Thrombotic Microangiopathies
Purpura, Thrombotic Thrombocytopenic
Hemolytic-Uremic Syndrome
HELLP Syndrome
Cancer awareness in atypical thrombotic microangiopathies. (1/86)
(+info)Pathogenesis of thrombotic microangiopathy: insights from animal models. (2/86)
(+info)Lupus nephritis combined with renal injury due to thrombotic thrombocytopaenic purpura-haemolytic uraemic syndrome. (3/86)
(+info)A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection. (4/86)
(+info)Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome. (5/86)
(+info)A phase II pilot study of tacrolimus/sirolimus GVHD prophylaxis for sibling donor hematopoietic stem cell transplantation using 3 conditioning regimens. (6/86)
(+info)Inhibition of tyrosine kinases by sunitinib associated with focal segmental glomerulosclerosis lesion in addition to thrombotic microangiopathy. (7/86)
(+info)A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13. (8/86)
(+info)Thrombotic microangiopathies (TMAs) are a group of disorders characterized by the formation of blood clots in small blood vessels, causing damage to the end organs. This process leads to a constellation of clinical symptoms including thrombocytopenia (low platelet count), microangiopathic hemolytic anemia (breakdown of red blood cells leading to anemia), and organ dysfunction such as renal failure, neurological impairment, or cardiac involvement.
TMAs can be primary or secondary. Primary TMAs are caused by genetic mutations affecting the complement system, coagulation cascade, or other regulatory proteins involved in vascular homeostasis. Examples of primary TMAs include atypical hemolytic uremic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP), and complement-mediated TMA.
Secondary TMAs are caused by various underlying conditions or exposures, such as infections, autoimmune diseases, malignancies, drugs, pregnancy-related complications, or other systemic disorders. The pathogenesis of secondary TMAs is often multifactorial and may involve endothelial injury, complement activation, and platelet aggregation.
The diagnosis of TMAs requires a combination of clinical, laboratory, and sometimes histopathological findings. Treatment depends on the underlying cause and may include supportive care, plasma exchange, immunosuppressive therapy, or targeted therapies such as complement inhibitors.
Thrombotic thrombocytopenic purpura (TTP) is a rare but serious blood disorder. It's characterized by the formation of small blood clots throughout the body, which can lead to serious complications such as low platelet count (thrombocytopenia), hemolytic anemia, neurological symptoms, and kidney damage.
The term "purpura" refers to the purple-colored spots on the skin that result from bleeding under the skin. In TTP, these spots are caused by the rupture of red blood cells that have been damaged by the abnormal clotting process.
TTP is often caused by a deficiency or inhibitor of ADAMTS13, a protein in the blood that helps to regulate the formation of blood clots. This deficiency or inhibitor can lead to the formation of large clots called microthrombi, which can block small blood vessels throughout the body and cause tissue damage.
TTP is a medical emergency that requires prompt treatment with plasma exchange therapy, which involves removing and replacing the patient's plasma to restore normal levels of ADAMTS13 and prevent further clotting. Other treatments may include corticosteroids, immunosuppressive drugs, and rituximab.
Hemolytic-Uremic Syndrome (HUS) is a serious condition that affects the blood and kidneys. It is characterized by three major features: the breakdown of red blood cells (hemolysis), the abnormal clotting of small blood vessels (microthrombosis), and acute kidney failure.
The breakdown of red blood cells leads to the release of hemoglobin into the bloodstream, which can cause anemia. The microthrombi can obstruct the flow of blood in the kidneys' filtering system (glomeruli), leading to damaged kidney function and potentially acute kidney failure.
HUS is often caused by a bacterial infection, most commonly Escherichia coli (E. coli) that produces Shiga toxins. This form of HUS is known as STEC-HUS or Stx-HUS. Other causes include infections with other bacteria, viruses, medications, pregnancy complications, and certain medical conditions such as autoimmune diseases.
Symptoms of HUS may include fever, fatigue, decreased urine output, blood in the stool, swelling in the face, hands, or feet, and irritability or confusion. Treatment typically involves supportive care, including dialysis for kidney failure, transfusions to replace lost red blood cells, and managing high blood pressure. In severe cases, a kidney transplant may be necessary.
HELLP syndrome is a serious complication in pregnancy, characterized by Hemolysis (the breakdown of red blood cells), Elevated Liver enzymes, and Low Platelet count. It is often considered a variant of severe preeclampsia or eclampsia, although it can also occur without these conditions.
The symptoms of HELLP syndrome include headache, nausea and vomiting, upper right abdominal pain, and visual disturbances. It can lead to serious complications for both the mother and the baby, such as liver failure, placental abruption, disseminated intravascular coagulation (DIC), and even death if not promptly diagnosed and treated.
The exact cause of HELLP syndrome is not known, but it is thought to be related to problems with the blood vessels that supply the placenta. Treatment typically involves delivering the baby as soon as possible, even if the baby is premature. Women who have had HELLP syndrome are at increased risk for complications in future pregnancies.
ADAM (A Disintegrin And Metalloprotease) proteins are a family of type I transmembrane proteins that contain several distinct domains, including a prodomain, a metalloprotease domain, a disintegrin-like domain, a cysteine-rich domain, a transmembrane domain, and a cytoplasmic tail. These proteins are involved in various biological processes such as cell adhesion, migration, proteolysis, and signal transduction.
ADAM proteins have been found to play important roles in many physiological and pathological conditions, including fertilization, neurodevelopment, inflammation, and cancer metastasis. For example, ADAM12 is involved in the fusion of myoblasts during muscle development, while ADAM17 (also known as TACE) plays a crucial role in the shedding of membrane-bound proteins such as tumor necrosis factor-alpha and epidermal growth factor receptor ligands.
Abnormalities in ADAM protein function have been implicated in various diseases, including cancer, Alzheimer's disease, and arthritis. Therefore, understanding the structure and function of these proteins has important implications for the development of novel therapeutic strategies.
Thrombotic thrombocytopenic purpura
Thrombotic microangiopathy
Atypical hemolytic uremic syndrome
Hemolytic-uremic syndrome
Thrombocytopenia
Membranoproliferative glomerulonephritis
HELLP syndrome
Transplant glomerulopathy
Red blood cell
Microangiopathy
Schistocyte
Onconephrology
Cholesterol embolism
Hemoglobinuria
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Thrombocytopenic15
- This combination of thrombocytopenia and MAHA, in which thrombi form in the microvasculature and schistocytes develop from red cell destruction as they pass over these thrombi [ 2 ], occurs in patients with disseminated intravascular coagulation (DIC), but also in those with thrombotic microangiopathies (TMAs), including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). (biomedcentral.com)
- An algorithm to rapidly differentiate disseminated intravascular coagulation (DIC) from thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) in the intensive care unit (ICU). (biomedcentral.com)
- Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. (wikipedia.org)
- and for plasma exchange in patients with thrombotic thrombocytopenic purpura ( TTP ). (rxlist.com)
- TMA is well known to occur in the setting of thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). (hindawi.com)
- How do you actually diagnose TTP [thrombotic thrombocytopenic purpura] and how do you differentiate TTP from many other thromboses or microangiopathies which have a similar clinical presentation? (ajmc.com)
- Neurological symptoms related to microthrombosis are the hallmark of acute manifestations of acquired thrombotic thrombocytopenic purpura (TTP). (haematologica.org)
- Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microangiopathy with fluctuating signs and symptoms. (haematologica.org)
- Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. (biomedcentral.com)
- Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. (biomedcentral.com)
- Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency. (biomedcentral.com)
- Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) fall into the broader category of thrombotic microangiopathies (TMA). (medscape.com)
- Though recent research has revealed that the two disease processes have underlying similarities, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura have historically been considered two separate disease entities. (medscape.com)
- Thrombotic thrombocytopenic purpura(TTP) is a multisystem disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia associated with red cell fragmentation, and neurological and renal symptoms. (elsevierpure.com)
- Introduction: Thrombotic microangiopathy (TM) of the fulminant type occurring in patients following bone marrow transplant (BMT) has clinical manifestations that are similar to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome, but the outcome is generally fatal despite conventional therapy. (johnshopkins.edu)
Microangiopathy8
- The role of complement dysregulation on vascular endothelial cells has been well established in atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and target organ injury. (jrheum.org)
- Thrombotic microangiopathy (TMA) is a well-known clinicopathologic entity characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury. (jrheum.org)
- Common anticancer chemotherapeutic agents causing drug-induced thrombotic microangiopathy via immune-mediated mechanism or dose-dependent toxicity or both [ 4 , 7 ]. (hindawi.com)
- 8 ] [ 9 ] Other organs may also be affected by this form of thrombotic microangiopathy, resulting in multiple organ dysfunction syndrome and eventually death. (thieme-connect.com)
- Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a rare condition associated with neoplastic disorders, predominantly gastric cancer, leading to pre-capillary Pulmonary Hypertension (PH). (unimib.it)
- We examined the activity of the vWF-cleaving protease(vWF-CP), by modified Furlan's method, in plasma from patients with a familial TTP, 3 acquired TTP, 4 thrombotic microangiopathy(TMA) and 2 veno-occlusive disease(VOD) associated after allo-BMT. (elsevierpure.com)
- Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management. (korea.ac.kr)
- In Scotland, most patients with pediatric thrombotic microangiopathy are referred to a specialist pediatric hospital, which immediately reports cases of HUS to Health Protection Scotland as part of national surveillance. (cdc.gov)
Hemolytic2
- Thrombotic microangiopathies are a group of disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia, and ischemic end organ damage mostly involving the kidneys and brain caused by disseminated occlusive microvascular thrombosis [ 1 ]. (hindawi.com)
- Thrombotic microangiopathies are characterized by the involvement of widespread occlusive microvascular thromboses resulting in thrombocytopenia, microangiopathic hemolytic anemia, and variable signs and symptoms of end-organ ischemia. (medscape.com)
TMAs3
- Abnormal coagulation studies, including prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen concentration, fibrin degradation products, D-dimers, and antithrombin, are required for differentiation of DIC from thrombotic microangiopathies (TMAs). (biomedcentral.com)
- Drug-induced thrombotic microangiopathies (DTMAs) are increasingly being recognized as an important category of thrombotic microangiopathies (TMAs). (hindawi.com)
- Introduction Infection with Human Immunodeficiency virus (HIV) is a risk factor for endothelial disease which can manifest as venous thromboembolic disease (VTE), arterial thrombosis and atherosclerosis and microvascular disease specifically thrombotic microangiopathies (TMAs) including disseminated intravascular coagulation (DIC) and Thrombotic Thrombocytopaenic Purpura (TTP). (wits.ac.za)
Complications1
- 4 ] [ 5 ] Approximately 27% of critically ill intensive care unit (ICU)-admitted COVID-19 patients develop venous thromboembolism during admission,[ 6 ] while 4.4% experience arterial thrombotic complications. (thieme-connect.com)
Webinar1
- Register for the ISN Frontiers Webinar on Thrombotic Microangiopathies - September 17 @ 2 p.m. (theisn.org)
Glomerulonephritis2
- Complement activation is known to occur in many diverse renal diseases, including glomerulonephritis, thrombotic microangiopathies and transplant rejection. (wjgnet.com)
- Among them are the thrombotic microangiopathies, many forms of glomerulonephritis, and acute inflammatory episodes like sepsis and COVID-19 illness. (qxmd.com)
Acute2
- To assess the long-term neuropsychological consequences of acute TTP, we recruited 35 acquired TTP patients (77% females, median age at onset 41 years, interquartile range: 35-48) regularly followed at our out-patient clinic of thrombotic microangiopathies in Milan (Italy) from December 2015 to October 2016. (haematologica.org)
- Prognostic Indicators for Recurrent Thrombotic Events in HIV-infected Patients with Acute Coronary Syndromes: Use of Registry Data From 12 sites in Europe, South Africa and the United States. (wustl.edu)
Arterial1
- Methods Data and samples were collected from 5 different sources - HIV-infected patients without documented arterial disease, HIV-infected patients with documented arterial disease, HIV-infected patients and HIV-uninfected patients with documented thrombotic microangiopathic disease and healthy HIV-uninfected controls. (wits.ac.za)
Patients1
- Clinically, these patients present with increased risk of thrombotic microangiopathies. (wits.ac.za)
Diseases1
- That's the key to differentiate TTP from many other thrombotic diseases or microangiopathies. (ajmc.com)
Common1
- Thrombotic Microangiopathies: Thrombus Formation Due to Common or Related Mechanisms? (abdominalkey.com)
Hemolytic uremic s2
- Atypical hemolytic-uremic syndrome is a genetic, life-threatening, chronic disease of complement-mediated thrombotic microangiopathy. (nih.gov)
- Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microthrombi, leading to ischemic tissue injury in the renal vasculature. (amjcaserep.com)
Microangiopathy8
- CC Grand Rounds: (1) Renal Complications after Hematopoietic Stem Cell Transplantation: Transplant-Associated Thrombotic Microangiopathy (2) Current Approaches to Assessing Kidney Function: Who, How, and Why? (nih.gov)
- Renal complications after hematopoietic stem cell transplantation : transplant-associated thrombotic microangiopathy / Meryl Waldman. (nih.gov)
- 6. Management of cancer-associated thrombotic microangiopathy: what is the right approach? (nih.gov)
- 10. [Breast cancer-related thrombotic microangiopathy: A review]. (nih.gov)
- A recent narrative review published in AJKD discusses the association between thrombotic microangiopathy (TMA) and hematopoietic stem cell transplantation (HSCT). (ajkdblog.org)
- We focused on EV-mediated interaction between the immune system and the kidney in autoimmune diseases displaying common renal damage, such as antiphospholipid syndrome, systemic lupus erythematosus, thrombotic microangiopathy, and vasculitis. (unicyte.ch)
- Thrombotic microangiopathy (TMA) is a rare but potentially lethal complication encountered in solid organ and bone marrow transplant recipients that requires rapid recognition, diagnosis, and initiation of therapy. (elsevierpure.com)
- The patient had acute kidney injury with hematuria and proteinuria, and kidney biopsy showed hypertensive arteriolar nephrosclerosis and fibrinoid arteriolar necrosis consistent with thrombotic microangiopathy. (amjcaserep.com)
Thrombocytopenia3
- Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. (hacettepe.edu.tr)
- NOTTO Guidelines for Vaccine Induced Thrombotic Thrombocytopenia in Organ Donation and Transplantation. (ucsd.edu)
- Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. (unifesp.br)
Purpura1
- The prevalence, incidence and outcomes of haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopaenic purpura (TTP) are not well established in adults or children from prospective studies. (nih.gov)
Glomerular1
- My research interests include glomerular disorders, disorders of potassium metabolism, military medicine, and thrombotic microangiopathies. (nih.gov)
Experience1
- A multicenter experience of thrombotic m. (hacettepe.edu.tr)