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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/28815344/long-term-survival-after-hematopoietic-stem-cell-transplantation-for-complete-stat1-deficiency
#1
Samuele Naviglio, Elena Soncini, Donatella Vairo, Arnalda Lanfranchi, Raffaele Badolato, Fulvio Porta
PURPOSE: Complete signal transducer and activator of transcription 1 (STAT1) deficiency is a rare autosomal recessive condition characterized by impairment of intracellular signaling from both type I and type II interferons (IFN). Affected patients are prone to early severe mycobacterial and viral infections, which usually result in death before 18 months of age. We previously reported a patient affected by complete STAT1 deficiency who underwent hematopoietic stem cell transplantation (HSCT)...
August 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28796639/development-and-validation-of-a-multivariable-prediction-rule-for-detecting-a-severe-acquired-adamts13-activity-deficiency-in-patients-with-thrombotic-microangiopathies
#2
Jorge M Nieto, Félix De La Fuente-Gonzalo, Fernando A González, Ana Villegas, Rafael Martínez, Manuel E Fuentes, Paloma Ropero
BACKGROUND: Thrombotic microangiopathies (TMAs) are a group of diseases that have different aetiologies and treatments, but a clinical differential diagnosis remains difficult. Among TMAs, thrombotic thrombocytopenic purpura (TTP) is characterised by a severe ADAMTS13 functional deficiency. However, assays exploring ADAMTS13 activity are limited to some specialised laboratories. Our objective was to develop and validate a diagnostic method for TTP in adult patients with TMA. METHODS: We generated a multivariable model (four predictors) on a cohort of 174 TMA patients in order to predict an ADAMTS13 activity deficiency (AUC of 0...
August 10, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28792176/chikungunya-fever-presenting-as-life-threatening-thrombotic-thrombocytopenic-purpura
#3
Vimal Kumar, Rujul Jain, Arvind Kumar, Neeraj Nischal, Pankaj Jorwal, Manish Soneja, Sudheer Arava, Naveet Wig
It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura...
July 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#4
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28780041/quinine-induced-thrombotic-microangiopathy-a-report-of%C3%A2-19%C3%A2-patients
#5
Evaren E Page, Dustin J Little, Sara K Vesely, James N George
BACKGROUND: Quinine can cause diverse and severe immune-mediated adverse reactions, including thrombotic microangiopathy (TMA). Our objective was to describe the presenting features and long-term outcomes of patients with quinine-induced TMA. STUDY DESIGN: A case series of 19 patients with quinine-induced TMA treated with plasma exchange. SETTING & PARTICIPANTS: Patients with quinine-induced TMA initially suspected of having thrombotic thrombocytopenic purpura (TTP) were identified among patients enrolled in the Oklahoma TTP-Hemolytic Uremic Syndrome Registry...
August 3, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28774273/thrombotic-microangiopathy-associated-with-valproic-acid-toxicity
#6
Sean A Hebert, Timothy P Bohan, Christian L Erikson, Rita D Swinford
BACKGROUND: Thrombotic microangiopathy (TMA) is a serious, sometimes life-threatening disorder marked by the presence of endothelial injury and microvascular thrombi. Drug-induced thrombotic microangiopathy (DI-TMA) is one specific TMA syndrome that occurs following drug exposure via drug-dependent antibodies or direct tissue toxicity. Common examples include calcineurin inhibitors Tacrolimus and Cyclosporine and antineoplastics Gemcitabine and Mitomycin. Valproic acid has not been implicated in DI-TMA...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28768626/pathophysiology-of-thrombotic-thrombocytopenic-purpura
#7
J Evan Sadler
The discovery of ADAMTS13 revolutionized our approach to thrombotic thrombocytopenic purpura (TTP). Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor (VWF) and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes and tissue injury that characterize TTP. Most patients with acquired TTP respond to a combination of plasma exchange and rituximab, but some die or acquire irreversible neurological deficits before they can respond, and relapses can occur unpredictably...
August 2, 2017: Blood
https://www.readbyqxmd.com/read/28764998/validation-of-the-plasmic-score-at-a-university-medical-center
#8
Ryan Jajosky, Mark Floyd, Thomas Thompson, James Shikle
BACKGROUND: The PLASMIC score was recently described as a convenient tool for predicting ADAMTS13 activity ≤10% in patients with possible thrombotic thrombocytopenic purpura (TTP), while awaiting the results of this send-out test. The purpose of this study was to validate the PLASMIC score at our University Medical Center. METHODS: Apheresis records were reviewed from 2008 to 2017 to identify patients who received plasma exchange (PLEX) for suspected TTP. The ADAMTS13 activity and PLASMIC scoring criteria were recorded, and the PLASMIC score was calculated...
July 8, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28759473/novel-therapeutic-approaches-for-thrombotic-thrombocytopenic-purpura
#9
Yvette C Tanhehco, Gowthami Arepally, Ara Metjian
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with therapeutic plasma exchange and immunosuppression with steroids results in high remission and low mortality rates. However, a number of patients remain refractory to frontline therapy and/or experience multiple relapses. This study reviews emerging therapies for thrombotic thrombocytopenic purpura...
July 28, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28758037/an-atypical-presentation-of-chronic-atrophic-gastritis-hemolytic-anemia-and-mesenteric-panniculitis
#10
Zurab Azmaiparashvili, Vinicius M Jorge, Catiele Antunes
Microangiopathic hemolytic anemia (MAHA) requires an aggressive approach since primary thrombotic microangiopathy syndromes such as thrombotic thrombocytopenic purpura (TTP) can progress rapidly to a fatal outcome. Differential diagnosis can be challenging even for an experienced hematologist. We present a case of a 52-year-old male who presented with symptoms of mesenteric panniculitis and showed signs of MAHA. His condition was attributed to severe vitamin B12 deficiency secondary to chronic atrophic gastritis and initiation of appropriate therapy was met with complete resolution of symptoms and normalization of hematologic parameters...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28751567/the-adamts13-1239-1253-peptide-is-a-dominant-hla-dr1-restricted-cd4-t-cell-epitope
#11
Laurent Gilardin, Sandrine Delignat, Ivan Peyron, Mathieu Ing, Yu-Chun Lone, Bagirath Gangadharan, Baptiste Michard, Yousra Kherabi, Meenu Sharma, Anastas Pashov, Jean-Baptiste Latouche, Mohamad Hamieh, Olivier Toutirais, Pascale Loiseau, Lionel Galicier, Agnès Veyradier, Srini Kaveri, Bernard Maillère, Paul Coppo, Sébastien Lacroix-Desmazes
Acquired thrombotic thrombocytopenic purpura is a rare and severe disease characterized by auto-antibodies directed against "A Disintegrin And Metalloproteinase with Thrombospondin type 1 repeats, 13th member" (ADAMTS13), a plasma protein involved in hemostasis. The implication of CD4+ T cells in the pathogenesis of the disease is suggested by the IgG isotype of the antibodies. However, the nature of the CD4+ T-cell epitopes remains poorly characterized. Here, we determined the HLA-DR-restricted CD4+ T-cell epitopes of ADAMTS13...
July 27, 2017: Haematologica
https://www.readbyqxmd.com/read/28748411/the-role-of-von-willebrand-factor-in-thrombotic-microangiopathy
#12
REVIEW
Damien G Noone, Magdalena Riedl, Christoph Licht
Thrombotic microangiopathy (TMA) is caused by thrombus formation in the microvasculature. The disease spectrum of TMA includes, amongst others, thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS). TTP is caused by defective cleavage of von Willebrand factor (VWF), whereas aHUS is caused by overshooting complement activation and subsequent endothelial cell (EC) injury. Despite their distinct pathophysiology, the clinical manifestation of TTP and aHUS consisting of microangiopathic haemolytic anaemia and thrombocytopenia is often similar and difficult to distinguish...
July 26, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28717280/priapism-with-penile-gangrene-an-unusual-presentation-of-multiple-myeloma
#13
Vikas Kumar Panwar, Ravimohan S Mavuduru, Sudheer Kumar Devana, Kim Vaiphei, Girdhar Singh Bora
Penile gangrene is very rare sequel of ischemic priapism. Previous published reports have shown its occurrence in patients with sickle cell disease, urethral carcinoma, bladder carcinoma, thrombotic thrombocytopenic purpura, idiopathic, traumatic, etc. Ischemic priapism with penile gangrene as an initial presentation of multiple myeloma has not been reported. We present a 44-year-old patient of multiple myeloma presenting with ischemic priapism and penile gangrene requiring partial penectomy.
July 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28711333/b12-deficiency-leading-to-marked-poikilocytosis-versus-true-schistocytosis-a-pernicious-problem
#14
James A Hall, James Mason, Julia Choi, Mark Holguin
Severe vitamin B12 deficiency is caused most commonly by autoimmune atrophic gastritis leading to loss of intrinsic factor. Vitamin B12 deficiency leading to megaloblastic anemia and demyelinating central nervous system disease is well known; however, a rare presentation of B12 deficiency described as pseudothrombotic microangiopathy is not well known. This complication presents with signs of mechanical hemolysis, elevated lactate dehydrogenase (LDH), thrombocytopenia, and a low reticulocyte count, which can be incorrectly diagnosed as thrombotic thrombocytopenic purpura and managed incorrectly...
June 20, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28711159/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-epidemiology-pathophysiology-and-clinics
#15
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Thrombotic microangiopathies (TMA) represent an eclectic group of conditions, which share hemolytic anemia and thrombocytopenia as a common defining basis. Remarkable breakthroughs in the physiopathological setting have allowed for a thorough recomposition of the disparate syndromes, which form the constellation of TMA. In this view, clinicians now discriminate thrombocytopenic thrombotic purpura (TTP) defined by a severe deficiency in ADAMTS13, which is rarely associated with a severe renal involvement and the hemolytic and uremic syndrome (HUS) in which renal impairment is the most prominent clinical feature...
July 12, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28711077/acute-kidney-injury-in-pregnancy
#16
Belinda Jim, Vesna D Garovic
Pregnancy-related acute kidney injury (AKI) has declined in incidence in the last three decades, although it remains an important cause of maternal and fetal morbidity and mortality. Pregnancy-related causes of AKI such as preeclampsia, acute fatty liver of pregnancy, HELLP (Hemolysis, Elevated Liver function tests, Low Platelets) syndrome, and the thrombotic microangiopathies (thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome [HUS]) exhibit overlapping features and often present as diagnostic dilemmas...
July 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28707764/development-of-systemic-lupus-erythematosus-5-years-after-onset-of-thrombotic-thrombocytopenic-purpura
#17
Keita Shingu, Yasutomo Funakoshi, Nobuhiro Ito, Masahiko Okada, Hiroyuki Moriuchi
No abstract text is available yet for this article.
July 14, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28702343/eb-virus-reactivation-triggers-thrombotic-thrombocytopenic-purpura-in-a-healthy-adult
#18
Satoko Oka, Masaharu Nohgawa
Thrombotic thrombocytopenic purpura (TTP) is rare but life-threatening disease, characterized typically by microangiopathic hemolytic anemia (MAHA), profound peripheral thrombocytopenia and severe deficiency in the von Willebrand factor-cleaving prortease ADAMTS13. It has been reported that acquired immune TTP is closely associated with human immunodeficiency virus infection and influenza infection or vaccination. However, it has not been reported to be associated with Epstein Barr Virus infection or reactivation...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/28697902/acquired-thrombotic-thrombocytopenic-purpura-and-pregnancy-more-light-than-shade-but-controversies-remain
#19
EDITORIAL
Jaume Alijotas-Reig
No abstract text is available yet for this article.
August 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28691954/maintenance-belatacept-based-immunosuppression-in-lung-transplantation-recipients-who-failed-calcineurin-inhibitors
#20
Carlo J Iasella, Ryan J Winstead, Cody A Moore, Bruce A Johnson, Ayelet T Feinberg, Matthew R Morrell, J W Awori Hayanga, Elizabeth A Lendermon, Adriana Zeevi, John F McDyer, Christopher R Ensor
BACKGROUND: Traditional immunosuppressive regimens (ISR) used in lung transplantation rely on calcineurin inhibitors (CNI) that occasionally cause severe adverse reactions necessitating discontinuation. Belatacept is a novel costimulation antagonist approved for use in renal transplantation which lacks data in lung transplantation. This series aims to describe the response to belatacept ISR in 11 lung transplantation recipients after CNI failure. METHODS: Single center, retrospective medical record review of adult lung transplant recipients (LTR) before and after conversion to belatacept-based ISR...
July 6, 2017: Transplantation
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