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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#1
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28445600/caplacizumab-reduces-the-frequency-of-major-thromboembolic-events-exacerbations-and-death-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#2
F Peyvandi, M Scully, J A Kremer Hovinga, P Knöbl, S Cataland, K De Beuf, F Callewaert, H De Winter, R K Zeldin
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations and death. In the Phase II TITAN study, treatment with caplacizumab, an anti-vWF Nanobody(®) , was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. OBJECTIVE: The clinical benefit of caplacizumab was further investigated in a post-hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and TTP-related death during the study...
April 26, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#3
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28442312/expert-statements-on-the-standard-of-care-in-critically-ill-adult-patients-with-atypical-haemolytic-uraemic-syndrome
#4
REVIEW
Elie Azoulay, Paul Knoebl, José Garnacho-Montero, Katerina Rusinova, Gennadii Galstian, Philippe Eggimann, Fekri Abroug, Dominique Benoit, Michael von Bergwelt-Baildon, Julia Wendon, Marie Scully
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU...
April 22, 2017: Chest
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#5
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#6
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#7
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28413093/-thrombotic-thrombocytopenia-purpura-in-martinique-retrospective-study-between-2008%C3%A2-and-2015
#8
M Patient, P Fuseau, C Deligny
INTRODUCTION: Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. METHODS: We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28408234/the-acute-hemolytic-anemias-the-importance-of-emergency-diagnosis-and-management
#9
Jennifer J Robertson, Elizabeth Brem, Alex Koyfman
BACKGROUND: Hemolytic anemias are defined by the premature destruction of red blood cells. These anemias have many causes that are mostly due to chronic diseases, but, occasionally, cases of acute life-threatening hemolysis can occur. OBJECTIVE: The objectives of this article were to review the pathophysiology of hemolytic anemias, to discuss the general emergency department (ED) evaluation, and to discuss the assessment and treatment of important and "cannot miss" hemolytic diseases...
April 10, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28405075/acquired-thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-successfully-treated-with-eculizumab
#10
Appalanaidu Sasapu, Michele Cottler-Fox, Pooja Motwani
Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with a mortality of up to 90%, if not promptly recognized and treated. We report a 64-year-old woman with this condition who presented with left-sided weakness and seizure-like activity preceded by headache and easy bruising. She did not achieve optimal response to plasma exchange, corticosteroids, rituximab, and vincristine. We initiated treatment with eculizumab, following which she had durable remission that continued for 30 months after discontinuation of the drug...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28401611/pegylated-bovine-carboxyhaemoglobin-utilisation-in-a-thrombotic-thrombocytopenic-purpura-patient
#11
C Sam, P Desai, D Laber, A Patel, N Visweshwar, M Jaglal
OBJECTIVE: To determine if pegylated bovine carboxyhaemoglobin can be utilised in a thrombotic thrombocytopenic purpura (TTP) patient. BACKGROUND: TTP is a condition characterized by thrombotic microangiopathy and has a high mortality rate when left untreated. Therapeutic plasma exchange is well established as the most effective and evidence-based treatment of TTP. METHODS: The ability to administer plasma exchange therapy is limited in Jehovah's Witnesses who decline blood products due to religious beliefs...
April 12, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28383422/acquired-idiopathic-thrombotic-thrombocytopenic-purpura-successfully-treated-with-intravenous-immunoglobulin-and-glucocorticoid-a-case-report
#12
Hiro Nakao, Akira Ishiguro, Nahoko Ikoma, Kentaro Nishi, Chemin Su, Hisaya Nakadate, Mitsuru Kubota, Masaki Hayakawa, Masanori Matsumoto
RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#13
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28381866/thrombotic-thrombocytopenic-purpura
#14
REVIEW
(no author information available yet)
No abstract text is available yet for this article.
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28381686/refractory-thrombotic-thrombocytopenic-purpura-achieving-complete-remission-with-rituximab-treatment
#15
Hiromasa Iino, Yoshiyuki Ogawa, Kunio Yanagisawa, Hiroaki Shimizu, Takeki Mitsui, Takuma Ishizaki, Masaki Hayakawa, Masanori Matsumoto, Yoshihisa Nojima, Hiroshi Handa
Some patients with thrombotic thrombocytopenic purpura (TTP) are refractory to standard treatment regimens comprised of plasma exchange (PEX) and steroids. This report describes a 40-year-old woman with refractory TTP who achieved complete remission (CR) in response to rituximab. She was referred to our institution from a rural hospital with purpura of the extremities, severe thrombocytopenia, anemia, and rapidly progressive disturbance of consciousness. TTP was diagnosed based on the clinical symptoms of TTP, low ADAMTS13 activity (<0...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28380082/heparin-induced-thrombocytopenia-as-a-cause-of-prolonged-low-platelet-count-in-a-patient-with-thrombotic-thrombocytopenic-purpura-treated-with-plasmapheresis
#16
Agata Winiarska, Norbert Kwella, Tomasz Stompór
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder belonging to thrombotic microangiopathies (TMA) and is caused by functional deficiency of the ADAMTS-13 metalloproteinase. Plasma exchange (PE) remains the treatment of choice in this disease. Here, were describe the case of a patient who apparently recovered from TTP following multiple sessions of PE, but remained thrombocytopenic. Careful analysis revealed the development of heparin-induced thrombocytopenia (HIT) that precluded platelet count (PLT) normalization...
April 5, 2017: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/28369949/evaluating-the-impact-of-the-abo-blood-group-on-the-clinical-outcome-of-thrombotic-thrombocytopenic-purpura-associated-with-severe-adamts13-deficiency
#17
E Hussein, J Teruya
BACKGROUND AND OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is caused by the decrease of ADAMTS13, leading to the accumulation of ultra large von Willebrand factor (ULVWF). It was proposed that the distribution of blood group O among TTP patients may be potentially lower than expected because of the lower levels of VWF. The aim of this study was to explore the relationship between various blood groups and the clinical outcome in TTP. MATERIALS AND METHODS: Thirty-three patients with TTP with severe ADAMTS13 deficiency were studied...
March 31, 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/28367342/remission-of-severe-relapsed-and-refractory-ttp-after-multiple-cycles-of-bortezomib
#18
Manu R Pandey, Pankit Vachhani, Evelena P Ontiveros
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28344807/ipilimumab-induced-thrombotic-thrombocytopenic-purpura-ttp
#19
Jeanelle King, Javier de la Cruz, Jose Lutzky
BACKGROUND: CTLA-4 (Cytotoxic T-lymphocyte-associated protein 4) was the first immune checkpoint receptor clinically targeted for use in cancer treatment. It is expressed exclusively on T-cells where its primary role is to regulate the amplitude of the early stages of T-cell activation.1 Ipilimumab, a CTLA-4 blocking antibody, has been widely used for the treatment of patients with high risk and metastatic melanoma. Given its mechanism of action and consequent immune activation, the side effect profile of this drug greatly differs from that of standard cytotoxic chemotherapy...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28339961/increased-plasma-levels-of-human-neutrophil-peptides-and-complement-activation-markers-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#20
Wenjing Cao, Huy P Pham, Lance A Williams, Rance Siniard, Robin Lorenz, Marisa B Marques, X Long Zhen
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
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