Thrombotic Microangiopathies

Diseases that result in THROMBOSIS in MICROVASCULATURE. The two most prominent diseases are PURPURA, THROMBOTIC THROMBOCYTOPENIC; and HEMOLYTIC-UREMIC SYNDROME. Multiple etiological factors include VASCULAR ENDOTHELIAL CELL damage due to SHIGA TOXIN; FACTOR H deficiency; and aberrant VON WILLEBRAND FACTOR formation.

Also Known As:

Networked: 1277 relevant articles (31 outcomes, 46 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Thrombotic Thrombocytopenic Purpura
3.	Hemolytic-Uremic Syndrome
4.	Nephritis
5.	Pulmonary Hypertension (Ayerza Syndrome)

Experts

1.	Cooper, David K C: 17 articles (11/2015 - 07/2003)
2.	Veyradier, Agnès: 15 articles (11/2014 - 04/2002)
3.	Coppo, Paul: 14 articles (09/2015 - 05/2002)
4.	Matsumoto, Masanori: 13 articles (10/2015 - 12/2003)
5.	Fujimura, Yoshihiro: 13 articles (07/2015 - 12/2003)
6.	Frémeaux-Bacchi, Véronique: 13 articles (02/2015 - 11/2007)
7.	Jodele, Sonata: 12 articles (11/2015 - 08/2011)
8.	Fakhouri, Fadi: 12 articles (02/2015 - 06/2005)
9.	George, James N: 10 articles (12/2015 - 01/2004)
10.	Davies, Stella M: 10 articles (11/2015 - 08/2011)

Drugs and Biologics

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

1.	thymidine 5'-triphosphate (TTP)IBA 06/21/2012 - "We therefore consider this newly established mouse model of thrombotic microangiopathy highly predictive for investigating the efficacy of new treatments for TTP." 07/01/2015 - "Of 38/40 patients who had ADMATS13 testing at study entry, 13/19 refractory and 10/19 relapsing patients had ADAMTS13 < 10% (typical TTP); whereas 6/19 refractory and 9/19 relapsing cases had ADAMTS13 > 10% (other thrombotic microangiopathy; TMA). " 02/01/2015 - "The study involved a prospective cohort of adult TTP patients with acquired severe ADAMTS-13 deficiency (< 10%) and included in the registry of the French Reference Center for Thrombotic Microangiopathies. " 06/14/2012 - "In the present study, we performed a cross-sectional analysis of the national registry of the French Reference Center for Thrombotic Microangiopathies from 2000-2010 to identify all women who were pregnant at their initial TTP presentation. " 09/01/2002 - "Further studies of ADAMTS13 seem likely to change our approach to the diagnosis and treatment of TTP and other thrombotic microangiopathies."
2.	Complement System Proteins (Complement)IBA 06/01/2014 - "Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches." 04/01/2015 - "Recent studies have shown that complement hyperactivation contributes to development of thrombotic microangiopathy. " 07/01/2014 - "In this study, we used homozygosity mapping and whole-exome sequencing to identify a novel truncating mutation in DGKE (p.K101X) in a consanguineous family with patients affected by thrombotic microangiopathy characterized by significant serum complement activation and consumption of the complement fraction C3. " 07/01/2006 - "This study confirms the association between MCP deficiency and aHUS and further establishes that a deficiency in complement regulation, specifically cofactor activity, predisposes to severe thrombotic microangiopathy in the renal vasculature." 10/28/2003 - "This study presents further evidence that complement dysregulation predisposes to development of thrombotic microangiopathy and that screening patients for such defects could provide informed treatment strategies."
3.	von Willebrand FactorIBA 09/15/2001 - "Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases." 10/01/2000 - "A novel interpretation of the role of von Willebrand factor in thrombotic microangiopathies based on platelet adhesion studies at high shear rate flow." 11/01/2014 - "Von Willebrand factor-cleaving protease activity in thrombotic microangiopathy: first report from iran." 07/01/2014 - "Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway." 05/01/2012 - "Ultra-large von Willebrand factor and deficiency of its cleaving protease are important factors in the events leading to thrombotic microangiopathy; however, the mechanisms involved are only partly understood. "
4.	eculizumabFDA Link 12/01/2015 - "Eculizumab for rescue of thrombotic microangiopathy in PM-Scl antibody-positive autoimmune overlap syndrome." 02/01/2015 - "Eculizumab for drug-induced de novo posttransplantation thrombotic microangiopathy: A case report." 06/01/2014 - "Here we present a pediatric case showing that first-line eculizumab treatment successfully blocked the progression of thrombotic microangiopathy in aHUS. " 01/01/2014 - "During long-term eculizumab treatment, thrombotic microangiopathy manifestations were inhibited and renal and cardiac function restored, with no need for other invasive treatments. " 01/01/2014 - "However, a transient pause in treatment resulted in thrombotic microangiopathy relapse, which was rapidly blocked with reintroduction of eculizumab treatment. "
5.	Immunoglobulin G (IgG)IBA 03/01/1994 - "Transplant-associated thrombotic microangiopathy: the role of IgG administration as initial therapy." 07/01/1997 - "This model was induced by selective renal artery perfusion with anti-GEN IgG and resulted in the severe acute renal failure with marked platelet deposition and development of a thrombotic microangiopathy involving glomeruli. " 05/01/2006 - "In this study we used an enzyme-linked immunoassay to analyze the ADAMTS13-binding IgG levels in six groups of individuals: normal, random hospitalized patients, acute TTP, TTP after receiving plasma therapy, TTP in remission, and other types of thrombotic microangiopathy (TMA). " 03/01/1994 - "Both developed clinical and histologic evidence of cyclosporine-associated thrombotic microangiopathy and responded favorably to intravenous IgG therapy. " 07/01/1992 - "Anecdotal reports of recovery with vincristine or IgG alone or with the use of IgG after the apparent failure of plasma therapy appear promising and deserve further investigation as initial therapeutic measures used in thrombotic microangiopathy. "
6.	Atypical hemolytic uremic syndromeIBA 10/01/2015 - "Pregnancy-associated atypical hemolytic uremic syndrome is a systemic disease associated with high morbidity and mortality rates, caused by dysregulation of the alternative complement pathway, leading to uncontrolled complement activation resulting in thrombotic microangiopathy. " 09/01/2015 - "Thrombotic microangiopathy after allogeneic HSCT shares similarities with atypical hemolytic uremic syndrome (aHUS) in the underlying pathomechanisms. " 06/01/2015 - "Atypical hemolytic uremic syndrome is a rare thrombotic microangiopathy caused by chronic defective regulation of the complement activation. " 06/01/2015 - "Thrombotic microangiopathies (TMA) such as atypical hemolytic uremic syndrome (aHUS) have evolved from rare, fulminant childhood afflictions to uncommon diseases with acute and chronic phases involving both children and adults. " 06/01/2015 - "Thrombotic microangiopathy: focus on atypical hemolytic uremic syndrome."
7.	Cyclosporine (Ciclosporin)FDA LinkGeneric 06/01/1993 - "Cyclosporine-associated thrombotic microangiopathy: report of two cases with good outcome and successful subsequent reuse." 09/01/2014 - "Furthermore, there have been reports of thrombotic microangiopathy precipitated by cyclosporine in patients with SSc. " 09/01/2007 - "Many pharmacologic agents, such as cyclosporine and high-intensity conditioning regimens, have been linked with thrombotic microangiopathy. " 05/01/2005 - "Cyclosporine (CsA)-related thrombotic microangiopathy (TMA) was observed in 15 (3.3%) patients. " 12/01/2004 - "We found that the most-frequent cause of thrombotic microangiopathy was drug related, secondary mainly to cyclosporine. "
8.	gemcitabineFDA Link 06/01/2013 - "Renal biopsy showed the classic findings of thrombotic microangiopathy Gemcitabine was discontinued and renal function and hematological parameters gradually improved." 06/01/2014 - "Gemcitabine-related thrombotic microangiopathy: a single-centre retrospective series." 11/01/2013 - "Gemcitabine-induced thrombotic microangiopathy." 07/01/2012 - "Cutaneous thrombotic microangiopathy occurred after the administration of his 17th cycle and a cumulative dose of 53.65 g/m(2) of gemcitabine. " 05/01/2009 - "Thrombotic microangiopathy associated with gemcitabine: rare but real."
9.	imatinib (Gleevec)FDA Link 01/01/2013 - "Imatinib may be effective for ameliorating pulmonary hypertension that is caused by pulmonary tumor thrombotic microangiopathy. " 01/01/2013 - "Pulmonary tumor thrombotic microangiopathy with circulatory failure treated with imatinib." 04/01/2015 - "Imatinib alleviated pulmonary hypertension caused by pulmonary tumor thrombotic microangiopathy in a patient with metastatic breast cancer." 01/01/2015 - "Platelet-derived growth factor receptor-tyrosine kinase inhibitor, imatinib, is effective for treating pulmonary hypertension induced by pulmonary tumor thrombotic microangiopathy."
10.	AntibodiesIBA 12/01/2015 - "She tested positive for PM-Scl antibodies, and a renal biopsy showed an acute thrombotic microangiopathy consistent with scleroderma renal crisis (SRC). " 07/01/2012 - "Laboratory evaluation revealed thrombotic microangiopathy with ADAMTS13 deficiency, with auto-antibodies to ADAMTS13. " 08/20/2009 - "Using different mouse monoclonal and human antiphospholipid (aPL) antibodies, we developed a new animal model of renal injury that shares many features with thrombotic microangiopathy (TMA). " 01/01/2007 - "A variety of mechanisms such as those involving antiglomerular basement membrane antibodies, antineutrophil cytoplasm antibodies or immunocomplexes and thrombotic microangiopathy are implicated in the pathogenesis of this syndrome. " 04/01/2004 - "Renal thrombotic microangiopathy has been described in HCV-positive patients with positive anti-cardolipin antibodies. "

Therapies and Procedures

1.	Plasma Exchange 07/01/2006 - "Plasma exchange (PE) therapy has dramatically improved the outcome of thrombotic microangiopathies (TMA) in adults. " 06/06/2013 - "The primary end points included a change in the platelet count (in trial 1) and thrombotic microangiopathy event-free status (no decrease in the platelet count of >25%, no plasma exchange or infusion, and no initiation of dialysis) (in trial 2). " 12/05/2014 - "Thrombotic microangiopathy and indications for therapeutic plasma exchange." 10/01/2014 - "Plasma exchange therapy may improve thrombotic microangiopathy. " 09/01/2014 - "Plasma exchange (PE) has been reported as successful first-line therapy in other thrombotic microangiopathies. "
2.	Transplants (Transplant) 08/01/2014 - "This study from the Indian subcontinent aims (i) to determine the incidence of transplant glomerulopathy (TG) and thrombotic microangiopathy (TMA) in a large cohort of indicated renal transplant biopsies, (ii) to evaluate the histological and ultrastructural features of TG and TMA, and (iii) to assess the relationship between the two glomerular lesions. " 01/01/2008 - "Further studies with a greater number of patients may be required to highlight the risk factors for post-renal transplant thrombotic microangiopathy." 01/01/2008 - "The aim of this study was to identify cases of post-renal transplant thrombotic microangiopathy in a single transplant center over a period of five years. " 03/01/1997 - "This study is a review of the case records of 18 patients with biopsy proven post transplant thrombotic microangiopathy, without cellular rejection. " 01/01/2016 - "Hematopoietic Stem Cell Transplant-Associated Thrombotic Microangiopathy."
3.	Aftercare (After-Treatment) 01/01/2004 - "Patientes with TAC induced thrombotic microangiopathy usually promptly recover after treatment withdrawal or reduction in the dose of TAC and a short course of plasma therapy, but the risk of rejection increases. " 07/01/2015 - "Although idiopathic pneumonia syndrome and thrombotic microangiopathy improved after treatment, the patient's lymphoma rapidly progressed nonetheless. " 04/13/1985 - "[Pneumo-renal syndrome with thrombotic microangiopathy after treatment with mitomycin]." 03/01/2014 - "We describe the case of a renal transplant patient who developed de novo biopsy-proven thrombotic microangiopathy (TMA) and deep venous thrombosis after treatment with sirolimus (SRL). " 05/01/1985 - "A thrombotic microangiopathy resembling the hemolytic uremic syndrome was diagnosed in 12 patients with adenocarcinoma, in whom the tumor was in complete or near-complete remission after treatment with mitomycin C-containing drug regimens. "
4.	Transplantation (Transplant Recipients) 08/18/2008 - "To study clinicopathological features and mechanism of renal injury in transplantation-associated thrombotic microangiopathy (TA-TMA). " 12/05/2015 - "Additionally, transplantation-associated thrombotic microangiopathy (TA-TMA) may occur and is associated with significant morbidity and mortality. " 01/01/2015 - "Transplantation-associated thrombotic microangiopathy (TA-TMA) is a challenge after allogeneic hematopoietic progenitor cell transplantation, considering the diagnostic uncertainties and lack of established treatment. " 07/01/2014 - "Xenogenic activation of hemostasis (XAH) represents a major hurdle for the transplantation of discordant animal organs into humans as it results in thrombotic microangiopathy (TMA). " 05/01/2014 - "Transplantation-associated thrombotic microangiopathy (TA-TMA) is a feared complication of allogeneic hematopoietic SCT (HSCT) owing to its high mortality rate. "
5.	Homologous Transplantation (Allograft) 04/01/2011 - "Allograft function returned to baseline 3 weeks after starting therapy, and post-treatment allograft biopsies showed improvement in thrombotic microangiopathy. " 02/01/2011 - "This study characterizes the pathologic and clinical relationships of thrombotic microangiopathy (TMA) to antibody-mediated rejection (AMR) in renal allograft biopsies. " 07/01/2014 - "Of the 10 renal allografts, three failed due to thrombotic microangiopathy or rejection. " 06/01/2014 - "Thrombotic microangiopathy in renal allografts: the diagnostic challenge." 01/01/2014 - "Thrombotic microangiopathy in renal allografts."