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thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29659973/the-diagnostic-challenge-of-acquired-thrombotic-thrombocytopenic-purpura-in-children-case-report-and-review-of-the-literature
#1
Elise Schapkaitz, Tanya Marié Schickerling
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease in children. Despite advances in diagnosis and treatment, acquired TTP remains a challenging disease due to the lack of a simple diagnostic test and the variable response to plasma exchange. Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. TTP was diagnosed, and plasma exchange and corticosteroids were initiated, with an excellent response...
April 12, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29657197/adamts-13-level-in-children-with-severe-diarrhea-associated-hemolytic-uremic-syndrome-unmasking-new-association
#2
Naglaa A Khalifa, Heba H Gawish, Noora A Khalifa, Doaa M Tawfeek, Saed M Morsy
Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657193/atypical-hemolytic-uremic-syndrome-laboratory-characteristics-complement-amplifying-conditions-renal-biopsy-and-genetic-mutations
#3
REVIEW
Mohammad A Hossain, Anmol Cheema, Sheila Kalathil, Ravneet Bajwa, Eric J Costanzo, James Cosentino, Jennifer Cheng, Tushar Vachharajani, Arif Asif
Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29650916/-a-case-of-sigmoid-colon-cancer-with-idiopathic-thrombocytopenic-purpura
#4
Kingo Kashimoto, Kenichiro Fukuda, Yosuke Kamada, Nobuki Yamaoka
An 80-year-old man reported at our hospital with the chiefcomplaint ofconstipation. He was diagnosed with sigmoid colon cancer(T4aN0M0, Stage II). He also had steroid-resistant idiopathic thrombocytopenic purpura(ITP)since 2001. After a high dose g-globulin therapy, he underwent sigmoidectomy and splenectomy simultaneously because ofthe steroidresistant ITP. While some reports suggest that ITP is dominant when it occurs with blood cancer, others suggest that it can be immunologically dominant even when it occurs with solid tumors...
March 2018: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29643888/spectrum-of-complications-of-severe-dka-in-children-in-pediatric-intensive-care-unit
#5
Qalab Abbas, Saba Arbab, Anwar Ul Haque, Khadija Nuzhat Humayun
Objectives: To describe the spectrum of complications of Diabetic Ketoacidosis (DKA) observed in children admitted with severe DKA. Methods: Retrospective review of the medical records of all children admitted with the diagnosis of severe DKA in Pediatric Intensive Care Unit (PICU) of the Aga Khan University Hospital, from January 2010 to December 2015 was done. Data was collected on a structured proforma and descriptive statistics were applied. Results: Total 37 children were admitted with complicated DKA (1...
January 2018: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29629225/igg4-related-disease-in-the-stomach-which-was-confused-with-gastrointestinal-stromal-tumor-gist-two-case-reports-and-review-of-the-literature
#6
Ho Seok Seo, Yoon Ju Jung, Cho Hyun Park, Kyo Young Song, Eun Sun Jung
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration...
March 2018: Journal of Gastric Cancer
https://www.readbyqxmd.com/read/29623717/-idiopathic-thrombocytopenia-refractery-to-therapy-of-cyclosporine-a-in-clinical-practice-case-report
#7
Lucia Schwarzová, Zdenko Pirník, Natália Štecová
Primary immune thrombocytopenia (idiopathic thrombocytopenic purpura; ITP) is an autoimmune disorder characterized by isolated thrombocytopenia without abnormalities in the erythroid and myeloid/lymphoid lineages. The incidence of ITP in adults is estimated at approximately 1.6-3.9 per 100 000 persons/year. The aim of this study was to analyze a case report of a patient with diagnosed immune thrombocytopenia (D69.3) resistant to cyclosporine A therapy as well as an evaluation of factors that could contribute to an inadequate response to cyclosporine A...
2018: Ceská a Slovenská Farmacie
https://www.readbyqxmd.com/read/29621007/disseminated-intravascular-coagulation-is-it-fact-or-fancy
#8
Jae C Chang
: 'Disseminated intravascular coagulation (DIC)' occurs commonly in critical illnesses such as sepsis, trauma, cancer, and complications of surgery and pregnancy. Mortality is very high. The pathogenesis has been ascribed to tissue factor-initiated coagulation disorder, resulting in disseminated microblood clots that are made of platelets, plasma factors, fibrins, and blood cells. True DIC depletes coagulation factors and consumes platelets, and activates fibrinolysis. 'DIC' is assumed to orchestrate thrombocytopenia, microangiopathic hemolytic anemia and hypoxic multiorgan dysfunction syndrome, and causes hemorrhagic disorder due to depleted coagulation factors...
April 4, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29620681/-immune-thrombocytopenia-as-key-feature-of-a-novel-ada2-deficiency-variant-implication-on-differential-diagnostics-of-itp-in-children
#9
Mikael Sundin, Per Marits, Stefan Nierkens, Antonios G A Kolios, Jakob Nilsson
Thrombocytopenia presenting during early childhood is most commonly diagnosed as immune/idiopathic thrombocytopenic purpura (ITP), where the antibody-mediated destruction of thrombocytes is often transient. If treatment is indicated, the majority of patients respond to immune-modulation by intravenous immunoglobulin G infusion or systemic corticosteroids. Differential diagnoses to childhood ITP includes thrombocytopenia due to infections, drugs, rheumatologic conditions, immune dysregulation, and inherited bone marrow failures, for example, congenital amegakaryocytic thrombocytopenia...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29607959/successful-treatment-of-aggressive-mature-b-cell-lymphoma-mimicking-immune-thrombocytopenic-purpura
#10
Koya Ono, Yasushi Onishi, Masahiro Kobayashi, Satoshi Ichikawa, Shunsuke Hatta, Shotaro Watanabe, Yoko Okitsu, Noriko Fukuhara, Ryo Ichinohasama, Hideo Harigae
A 55-year-old woman suffered from hemorrhagic tendency. She had severe thrombocytopenia without any hematological or coagulatory abnormalities, and a bone marrow examination revealed an increased number of megakaryocytes without any abnormal cells or blasts. No lymphadenopathy or hepatosplenomegaly was observed on computed tomography. She was initially diagnosed with immune thrombocytopenic purpura (ITP). None of the treatments administered for ITP produced a response. However, abnormal cells were eventually found during the third bone marrow examination...
March 30, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29595692/color-doppler-ultrasound-diagnosis-of-intrarenal-vein-thrombosis-a-rare-case-report-and-literature-review
#11
Yixiu Zhang, Ying Wang, Jianchu Li, Sheng Cai
RATIONALE: We present a case of intrarenal vein thrombosis (IRVT) diagnosed by ultrasound (US). To the best of our knowledge, this is the first reported case in the imaging literature. PATIENT CONCERNS: A 15-year-old boy with a 4-year history of thrombocytopenic purpura presented to the emergency room with a 2-day history of sudden-onset severe left flank pain associated with gross hematuria. DIAGNOSES: Hypercholesterolemia, proteinuria, and elevated plasma creatinine level were present...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29593914/rituximab-induced-pulmonary-edema-managed-with-extracorporeal-life-support
#12
Jacob R Miller, Warren Isakow, Muhammad F Masood, Patrick Aguilar, Kristen M Sanfilippo, Keki R Balsara, Akinobu Itoh
Though rare, rituximab has been reported to induce severe pulmonary edema. We describe the first report of ECLS utilization for this indication. A 31-year-old female with severe thrombotic thrombocytopenic purpura developed florid pulmonary edema after rituximab infusion. Despite advanced ventilatory settings, she developed severe respiratory acidosis and remained hypoxemic with a significant vasopressor requirement. Since her pulmonary insult was likely transient, ECLS was considered. Due to combined cardiorespiratory failure, she received support with peripheral venoarterial ECLS...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29588177/a-43-year-old-woman-with-unexplained-elevation-of-hcg
#13
Lisa M Johnson, Thomas J Gniadek, Claudia S Cohn, Gary Bachowski, Amy B Karger
OBJECTIVE: This case report investigates an unusual hCG result in a woman who is not pregnant. PATIENT AND METHODS: A 43-year-old woman was admitted for recurrence of thrombotic thrombocytopenic purpura (TTP) and therapeutic plasma exchange (TPE) was initiated. Prior to transitioning the patient from TPE to immunosuppressive therapy, a serum qualitative hCG test was performed and was positive. Several etiologies for elevated hCG were considered and investigated, including heterophile antibody interference, endogenous hCG from pituitary or malignancy, and exogenous hCG...
March 26, 2018: Clinical Biochemistry
https://www.readbyqxmd.com/read/29582550/consensus-opinion-on-diagnosis-and-management-of-thrombotic-microangiopathy-in-australia-and-new-zealand
#14
REVIEW
Lucy C Fox, Solomon J Cohney, Joshua Y Kausman, Jake Shortt, Peter D Hughes, Erica M Wood, Nicole M Isbel, Theo de Malmanche, Anne Durkan, Pravin Hissaria, Piers Blombery, Thomas D Barbour
Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Whilst TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal...
March 27, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29572925/correlating-s100b-with-disease-course-in-a-case-of-new-onset-acquired-thrombotic-thrombocytopenic-purpura-ttp-could-this-be-a-new-predictive-biomarker-in-ttp
#15
Patrick Kuhlman, Peter Miller, Andrew Farland, John Owen, Katharine Batt
Acute thrombotic thrombocytopenic purpura (TTP) is an aggressive thrombotic microangiopathy that if not treated, can have a 90% mortality rate. Clinical manifestations of this disease include profound thrombocytopenia, hemolytic anemia, and end-organ dysfunction. Neurologic symptoms can occur in 80% of patients and range from mild confusion to coma (Scully et al., Br J Haematol 142:819-826). Here, we present the clinical course of a patient diagnosed with new onset acquired TTP who presented with neurologic changes that waxed and waned during her disease course...
March 23, 2018: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29567779/mass-spectrometry-assisted-identification-of-adamts13-derived-peptides-presented-on-hla-dr-and-hla-dq
#16
Johana Hrdinová, Fabian C Verbij, Paul H P Kaijen, Robin B Hartholt, Floris van Alphen, Neubury Lardy, Anja Ten Brinke, Karen Vanhoorelbeke, Pooja J Hindocha, Anne S De Groot, Alexander B Meijer, Jan Voorberg, Ivan Peyron
Formation of microthrombi is a hallmark of acquired thrombotic thrombocytopenic purpura. These microthrombi originate from insufficient processing of ultra large von Willebrand factor multimers by ADAMTS13 due to the development of anti-ADAMTS13 autoantibodies. Several studies have identified the major histocompatibility complex class II alleles HLA-DRB1*11, HLA-DQB1*03 and HLA-DQB1*02:02 as risk factors for acquired thrombotic thrombocytopenic purpura development. Previous research in our department indicated that ADAMTS13 CUB2 domain-derived peptides FINVAPHAR and LIRDTHSLR are presented on HLA-DRB1*11 and HLA-DRB1*03, respectively...
March 22, 2018: Haematologica
https://www.readbyqxmd.com/read/29567368/efficacy-of-eculizumab-in-severe-adamts13-deficient-thrombotic-thrombocytopenic-purpura-ttp-refractory-to-standard-therapies
#17
Ernesto Vigna, Annamaria Petrungaro, Anna Perri, Dario Terzi, Anna Grazia Recchia, Francesco Mendicino, Antonella La Russa, Sabrina Bossio, Laura De Stefano, Francesco Zinno, Renzo Bonofiglio, Fortunato Morabito, Massimo Gentile
Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathic hemolytic anemia (MAHA) defined by mechanical hemolytic anemia, severe thrombocytopenia, and systemic visceral ischemia due to systemic platelet-rich microthrombi. Forty percent of patients with autoimmune TTP experience one or multiple relapses. Patients with refractory TTP are currently managed by corticosteroids, twice-daily PEX, and the anti-CD20 monoclonal antibody rituximab. Herein, we report two cases of severe TTP, refractory to those standard agents...
March 15, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29564686/ofatumumab-for-acute-treatment-and-prophylaxis-of-a-patient-with-multiple-relapses-of-acquired-thrombotic-thrombocytopenic-purpura
#18
Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
March 21, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29563339/germline-mutations-in-the-alternative-pathway-of-complement-predispose-to-hellp-syndrome
#19
Arthur J Vaught, Evan M Braunstein, Jagar Jasem, Xuan Yuan, Igor Makhlin, Solange Eloundou, Andrea C Baines, Samuel A Merrill, Shruti Chaturvedi, Karin Blakemore, C John Sperati, Robert A Brodsky
BACKGROUND: HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a severe variant of hypertensive disorders of pregnancy affecting approximately 1% of all pregnancies, and has significant maternal and fetal morbidity. Previously, we showed that upregulation of the alternative pathway of complement (APC) plays a role in HELLP syndrome. We hypothesize that HELLP syndrome follows a 2-hit disease model similar to atypical hemolytic uremic syndrome (aHUS), requiring both genetic susceptibility and an environmental risk factor...
March 22, 2018: JCI Insight
https://www.readbyqxmd.com/read/29554892/immune-thrombocytopenic-purpura-presenting-in-a-patient-after-renal-transplant-for-diabetic-nephropathy
#20
Raja Muhammad Rashid, Zahid Nabi, Ahmad Zaki Ansari, Quratul-Ain Qaiser
BACKGROUND: Immune thrombocytopenic purpura (ITP) is primarily characterized by immune-mediated destruction of platelets in circulation. Major treatment options range from careful observation, steroids, immunosuppressive medications, immunoglobulins to splenectomy. Interestingly and rarely, ITP has also been reported after solid organ transplantation in patients receiving immunosuppressive medications. While the incidence of new onset ITP after solid organ transplant is comparatively well documented, new onset ITP after renal transplant has only been reported in two patients...
March 20, 2018: BMC Nephrology
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