopenic purpura, the clinical course of which was complicated and in which no improvement was observed after fresh frozen plasma transfusions and treatment with steroids. " 01/01/2011 - "A diagnosis of thrombotic thrombocytopenic purpura was made and the child recovered dramatically after giving fresh frozen plasma and steroids." 04/01/1981 - "Thrombotic thrombocytopenic purpura with a subacute course: remission after steroids and high-dose plasma exchange." 01/01/1999 - "Response to plasma exchange and steroids as combined therapy for patients with thrombotic thrombocytopenic purpura."
7.	Platelet Aggregation Inhibitors (Antiplatelet Drugs)IBA 08/01/1981 - "Effects of platelet inhibitors on the platelet aggregation induced by plasma from patients with thrombotic thrombocytopenic purpura." 08/01/1974 - "Thrombotic thrombocytopenic purpura; treatment with antiplatelet agents." 07/01/1973 - "Treatment of thrombotic thrombocytopenic purpura with antiplatelet drugs." 01/01/1982 - "Treatment of thrombotic thrombocytopenic purpura with plasma exchange, antiplatelet agents, corticosteroid, and plasma infusion: Mayo Clinic experience." 02/01/1980 - "Thrombotic thrombocytopenic purpura: combined treatment with plasmapheresis and antiplatelet agents."
8.	Immunoglobulin G (IgG)IBA 08/01/1991 - "Should intravenous immunoglobulin G be first-line treatment for acute thrombotic thrombocytopenic purpura? " 08/01/2011 - "Thrombotic thrombocytopenic purpura in IgG4-related disease with severe deficiency of ADAMTS-13 activity and IgG4 autoantibody against ADAMTS-13." 03/18/2010 - "Epitope mapping of anti-ADAMTS13 immunoglobulin G from patients with thrombotic thrombocytopenic purpura and sequence alignment of the ADAMTS13 spacer domains of human, mouse, and zebrafish with these of human and murine ADAMTS1, a closely related member of ADAMTS family, have provided hints to investigate the role of the amino acid residues between Arg(659) and Glu(664) of the ADAMTS13 spacer domain in substrate recognition. " 05/01/2006 - "ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura." 12/01/2002 - "Intensive plasma IgG removal therapy for severe thrombotic thrombocytopenic purpura."
9.	Immunoglobulins (Immunoglobulin)IBA 01/01/2013 - "Intravenous immunoglobulin and plasmapharesis were subsequently added to the treatment plan to cover thrombotic thrombocytopenic purpura while his autoimmune panel was pending. " 09/01/2010 - "However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a large cohort of patients with thrombotic thrombocytopenic purpura. " 03/01/2000 - "Effects of intravenous immunoglobulin in a patient with intermittent thrombotic thrombocytopenic purpura." 07/01/1995 - "Treatment of thrombotic thrombocytopenic purpura with high-dose immunoglobulins. " 01/01/1994 - "High-dose human immunoglobulins in thrombotic thrombocytopenic purpura."
10.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 05/01/2002 - "They have not been proven more clinically effective than aspirin and have been associated with thrombotic thrombocytopenic purpura. " 04/01/2001 - "They are no more effective than aspirin and have been associated with thrombotic thrombocytopenic purpura. " 05/16/1999 - "Ticlopidine has a slightly greater antiaggregant effect than Aspirin, but has the disadvantage of being more expensive and having serious haematological effects such as thrombotic thrombocytopenic purpura. " 05/01/1982 - "Aspirin dipyridamole, and thrombotic thrombocytopenic purpura." 01/01/1982 - "Ineffectiveness of aspirin and dipyridamole in the treatment of thrombotic thrombocytopenic purpura."

Therapies and Procedures

1.	Plasma Exchange 01/01/2002 - "The clinical course of thrombotic thrombocytopenic purpura has dramatically improved after the introduction of plasma-based therapy, including plasma exchange and plasma infusion. " 04/01/1991 - "This study supports the hypothesis that plasma exchange has significantly improved the prognosis of patients with thrombotic thrombocytopenic purpura. " 06/01/1989 - "Laboratory data then were conclusive for a thrombotic thrombocytopenic purpura (Moschcowitz's disease) and therefore the patient was subjected to plasma exchange during seven days: this measure induced rapid and full clinical and laboratory remission. " 08/08/1991 - "Plasma exchange is more effective than plasma infusion in the treatment of thrombotic thrombocytopenic purpura." 12/01/2014 - "Safety and efficacy of cryoprecipitate-poor plasma as a replacement fluid for therapeutic plasma exchange in thrombotic thrombocytopenic purpura: a single center retrospective evaluation."
2.	Plasmapheresis 09/01/1990 - "Plasmapheresis, a process in which blood is withdrawn, the plasma removed, and the red cells reinfused after resuspension in a replacement solution, has been used for a variety of conditions during pregnancy, but is most useful in the treatment of pregnancy complicated by thrombotic thrombocytopenic purpura or persistent postpartum microangiopathic hemolytic disorders. " 08/01/2001 - "Diagnosis is challenging, but prompt diagnosis is necessary because thrombotic thrombocytopenic purpura is a life-threatening syndrome whose prognosis has been improved significantly by early plasmapheresis treatment." 12/01/1987 - "Efficacy of intensive plasmapheresis in thrombotic thrombocytopenic purpura." 11/01/2013 - "Among the 4032 patients (mean age 47.5 years, 67.7% women, and 36.9% white) with thrombotic thrombocytopenic purpura who also underwent plasmapheresis, in-hospital mortality was 11.1%. " 11/01/2013 - "We used the 2001-2010 Nationwide Inpatient Sample database to identify patients aged ≥18 years with the diagnosis of thrombotic thrombocytopenic purpura (International Classification of Diseases, 9th Revision, Clinical Modification [ICD-9-CM] code 446.6) who also received therapeutic plasmapheresis (ICD-9-CM code 99.71) during the hospitalization. "
3.	Splenectomy 08/15/1996 - "In patients who have had one or more relapses of thrombotic thrombocytopenic purpura, splenectomy done during hematologic remission reduces the frequency of acute relapse and the resulting need for medical therapy." 09/01/1976 - "A case of thrombotic thrombocytopenic purpura in which sustained remission followed splenectomy is described. " 08/15/1996 - "To assess whether splenectomy done during hematologic remission reduces the relapse rate in patients with relapsing thrombotic thrombocytopenic purpura. " 08/15/1996 - "Splenectomy done during hematologic remission to prevent relapse in patients with thrombotic thrombocytopenic purpura." 11/01/1991 - "Because thrombotic thrombocytopenic purpura is nearly always fatal if a remission is not obtained, splenectomy should be considered in patients who prove to be resistant to medical therapy."
4.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 04/01/2001 - "The clinical features, risk factors and outcome of thrombotic thrombocytopenic purpura occurring after bone marrow transplantation." 03/01/2001 - "Thrombotic thrombocytopenic purpura-like syndromes following bone marrow transplantation: an analysis of associated conditions and clinical outcomes." 11/01/2000 - "Impact of thrombotic thrombocytopenic purpura on leukemic children undergoing bone marrow transplantation." 07/01/1994 - "Thrombotic thrombocytopenic purpura: a rare late complication of allogeneic bone marrow transplantation." 04/01/1991 - "Thrombotic thrombocytopenic purpura following allogeneic bone marrow transplantation."
5.	Blood Component Removal (Apheresis) 06/01/2014 - "Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research." 01/01/2012 - "[Use of virus-inactivated plasma in apheresis treatment of thrombotic thrombocytopenic purpura: preliminary data]." 02/01/1999 - "Apheresis and the thrombotic thrombocytopenic purpura syndrome: current advances in diagnosis, pathophysiology, and management." 01/01/1995 - "Influence of type of exchange fluid on survival in therapeutic apheresis for thrombotic thrombocytopenic purpura." 09/10/2003 - "The majority of the increased use of apheresis was outside of the trials: for multiple sclerosis, 30 of 49 patients per year (61% of increase); thrombotic thrombocytopenic purpura, 49 of 56 patients per year (72% of increase); and myeloma cast nephropathy, 60 of 72 patients per year (57% of increase). "