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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29915654/a-dramatic-recovery-in-a-patient-initially-expected-to-die-of-ttp-its-complications
#1
Jennifer Ogilvie, Jaskeerat Singh
Thrombotic thrombocytopenic purpura (TTP) has a high fatality rate if not caught early and treated with plasmapheresis. When TTP patients present late in their sequelae with neuro symptoms, an elevated lactate dehydrogenase and systemic symptoms, there is a high mortality rate. This report describes the case of a young female who had no significant medical problems and presented to our hospital after several days of hematuria, new onset blurry vision and dizziness. She was found to have thrombocytopenia and microangiopathic hemolytic anemia consistent with TTP and was thus started on plasmapheresis...
2018: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29889319/pediatric-thrombotic-thrombocytopenic-purpura
#2
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
June 11, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29885460/adamts-proteins-in-human-disorders
#3
REVIEW
Timothy J Mead, Suneel S Apte
ADAMTS proteins are a superfamily of 26 secreted molecules comprising two related, but distinct families. ADAMTS proteases are zinc metalloendopeptidases, most of whose substrates are extracellular matrix (ECM) components, whereas ADAMTS-like proteins lack a metalloprotease domain, reside in the ECM and have regulatory roles vis-à-vis ECM assembly and/or ADAMTS activity. Evolutionary conservation and expansion of ADAMTS proteins in mammals is suggestive of crucial embryologic or physiological roles in humans...
June 6, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29871663/two-potentially-lethal-conditions-of-probable-immune-origin-occurring-in-a-pregnant-woman-a-case-report
#4
H M Senanayake, M Patabendige
BACKGROUND: Thrombotic thrombocytopenic purpura and peripartum cardiomyopathy are potentially lethal complications of pregnancy. We describe a case in which both of these developed in the same patient. The etiologies of both conditions remain uncertain, but they share immune hyperreactivity as a possible cause. CASE PRESENTATION: A 33-year-old Lankan primigravida gave birth at 38 weeks of gestation by cesarean section when she presented with right-sided abdominal pain and a provisional diagnosis of appendicitis...
June 6, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29868970/does-this-patient-with-thrombotic-thrombocytopenic-purpura-have-a-cardiac-involvement
#5
Lara Zafrani, Lene Russell, Elie Azoulay
No abstract text is available yet for this article.
June 4, 2018: Intensive Care Medicine
https://www.readbyqxmd.com/read/29847840/targeting-von-willebrand-factor-in-ischaemic-stroke-focus-on-clinical-evidence
#6
Nina Buchtele, Michael Schwameis, James C Gilbert, Christian Schörgenhofer, Bernd Jilma
Despite great efforts in stroke research, disability and recurrence rates in ischaemic stroke remain unacceptably high. To address this issue, one potential target for novel therapeutics is the glycoprotein von Willebrand factor (vWF), which increases in thrombogenicity especially under high shear rates as it bridges between vascular sub-endothelial collagen and platelets. The rationale for vWF as a potential target in stroke comes from four bodies of evidence. (1) Animal models which recapitulate the pathogenesis of stroke and validate the concept of targeting vWF for stroke prevention and the use of the vWF cleavage enzyme ADAMTS13 in acute stroke treatment...
June 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29846661/therapeutic-plasma-exchange-performed-via-2-apheresis-devices-and-its-impact-on-the-blood-profile-of-hematological-and-neurological-patients
#7
Eiman Hussein
Background: An efficient apheresis device performs therapeutic plasma exchange (TPE) sessions in short times, while avoiding significant potential cell losses in the removed plasma. Methods: To evaluate TPE performed via Optia (hereinafter, Optia) and COBE Spectra (hereinafter, COBE) apheresis devices, a paired randomized comparison study was conducted. We evaluated the device performance and its impact on cell loss in the removed plasma, as well as blood counts of patients...
May 26, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29786866/characterization-of-efficacy-and-safety-of-pathogen-inactivated-and-quarantine-plasma-in-routine-use-for-treatment-of-acquired-immune-thrombotic-thrombocytopenic-purpura
#8
R Herbrecht, M Ojeda-Uribe, D Kientz, C Fohrer, A Bohbot, O Hinschberger, K-L Liu, E Remy, C Ernst, J-S Lin, L Corash, J P Cazenave
BACKGROUND: Auto-immune thrombotic thrombocytopenic purpura (TTP) is a morbid multi-organ disorder. Cardiac involvement not recognized in initial disease descriptions is a major cause of morbidity. Therapeutic plasma exchange (TPE) requires exposure to multiple plasma donors with risk of transfusion-transmitted infection (TTI). Pathogen inactivation (PI) with amotosalen-UVA, the INTERCEPT Blood System for Plasma (IBSP) is licensed to reduce TTI risk. METHODS: An open-label, retrospective study evaluated the efficacy of quarantine plasma (QP) and IBSP in TTP and defined treatment emergent cardiac abnormalities...
May 21, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#9
REVIEW
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
May 17, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29777164/mrna-treatment-produces-sustained-expression-of-enzymatically-active-human-adamts13-in-mice
#10
Susan Liu-Chen, Brendan Connolly, Lei Cheng, Romesh R Subramanian, Zhaozhong Han
Thrombotic thrombocytopenic purpura (TTP) is primarily caused by deficiency of ADAMTS13 within the blood stream due to either genetic defects or presence of inhibitory autoantibodies. Preclinical and clinical studies suggest that enzyme replacement therapy with recombinant human ADAMTS13 protein (rhADAMTS13) is effective and safe in treatment of TTP. However, frequent dosing would be required due to the relatively short half-life of rhADAMTS13 in circulation as well as the presence of inhibitory autoantibodies that collectively result in the poor pharmacological profile of rhADAMTS13...
May 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29771863/upshaw-schulman-syndrome-with-c-2728c-t-mutation-in-adamts13-gene
#11
Shahzadi Resham, Zehra Fadoo, Bushra Moiz
Congenital thrombotic thrombocytopenic purpura is a rare autosomal recessive disorder presenting with hemolytic anemia, thrombocytopenia, micro vascular thrombosis, and end organ damage. Here, we present a case of a 7-year-old girl having recurrent neonatal hemolysis, developmental delay, frequent seizures, and thrombocytopenia. Characteristic clinical picture and gene sequencing of a disintegrin and metalloproteinase with thrombospondin motifs 13 confirmed the diagnosis of Upshaw-Schulman syndrome. She was treated successfully with plasma infusion...
May 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29763513/child-onset-thrombotic-thrombocytopenic-purpura-caused-by-p-r498c-and-p-g259pfsx133-mutations-in-adamts13
#12
An-Sofie Schelpe, Christelle Orlando, Bogaç Ercig, Chloë Geeroms, Inge Pareyn, Nele Vandeputte, Leydi Carolina Velásquez Pereira, Elien Roose, Karel Fostier, Gerry A F Nicolaes, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke, Kristin Jochmans
INTRODUCTION: Patients suffering from congenital thrombotic thrombocytopenic purpura (cTTP) have a deficiency in ADAMTS13 due to mutations in their ADAMTS13 gene. OBJECTIVE: The aim of this study was to determine ADAMTS13 parameters (activity, antigen and mutations), to investigate if the propositus suffered from child-onset cTTP and to study the in vitro effect of the ADAMTS13 mutations. METHODS: ADAMTS13 activity and antigen were determined using the FRETS VWF73 assay and ELISA and ADAMTS13 mutations via sequencing of the exons...
May 15, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29761111/myocardial-infarction-as-an-early-presentation-in-thrombotic-thrombocytopenic-purpura-a-rare-case-series
#13
Sumit Dahal, Dipesh K C Ghimire, Saroj Sapkota, Suyash Dahal, Paritosh Kafle, Manjul Bhandari
Renal and neurological involvements are frequently seen in thrombotic thrombocytopenic purpura (TTP). Cardiac involvement, however, has been rarely reported. In this article, we present 2 cases of myocardial infarction in patients with TTP. In the first case, a young man presented with non-ST-segment elevation myocardial infarction that resolved promptly with plasmapheresis. The second patient developed ST-segment elevation myocardial infarction early in the course of the disease and died before plasmapheresis could be initiated...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29758989/the-double-hazard-of-bleeding-and-thrombosis-in-hemostasis-from-a-clinical-point-of-view-a-global-assessment-by-rotational-thromboelastometry-rotem
#14
Olga Meltem Akay
Hemostasis is a complex dynamic process involving bleeding and thrombosis as two end-points. Conventional coagulation tests which are measured in plasma examine only isolated portions of the coagulation cascade, thereby giving no information on important interactions essential to the clinical evaluation of hemostatic function. Thromboelastography (TEG), originally described in 1948 has improved over the decades and become a valuable tool of coagulation testing because of the limitations of standard coagulation tests...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29753419/severe-renal-failure-and-thrombotic-microangiopathy-induced-by-malignant-hypertension-successfully-treated-with-spironolactone
#15
H M Villafuerte Ledesma, J M Peña Porta, P Iñigo Gil, P Martin Azara, J E Ruiz Lalglesia, A Tomás LaTorre, A Martínez Burillo, P Vernet Perna, R Álvarez Lipe
Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0...
May 9, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29744115/thrombotic-thrombocytopenic-purpura-possibly-triggered-by-graves-disease
#16
Saurabh D Chitnis, Tuoyo O Mene-Afejuku, Amandeep Aujla, Ahmed Shady, Gaby S Gil, Eder Hans Cativo, Andrea Popescu-Martinez
Thrombotic thrombocytopenic purpura (TTP) is a part of a spectrum of thrombotic microangiopathy syndromes which are mainly characterized by platelet aggregation causing microangiopathic hemolytic anemia, thrombocytopenia and microvascular occlusion. In literature, very few cases expressing a direct association between pre-existing Grave's disease and TTP have been described. A 37-year-old African-American woman with past medical history of Grave's disease and polysubstance abuse who presented with complaints of dyspnoea at rest and chest pain was diagnosed to have TTP on further evaluation...
October 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29739267/relapsed-refractory-thrombotic-thrombocytopenic-purpura-treated-with-n-acetylcysteine-a-case-report
#17
Sinan Demircioğlu, Ömer Ekinci, Ali Doğan, Cengiz Demir
Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition. However, some cases may be non-responsive to plasma exchange, or loss of response may occur. Treatment options for refractory cases include high-dose corticosteroids, rituximab, vincristine, cyclophosphamide, splenectomy, bortezomib and N-acetylcysteine. We present a refractory case of thrombotic thrombocytopenic purpura responding to the last of these therapies...
January 1, 2018: Scottish Medical Journal
https://www.readbyqxmd.com/read/29737462/validation-of-plasmic-score-and-follow-up-data-in-a-cohort-of-patients-with-suspected-microangiopathies-from-southern-italy
#18
Giovanni Luca Tiscia, Angelo Ostuni, Nicola Cascavilla, Filomena Cappucci, Potito Scalzulli, Cosima Battista, Antonio Abrescia, Filippo Aucella, Caterina Buquicchio, Maurizio Brigante, Giovanna D'Andrea, Bruno Di Paolo, Giulio Giordano, Barbara Infante, Silvia Piano, Prudenza Ranieri, Livio Tullo, Elvira Grandone
Severe ADAMTS13 deficiency (activity < 10%) is pathognomonic of thrombotic thrombocytopenic purpura. ADAMTS13 testing is time-consuming and unavailable in many hospitals. Recently, a seven-variables score named PLASMIC score, has been developed to stratify acute patients, based on their risk of having a severe ADAMTS13 deficiency. We present the application of this score in a cohort of patients referred to our Center. From 2012 to 2017, 42 patients with suspected thrombotic microangiopathies from 6 Centers were referred to Hemostasis and Thrombosis Center of "Casa Sollievo della Sofferenza" Hospital/Research Institute for ADAMTS13 testing...
May 8, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#19
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29727738/differential-sensitivity-of-von-willebrand-factor-activity-assays-to-reduced-vwf-molecular-weight-forms-a-large-international-cross-laboratory-study
#20
Emmanuel J Favaloro, Roslyn Bonar, Martine J Hollestelle, Ian Jennings, Soma Mohammed, Piet Meijer, Timothy Woods, Muriel Meiring
INTRODUCTION: von Willebrand disease (VWD), the most common inherited bleeding disorder, is due to deficiencies/defects in von Willebrand factor (VWF). Effective diagnosis requires testing for FVIII, VWF antigen and one or more VWF 'activity' assays. Classically, 'activity' is assessed using ristocetin cofactor (VWF:RCo), but collagen binding (VWF:CB) and/or other assays are used by many laboratories. This extensive international cross-laboratory study has specifically evaluated contemporary VWF activity assays for comparative sensitivity to reduction in high molecular weight (HMW) VWF, and their ability to differentiate type 1 vs 2A VWD-like samples...
April 19, 2018: Thrombosis Research
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