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https://www.readbyqxmd.com/read/28748411/the-role-of-von-willebrand-factor-in-thrombotic-microangiopathy
#1
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/28722150/lessons-in-the-diagnosis-and-management-of-immune-thrombocytopenic-purpura-in-children
#2
Catherine Cole
No abstract text is available yet for this article.
July 19, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28719512/a-pediatric-case-of-persistent-thrombocytopenia-in-a-patient-who-developed-all
#3
Haroon Shaukat, Jessica O'Mara, Michelle Neier
Inherited thrombocytopenia is a topic that was expanded greatly over the last decade and many new genes are being identified. However, inheritance patterns are not always easy to identify because sporadic cases from de novo mutations may in fact be more common. Few studies have assessed the relationship between thrombocytopenia and malignancies, specifically acute lymphoblastic leukemia (ALL). Here we present a pediatric case of persistent thrombocytopenia associated with T-cell ALL. Our patient was initially diagnosed with immune thrombocytopenic purpura with no evidence of malignancy on bone marrow biopsy but presented shortly after with ALL...
July 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28718212/effectiveness-of-helicobacter-pylori-eradication-in-pregnant-women-with-idiopathic-thrombocytopenic-purpura
#4
Yosuke Ono, Arihiro Shiozaki, Noriko Yoneda, Satoshi Yoneda, Osamu Yoshino, Shigeru Saito
Platelet counts increase after eradication of Helicobacter pylori (Hp) infection in non-pregnant patients with Hp-associated idiopathic thrombocytopenic purpura (ITP); however, improvement in pregnant patients has not yet been reported. We treated four pregnant women for Hp-positive ITP after Hp eradication. In three of four cases, platelet counts increased to levels exceeding 10 × 10(9) /L two weeks after eradication, and these levels were maintained until delivery. Vaginal deliveries were uncomplicated, and no excessive blood loss occurred in any of the cases...
July 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28717280/priapism-with-penile-gangrene-an-unusual-presentation-of-multiple-myeloma
#5
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
#6
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
#7
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
#8
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
#9
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/28702966/possible-role-of-helicobacter-pylori-in-diseases-of-dermatological-interest
#10
C Guarneri, J Lotti, M Fioranelli, M G Roccia, T Lotti, F Guarneri
Helicobacter pylori is a gram-negative, flagellate, microaerophilic bacterium identified for the first time about 30 years ago, as a pathogenic factor of gastritis and peptic ulcer. Soon after, it was linked to several gastrointestinal and extra-gastrointestinal diseases (hematological, cardiovascular, neurological, pulmonary and ocular diseases, obesity, diabetes mellitus, growth retardation and extragastric MALT lymphoma). Association and possible cause-effect correlation with H. pylori infection were suggested in diseases of dermatological interest such as chronic urticaria, rosacea, Henoch-Schoenleins purpura, idiopathic thrombocytopenic purpura, cutaneous and oral lichen planus, atopic dermatitis, recurrent aphthous stomatitis, systemic sclerosis, psoriasis, Sjögrens syndrome, Behçet's disease, pruritus, alopecia areata, primary cutaneous marginal zone B-cell lymphomas, vitiligo, chronic prurigo, multiformis, prurigo nodularis, leukocytoclastic vasculitis, prurigo pigmentosa, eczema nummulare, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, Sweet's syndrome, cutaneous T-cell pseudolymphoma and pemphigus vulgaris...
July 13, 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28702343/eb-virus-reactivation-triggers-thrombotic-thrombocytopenic-purpura-in-a-healthy-adult
#11
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/28698839/dapsone-therapy-for-immune-thrombocytopenic-purpura-old-but-still-unfamiliar
#12
EDITORIAL
Jaewoo Song
No abstract text is available yet for this article.
June 2017: Blood Research
https://www.readbyqxmd.com/read/28697902/acquired-thrombotic-thrombocytopenic-purpura-and-pregnancy-more-light-than-shade-but-controversies-remain
#13
EDITORIAL
Jaume Alijotas-Reig
No abstract text is available yet for this article.
July 5, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28691954/maintenance-belatacept-based-immunosuppression-in-lung-transplantation-recipients-who-failed-calcineurin-inhibitors
#14
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
https://www.readbyqxmd.com/read/28691162/systemic-lupus-erythematosus-following-prodromal-idiopathic-thrombocytopenic-purpura-presenting-with-skin-lesions-resembling-malignant-atrophic-papulosis
#15
M P Wallace, J M Thomas, G Meligonis, T Ha
Systemic lupus erythematosus (SLE) is an autoimmune disease. Its incidence in the UK is approximately 1 per 10 000. Cutaneous involvement, encompassing acute, subacute and chronic disease, occurs in over two-thirds of cases, and can often be the first clue to diagnosis. We describe a highly unusual case of SLE occurring after prodromal idiopathic thrombocytopenic purpura (ITP) and presenting with skin lesions more typical of malignant atrophic papulosis, a rare and often fatal vasculopathy. Such a combination of rare features emphasizes the potential for complexity in this multisystem disease...
July 10, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28689597/other-extrahepatic-manifestations-of-hepatitis-c-virus-infection-pulmonary-idiopathic-thrombocytopenic-purpura-nondiabetes-endocrine-disorders
#16
REVIEW
Daniel Segna, Jean-François Dufour
Extrahepatic manifestations of hepatitis C virus (HCV) infection are a rare but serious condition. This article summarizes the current literature on the association between HCV and endocrine and pulmonary manifestations, as well as idiopathic thrombocytopenic purpura (ITP). HCV may directly infect extrahepatic tissues and interact with the immune system predisposing for obstructive and interstitial lung disease, ITP, autoimmune thyroiditis, infertility, growth hormone and adrenal deficiencies, osteoporosis, and potentially lung and thyroid cancers...
August 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28681548/how-do-we-reduce-plasma-transfusion-in-rhode-island
#17
Christian P Nixon, Maria F Tavares, Joseph D Sweeney
BACKGROUND: Plasma transfusions are given to patients with coagulopathy, either prophylactically, before an invasive procedure; or therapeutically, in the presence of active bleeding; and as an exchange fluid in therapeutic plasma exchange for disorders such as thrombotic thrombocytopenic purpura. There is consensus that many prophylactic plasma transfusions are non-efficacious, and the misdiagnosis of thrombotic thrombocytopenic purpura results in unnecessary therapeutic plasma exchange...
July 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28678087/a-severe-case-of-congenital-thrombotic-thrombocytopenia-purpura-resulting-from-compound-heterozygosity-involving-a-novel-adamts13-pathogenic-variant
#18
Erin Conboy, Paige I Partain, Deepti Warad, Michelle L Kluge, Carola Arndt, Dong Chen, Vilmarie Rodriguez
We report a 9-year-old Chinese girl with congenital thrombotic thrombocytopenic purpura found to be a compound heterozygote for 2 pathogenic variants in the ADAMTS13 gene, including a novel variation. The girl suffered from recurrent, life-threatening episodes of thrombocytopenia and hemolysis, and laboratory testing showed ADAMST13 enzyme activity of <5%. Sequencing of the ADAMTS13 gene revealed a previously reported missense variant, c.1787C>T (p.Ala596Val), and a novel duplication defined as c.1007_1025dup19 (p...
July 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28673116/patterns-of-care-and-burden-of-chronic-idiopathic-thrombocytopenic-purpura-in-brazil
#19
Ana Clara Kneese Virgilio do Nascimento, Joyce Maria Annichino-Bizzacchi, Claudia de Alvarenga Maximo, Eimy Minowa, Guilherme Silva Julian, Rafael Freitas Dos Santos
AIMS: Although several therapeutic options are available for chronic immune thrombocytopenic purpura (cITP), little is known about the treatment of cITP in Brazil. MATERIALS AND METHODS: A multi-center, retrospective chart review, observational study was designed to describe the treatment patterns, clinical burden, resources use, and associated costs for adult patients diagnosed with cITP and treated in public and private institutions in Brazil. Patient charts were screened in reverse chronological order based on their last visit post January 1, 2012...
July 4, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/28671324/rituximab-for-thrombotic-thrombocytopenic-purpura-lessons-from-the-star-trial
#20
Lynne Uhl, Joseph E Kiss, Elizabeth Malynn, Deirdra R Terrell, Sara K Vesely, James N George
No abstract text is available yet for this article.
July 3, 2017: Transfusion
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