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Thrombotic microangiopathy

Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
Daniel W Abbott, Kenneth D Friedman, Matthew S Karafin
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia that requires emergent treatment with plasma exchange and is one of the most important conditions for which apheresis service professionals are consulted. Careful interpretation of initial laboratory values and the peripheral blood smear is a critical first step to determining the need for plasma exchange because other conditions can show deceptively similar red cell morphology, and ADAMTS13 levels are often not rapidly available...
September 28, 2016: Transfusion and Apheresis Science
I Mancini, I Ricaño-Ponce, E Pappalardo, A Cairo, M M Gorski, G Casoli, B Ferrari, M Alberti, D Mikovic, M Noris, C Wijmenga, F Peyvandi
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor cleaving protease, ADAMTS13. Similarly to other autoimmune disorders, evidences of a genetic contribution have been reported, including the association of the human leukocyte antigen (HLA) class II complex with disease risk OBJECTIVE: To identify novel genetic risk factors in acquired TTP...
October 20, 2016: Journal of Thrombosis and Haemostasis: JTH
Donald M Arnold, Christopher J Patriquin, Ishac Nazy
No abstract text is available yet for this article.
October 17, 2016: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
Omer Akyol, Sumeyya Akyol, Chu-Huang Chen
Endothelial cells (EC) respond to injury by releasing numerous factors, including von Willebrand factor (VWF). High circulating levels of unusually large VWF multimers (UL-VWFM) have strong procoagulant activity and facilitate platelet adhesion and aggregation by interacting with platelets after an acute event superimposed on peripheral arterial disease and coronary artery disease. ADAMTS13-a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13-regulates a key physiological process of coagulation in the circulation by cleaving VWF multimers into small, inactive fragments...
October 13, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
Jeffrey L Turner, Joshua Reardon, Tanios Bekaii-Saab, Spero R Cataland, Matthew J Arango
No abstract text is available yet for this article.
September 20, 2016: Clinical Colorectal Cancer
Fusun Gediz, Bahriye Kadriye Payzin, Sertac Ecemis, Naile Güler, Asu Fergun Yilmaz, Fusun Topcugil, Afig Berdeli
INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy, which develops as a result of defective activity of the alternative complement pathway and excessive complement activation due to genetic or acquired factors. No satisfactory responses were obtained by plasmapheresis, corticosteroids and fresh frozen plasma (FFP) transfusion. However, promising results are obtained in recent years by eculuzimab treatment, which inhibits C5 activation. OBJECTIVE: To evaluate the efficacy, safety and effect of eculizumab on quality of life of adult aHUS patients followed in our center...
September 30, 2016: Transfusion and Apheresis Science
Adrien Flahault, Marguerite Vignon, Marion Rabant, Aurélie Hummel, Laure-Hélène Noël, Danielle Canioni, Bertrand Knebelmann, Felipe Suarez, Khalil El Karoui
INTRODUCTION: We report the case of a multicentric Castleman disease (MCD) with initial renal involvement. Although the renal involvement in this case was typical of MCD, it constitutes a rare presentation of the disease, and in our case the renal manifestations led to the haematological diagnosis. CLINICAL FINDINGS/PATIENT CONCERNS: The patient was admitted for fever, diarrhea, anasarca, lymphadenopathies and acute renal failure. Despite intravenous rehydration using saline and albumin, renal function worsened and the patient required dialysis...
October 2016: Medicine (Baltimore)
Anton M Kolomeyer, Ken K Nischal, Ellen Mitchell
PURPOSE: To report a case of bilateral cavernous sinus thrombosis (CST) in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy. METHODS: Case report. RESULTS: An 8-year-old boy with a medical history of orthotopic heart transplant, posttransplant lymphoproliferative disease, and recurrent infections was hospitalized for nausea, vomiting, and diarrhea. His ocular history included accommodative esotropia, hyperopia with astigmatism, Molluscum contagiosum lid lesions, and idiopathic intracranial hypertension...
October 13, 2016: European Journal of Ophthalmology
Ramachandran Rameshkumar, Sriram Krishnamurthy, Rajesh Nachiappa Ganesh, Subramanian Mahadevan, Parameswaran Narayanan, Ponnarmeni Satheesh, Puneet Jain
BACKGROUND: Septic acute kidney injury (AKI) accounts for more than half of all cases of AKI in critically ill children. The renal histology was found to alter the management in more than two-third of cases of adult acute renal failure. Better insight into the pathogenesis of pediatric septic AKI could be based on developing a clearer appreciation of the histopathological changes. No comprehensive study of the histopathological features of septic AKI in critically ill children has yet been performed...
October 13, 2016: Clinical and Experimental Nephrology
Daniel Croom, Heather Tracy
Thrombotic microangiopathy (TMA) syndromes represent a spectrum of illnesses that share common clinical and pathologic features of microangiopathic hemolytic anemia, thrombocytopenia, and organ injury from pathologic small-vessel thrombosis. At least nine primary TMA syndromes have been described and classified based on common probable etiologies, diagnostic criteria, and treatments. The most recognized of the TMA syndromes include thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS)...
2016: Journal of Special Operations Medicine: a Peer Reviewed Journal for SOF Medical Professionals
Diana Karpman, Sebastian Loos, Ramesh Tati, Ida Arvidsson
Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains...
October 10, 2016: Journal of Internal Medicine
Mony Chenda Morisse, Loay Kontar, Céline Bihan, Mathieu Boone, Emma Lachaier, Dimitri Titeca-Beauport, Julien Maizel, Bruno Chauffert
Cancer-related thrombotic microangiopathy (TMA) is a rare entity whose clinical and biological characteristics have been described in various tumors. Here we describe the first case of cancer-related TMA revealing diffuse bone metastases from an ethmoid sinus carcinoma.
May 2016: Case Reports in Oncology
Bérangère S Joly, Alain Stepanian, Thierry Leblanc, David Hajage, Hervé Chambost, Jérôme Harambat, Fanny Fouyssac, Vincent Guigonis, Guy Leverger, Tim Ulinski, Thérésa Kwon, Chantal Loirat, Paul Coppo, Agnès Veyradier
BACKGROUND: Thrombotic thrombocytopenic purpura is a rare thrombotic microangiopathy, related to a severe ADAMTS13 deficiency (a disintegrin and metalloprotease with thromboSpondin type 1 repeats, member 13; activity <10% of normal). Childhood-onset thrombotic thrombocytopenic purpura is very rare and initially often misdiagnosed, especially when ADAMTS13 deficiency is acquired (ie, not linked to inherited mutations of the ADAMTS13 gene). We aimed to investigate initial presentation, management, and outcome of acquired thrombotic thrombocytopenic purpura in children...
October 3, 2016: Lancet Haematology
Nathaniel C Warner, Leroy B Vaughan, Richard P Wenzel
HIV complicates the diagnostic and therapeutic approaches to idiopathic thrombotic thrombocytopenic purpura (TTP), prompting debate in the literature regarding the benefit of plasma exchange versus simple plasma infusion. Herein we present a case of HIV-TTP, initially treated conservatively with plasma infusion but because of progressive neurologic decline, required urgent plasma exchange for resolution of hematologic derangements and neurologic sequelae. Based on the available literature, there appears to be a spectrum of HIV-associated TTP disorders...
October 6, 2016: Journal of Clinical Apheresis
Satoshi Suzuki, Shihoko Nakajima, Taiki Ando, Keisuke Oda, Manabu Sugita, Kunimi Maeda, Yutaka Nakiri, Yoshinari Takasaki
A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT...
2016: Case Reports in Rheumatology
Jae-Bum Jun, Yunsuk Choi, Hawk Kim, Sun Ho Lee, Joseph Jeong, Jiwon Jung
To date, only 1 case of BK polyomavirus (BKPyV) encephalitis combined with transplant-associated thrombotic microangiopathy has been reported in a hematopoietic stem cell transplantation (HCT) recipient. We report the case of a HCT recipient who developed thrombotic microangiopathy and subsequent BKPyV encephalitis. She died despite treatment with cidofovir, ciprofloxacin, and intravenous immunoglobulin without improvement in mental status. Early suspicion of BKPyV encephalitis in a HCT recipient presenting with altered mentality and hemorrhagic cystitis is important...
October 1, 2016: Transplant Infectious Disease: An Official Journal of the Transplantation Society
Nariman Noorbakhsh-Sabet, Ramin Zand
Thrombotic thrombocytopenic purpura (TTP) is a disease caused by excessive platelet thrombi and multisystem vasculopathy. We report a young patient with refractory TTP who has developed multiple multifocal cerebral microbleeds on his brain magnetic resonance imaging scans. TTP-associated thrombotic microangiopathy resulting in diffuse microthrombi formation in the microcirculation and hypoperfusion might be an underlying etiology.
September 28, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Justine Perrin, Julien Carvelli, Bertrand Gondouin, Laurent Daniel, Megan Fraisse, Céline Gaudon, Fouad Bouzana, Henri Vacher-Coponat, Julie Moussi-Francès, Bertrand Dussol, Noémie Jourde-Chiche
Renal involvement of systemic vasculitides is an emergency in nephrology. Although it has become very rare, the diagnosis of polyarteritis nodosa must be considered in some patients. A 70-year-old patient, previously healthy, presented with acute renal failure and malignant hypertension and abundant albuminuria. Subcutaneous nodule, orchitis and mononeuritis appeared subsequently. The search for auto-immunity or viral infection was negative. Markers of thrombotic microangiopathy, present initially, resolved after blood pressure control...
September 24, 2016: Néphrologie & Thérapeutique
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