124 results match your criteria testing adamts13


ADAMTS13 testing update: Focus on laboratory aspects of difficult thrombotic thrombocytopenic purpura diagnoses and effects of new therapies.

Authors:
Kristi J Smock

Int J Lab Hematol 2021 Jul;43 Suppl 1:103-108

Department of Pathology, ARUP Laboratories, University of Utah, Salt Lake City, UT, USA.

TTP is a life-threatening disorder diagnosed using a combination of clinical information and laboratory results. ADAMTS13 activity and antibody testing represent a major advance in the field, but results can sometimes be difficult to interpret due to technical aspects of the tests and characteristics of the causative antibodies in acquired TTP. Genetic testing for ADAMTS13 mutations is also now available to assist with the diagnosis of inherited TTP. Read More

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Indicators Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies in a Canadian Apheresis Referral Center.

Am J Clin Pathol 2021 Jun 23. Epub 2021 Jun 23.

Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada.

Objectives: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy (TMA) caused by ADAMTS13 deficiency with mortality of up to 90% in the absence of treatment, typically therapeutic plasma exchange (TPE). TTP presents similarly to other TMAs in which TPE is ineffective and associated with morbidity and additional costs. Thus, we sought to assess clinical and laboratory parameters differentiating TTP from other TMAs in our institution's catchment population. Read More

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Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond.

Am J Hematol 2021 08 31;96(8):1049-1055. Epub 2021 May 31.

Section of Clinical Biochemistry, University of Verona, Verona, Italy.

The metalloproteinase ADAMTS13 (a disintegrin with a thrombospondin type 1 motif, member 13), also known as VWF (von Willebrand factor) protease, may be assessed in a vast array of clinical conditions. Notably, a severe deficiency of ADAMTS13 characterizes TTP (thrombotic thrombocytopenic purpura), a rare but potentially fatal disorder associated with thrombosis due to accumulation of prothrombotic ultra-large VWF multimers. Although prompt identification/exclusion of TTP can be facilitated by rapid ADAMTS13 testing, the most commonly utilized assays are based on ELISA (enzyme linked immunosorbent assay) and require long turnaround time and have relatively limited throughput. Read More

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Early ADAMTS13 testing associates with pre-eclampsia occurrence in antiphospholipid syndrome.

Thromb Res 2021 07 27;203:101-109. Epub 2021 Apr 27.

Department of Gynaecology and Obstetrics, First Moscow State Medical University (Sechenov University), Russian Federation; Department of Haematology, CHU Nîmes, Univ Montpellier, Nîmes, France; Faculty of Pharmaceutical and Biological Sciences, Montpellier University, Montpellier, France; UA 011 INSERM- Université de Montpellier, Institut Desbrest d'Epidémiologie et de Santé Publique, Montpellier, France. Electronic address:

Introduction: Women with obstetric antiphospholipid syndrome (oAPS) still develop placental diseases, mainly pre-eclampsia (PEcl), which diagnosis is associated with reduced ADAMTS13 levels. Testing ADAMTS13 in newly pregnant oAPS may provide evidence for risk stratification.

Materials And Methods: We retrospectively investigated the prognostic value of ADAMTS13 activity, antigen and antibodies on stored plasma samples obtained prior to beginning low-molecular weight heparin-low dose aspirin treatment in 513 oAPS women. Read More

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Serum and cerebrospinal fluid host proteins indicate stroke in children with tuberculous meningitis.

PLoS One 2021 30;16(4):e0250944. Epub 2021 Apr 30.

Department of Paediatrics and Child Health, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa.

Introduction: Stroke is a common complication in children with tuberculous meningitis (TBM). Host proteins may give us insight into the mechanisms of stroke in TBM and serve as biomarkers for detection of stroke, however, they have not been widely explored. In this study, we compared the concentrations of cerebrospinal fluid (CSF) and serum proteins between children who had TBM-related stroke and children with TBM without stroke. Read More

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Genetic justification of severe COVID-19 using a rigorous algorithm.

Clin Immunol 2021 05 13;226:108726. Epub 2021 Apr 13.

Hematology Division, Department of Internal Medicine, Johns Hopkins University, Baltimore, USA.

Recent studies suggest excessive complement activation in severe coronavirus disease-19 (COVID-19). The latter shares common characteristics with complement-mediated thrombotic microangiopathy (TMA). We hypothesized that genetic susceptibility would be evident in patients with severe COVID-19 (similar to TMA) and associated with disease severity. Read More

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Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome With Severe Thrombocytopenia and Severe ADAMTS13 Activity Deficiency.

Obstet Gynecol 2021 05;137(5):873-876

Division of Obstetrics and Gynecology, University of British Columbia and Surrey Memorial Hospital, Vancouver, British Columbia, Canada.

Background: Differentiating preeclampsia with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome from thrombotic thrombocytopenic purpura (TTP) can present a diagnostic dilemma.

Case: We report the case of a 34-year-old woman, G1P0, with monochorionic diamniotic twins who presented with new-onset blurry vision, hypertension, and a platelet count of 4×109/L. After a multidisciplinary discussion, a diagnosis of atypical HELLP syndrome was made, despite overlapping features concerning for TTP. Read More

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Immature platelet dynamics correlate with ADAMTS13 deficiency and predict therapy response in immune-mediated thrombotic thrombocytopenic purpura.

Thromb Res 2021 02 28;198:72-78. Epub 2020 Nov 28.

University Hospitals Cleveland Medical Center and Case Western Reserve University School of Medicine, Cleveland, OH, United States of America. Electronic address:

Introduction: Thrombotic thrombocytopenic purpura (TTP) requires prompt initiation of therapeutic plasma exchange (TPE) to avoid significant morbidity and mortality. ADAMTS13 activity testing defines TTP, however, at most institutions this is a send-out test and therapy is often initiated prior to measurement availability. We describe our experience looking at absolute immature platelet counts (A-IPC) in patients suspected with TTP at presentation and in response to therapy. Read More

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February 2021

PLASMIC score: Not intended to replace but rather to prompt the ADAMTS13 testing.

Transfusion 2020 12;60(12):3070-3072

Thrombosis and Hemostasis Unit, Fondazione I.R.C.C.S. Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.

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December 2020

Spontaneous recovery in a patient with acquired thrombotic thrombocytopenic purpura (TTP): observation of a 'subclinical' TTP state.

Hematology 2020 Dec;25(1):473-477

Section of Hematology, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.

Objectives: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy that can have high mortality rates without prompt treatment. Standard treatment is urgent plasma exchange (PLEX), which leads to disease remission in the vast majority of patients. Deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) alone is not sufficient to cause the clinical manifestations characteristic of TTP. Read More

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December 2020

Multicentric evaluation of the new HemosIL Acustar chemiluminescence ADAMTS13 activity assay.

Int J Lab Hematol 2021 Jun 2;43(3):485-493. Epub 2020 Dec 2.

Hospital del Sagrat Cor, Universitat Internacional de Catalunya, Banc de Sang i Teixits de Catalunya, Barcelona, Spain.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy (TMA) characterized by the severe deficiency of ADAMTS13 activity (<10%). Rapid ADAMTS13 testing is crucial for early diagnosis and optimal management of TTP patients and other TMAs. The objective of this study was to retrospectively evaluate the performance of the recently commercialized HemosIL Acustar ADAMTS13 activity chemiluminescent immunoassay (Instrumentation Laboratory, Bedford, Massachusetts, United States) in a multicentric study between Spain and Portugal. Read More

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Reduced sensitivity of PLASMIC and French scores for the diagnosis of thrombotic thrombocytopenic purpura in older individuals.

Transfusion 2021 01 12;61(1):266-273. Epub 2020 Nov 12.

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by thrombocytopenia, microangiopathic hemolysis, and ischemic organ failure. The PLASMIC and French TTP scores can help guide clinical decisions when ADAMTS13 testing is not immediately available. Older individuals often present atypically, but the impact of age on these tools is not known. Read More

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January 2021

A multicenter laboratory assessment of a new automated chemiluminescent assay for ADAMTS13 activity.

J Thromb Haemost 2021 02 21;19(2):417-428. Epub 2020 Nov 21.

Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal disorder caused by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency. Prompt identification/exclusion of TTP can thus be facilitated by rapid ADAMTS13 testing. The most commonly utilized (enzyme-linked immunosorbent assay [ELISA]-based) assay takes several hours to perform and so does not generally permit rapid testing. Read More

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February 2021

Clinical Features and Gene Mutation Analysis of Congenital Thrombotic Thrombocytopenic Purpura in Neonates.

Authors:
Jiali Wang Li Zhao

Front Pediatr 2020 22;8:546248. Epub 2020 Sep 22.

Department of Neonatology, Children's Hospital of Nanjing Medical University, Nanjing, China.

Congenital thrombotic thrombocytopenic purpura (TTP) is a rare hereditary disease with a high mortality rate; however, improved patient survival is possible with prompt diagnosis and treatment. The clinical features and mutation sites of a disintegrin and metalloproteinase with thrombospondin motifs () in congenital TTP were analyzed in a neonate with suspected congenital TTP. High-throughput sequencing, polymerase chain reaction, and Sanger sequencing were utilized for screening of genes related to thrombocytopenic diseases and gene mutation testing on blood samples from the neonate and the parents. Read More

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September 2020

ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura.

J Thromb Haemost 2020 10 11;18(10):2486-2495. Epub 2020 Sep 11.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy.

Background: Despite an increase in our understandings of pathogenesis of thrombotic thrombocytopenic purpura (TTP), the approaches for initial diagnosis and management of TTP vary significantly.

Objective: The evidence-based guidelines of the International Society on Thrombosis and Haemostasis (ISTH) are intended to support patients, clinicians, and other health care professionals in their decisions about the initial diagnosis and management of acute TTP.

Methods: In June 2018, ISTH formed a multidisciplinary panel that included hematologists, an intensive care physician, nephrologist, clinical pathologist, biostatistician, and patient representatives, as well as a methodology team from McMaster University. Read More

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October 2020

Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.

Blood 2020 11;136(19):2103-2117

Maternité Port-Royal, Hôpital Cochin, Université de Paris/AP-HP, Fighting Prematurity University Hospital Federation (FHU PREMA), INSERM UMR 1139, Paris, France.

Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill defined. This report, by an international multidisciplinary working group of obstetricians, nephrologists, hematologists, intensivists, neonatologists, and complement biologists, summarizes the current knowledge of these potentially severe disorders and proposes a practical clinical approach to diagnose and manage an episode of pregnancy-associated TMA. Read More

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November 2020

MED-TMA: A clinical decision support tool for differential diagnosis of TMA with enhanced accuracy using an ensemble method.

Thromb Res 2020 09 27;193:154-159. Epub 2020 Jun 27.

Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea; Center for Precision Medicine, Seoul National University Hospital, Seoul, Republic of Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea; Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea. Electronic address:

Considering difficulties in on-site ADAMTS13 testing and the performance instability of PLASMIC score according to ethnicity, we developed a prediction tool, MED-TMA (machine learning (ML) method for differential diagnosis (DDx) of thrombotic microangiopathy (TMA)) to support clinical decision. Data from 319 patients visiting 31 hospitals in Korea clinically diagnosed with primary TMA was randomly separated by 2:1 into two groups - the development dataset (D-set, n = 212), the validation dataset (V-set, n = 107). Feature elimination was conducted to select optimal clinical predictors. Read More

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September 2020

Validation of PLASMIC score: an academic medical center case series (2012-present).

Transfusion 2020 07 26;60(7):1536-1543. Epub 2020 Jun 26.

Department of Pathology and Laboratory Medicine, Center for Transfusion and Cellular Therapies, Emory University School of Medicine, Atlanta, Georgia, USA.

Background: The PLASMIC score is a rapid and inexpensive tool for predicting severe ADAMTS13 deficiency (activity <10%) in patients with suspected thrombotic thrombocytopenic purpura (TTP) by analyzing seven parameters (platelet count; combined hemolysis variable; absence of active neoplasia; absence of an organ or stem-cell transplant; mean corpuscular value; international normalized ratio; and serum creatinine). The purpose of this study was to validate the PLASMIC score at a large multi-institutional academic medical center.

Methods: An internal database of consultations to the transfusion medicine service, which oversees therapeutic apheresis at our institution, was reviewed to identify patients who were evaluated for and/or received plasma exchange for suspected TTP. Read More

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Hereditary Thrombotic Thrombocytopenic Purpura in a 9-Month-old: Diagnosing and Managing an Ultra-rare Disorder.

J Pediatr Hematol Oncol 2021 05;43(4):e577-e579

Department of Pediatric and Adolescent Medicine, Division of Pediatric Hematology/Oncology.

Hereditary thrombotic thrombocytopenic purpura is an ultra-rare disorder caused by biallelic mutations in the ADAMTS13 gene. Because it can be difficult to diagnose, plasma ADAMTS13 activity assessment should be considered in patients with thrombocytopenia, anemia, and schistocytes on peripheral blood smear. We present the diagnostic evaluation of a patient with hereditary thrombotic thrombocytopenic purpura. Read More

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Combined Immature Platelet Fraction and Schistocyte Count to Differentiate Pregnancy-Associated Thrombotic Thrombocytopenic Purpura from Severe Preeclampsia/Haemolysis, Elevated Liver Enzymes, and Low Platelet Syndrome (SPE/HELLP).

Indian J Hematol Blood Transfus 2020 Apr 5;36(2):316-323. Epub 2019 Oct 5.

5Department of Clinical Pathology, Misr University for Science and Technology (MUST), Giza, Egypt.

The occurrence of thrombotic microangiopathy (TMA) in pregnancy is an unfortunate emergency condition. Proper diagnosis is mandatory which requires the consideration of two overlapping diagnoses: severe preeclampsia/haemolysis, elevated liver enzymes, and low platelet syndrome (SPE/HELLP) and thrombotic thrombocytopenic purpura (TTP). The long turn-around times of ADAMTS13 testing precludes the timely distinction between the two conditions. Read More

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Acute thrombotic thrombocytopenic purpura in Louisiana: Seasonal distribution and evaluation of an ADAMTS13 order screening protocol.

J Clin Apher 2020 Aug 14;35(4):264-270. Epub 2020 May 14.

Department of Pathology & Laboratory Medicine, Ochsner Health System, New Orleans, Louisiana, USA.

Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by inactivation of ADAMTS13. It is characterized by thrombocytopenia and microangiopathic hemolytic anemia. Previous studies have produced conflicting data regarding seasonal association of TTP diagnoses. Read More

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HLA loci predisposing to immune TTP in Japanese: potential role of the shared ADAMTS13 peptide bound to different HLA-DR.

Blood 2020 06;135(26):2413-2419

Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder caused by neutralizing anti-ADAMTS13 autoantibodies. In white individuals, HLA allele DRB1*11 is a predisposing factor for iTTP, whereas DRB1*04 is a protective factor. However, the role of HLA in Asians is unclear. Read More

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Potential impact of a delayed ADAMTS13 result in the treatment of thrombotic microangiopathy: an economic analysis.

Vox Sang 2020 Jul 30;115(5):433-442. Epub 2020 Mar 30.

Department of Pathology, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.

Background: A pre-plasma exchange ADAMTS13 measurement differentiates thrombotic thrombocytopenic purpura (TTP) from other forms of thrombotic microangiopathy (TMA). Given that many hospitals do not perform the ADAMTS13 assay in-house and that the turnaround time (TAT) differs among reference laboratories, we performed an analysis investigating the potential impact of a delay in obtaining the results on the healthcare system.

Methods: An economic model was developed to estimate the impact of a delay in obtaining the pretreatment ADAMTS13 results on patients admitted with TMA with cost (US dollars) as the primary outcome. Read More

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When to consider targeted therapies in thrombotic microangiopathies in the modern era: walking the tightrope between cost, safety, and efficacy.

J Thromb Thrombolysis 2020 May;49(4):602-605

Division of Hematology, Oregon Health & Science University, Knight Cancer Institute, 3181 SW Sam Jackson Park Rd, Portland, OR, 97239, USA.

Thrombotic Microangiopathy (TMA) is a heterogeneous collection of syndromes that encompasses TTP, HUS, and other processes characterized by thrombocytopenia, microangiopathic hemolytic anemia, and, if untreated, organ failure and death. Novel therapies have recently been approved for the management of certain thrombotic microangiopathies, including caplacizumab for immune-mediated TTP, and eculizumab for atypical HUS. These options have complicated the standard workflow, which includes initiation of plasma exchange until ADAMTS13 testing can be resulted. Read More

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Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study.

J Thromb Thrombolysis 2020 Oct;50(3):628-631

Department of Hemostaseology and Hemophilia Center, University Hospital Frankfurt, Theodor Stern Kai 7, 60596, Frankfurt, Germany.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severely reduced activity of the von-Willebrand factor-cleaving protease ADAMTS13, mainly caused by anti-ADAMTS-13 antibodies. Although several test systems for ADAMTS13 measurement exist, long turn-around times hamper the usability in daily practice. We performed a method comparison study for two commercially available ADAMTS13 assays and evaluated the agreement between the fully-automated rapid turn-over HemosIL AcuStar ADAMTS13 Activity assay and the manually performed TECHNOZYM ADAMTS-13 Activity assay. Read More

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October 2020

Clinical Characteristics and Outcome of Canadian Patients Diagnosed With Atypical Hemolytic Uremic Syndrome.

Can J Kidney Health Dis 2020 24;7:2054358119897229. Epub 2020 Jan 24.

Hôtel-Dieu de Québec, Quebec, QC, Canada.

Background: Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, heterogeneous disease of uncontrolled activation of the alternative complement pathway that is difficult to diagnose. We have evaluated the Canadian patients enrolled in the Global aHUS Registry to provide a Canadian perspective regarding the diagnosis and management of aHUS and the specific challenges faced.

Objective: To evaluate Canadian patients enrolled in the Global aHUS Registry to provide a Canadian perspective regarding the diagnosis and management of aHUS and the specific challenges faced. Read More

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January 2020

Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Systematic Review.

Obstet Gynecol 2020 01;135(1):46-58

Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, the University of Texas Health Science Center at Houston, Houston, Texas; and the Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Cedars-Sinai Medical Center, Los Angeles, California.

Objective: To evaluate disease presentation, diagnosis, treatment, and clinical outcomes in pregnancy-associated atypical hemolytic uremic syndrome (aHUS).

Data Sources: We searched PubMed, MEDLINE, Cochrane Library, ClinicalTrials.gov, Web of Science, EMBASE and Google Scholar, from inception until March 2018. Read More

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January 2020

Evaluation of a New, Rapid, Fully Automated Assay for the Measurement of ADAMTS13 Activity.

Thromb Haemost 2019 Nov 6;119(11):1767-1772. Epub 2019 Oct 6.

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, A. Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy.

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy (TMA) characterized by the severe deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity (< 10%). Rapid ADAMTS13 testing is crucial for an early diagnosis and optimal management of acute TTP. We evaluated the performance of the HemosIL AcuStar ADAMTS13 activity assay (Instrumentation Laboratory, Bedford, Massachusetts, United States), a fully automated chemiluminescent immunoassay with an analytical time of 33 minutes. Read More

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November 2019

Congenital thrombotic thrombocytopenic purpura presenting in adulthood with recurrent cerebrovascular events.

BMJ Case Rep 2019 Oct 3;12(10). Epub 2019 Oct 3.

Bristol Haematology and Oncology Centre, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, life-threatening disease, characterised by episodes of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and small vessel thrombosis. We describe a case of cTTP first diagnosed at age 70 years in a female presenting with an acute ischaemic stroke and thrombocytopenia, in whom A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS13) levels were <10%, suggestive of thrombotic thrombocytopaenic purpura (TTP). The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. Read More

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October 2019

Active Hepatitis C Leading to Refractory Thrombotic Thrombocytopenic Purpura - A Dubious Association and The Challenges Faced in Management.

Cureus 2019 Jul 16;11(7):e5147. Epub 2019 Jul 16.

Internal Medicine, University of Pittsburgh Medical Center - UPMC - Pinnacle, Harrisburg, USA.

Acquired thrombotic thrombocytopenic purpura is a combination of thrombocytopenia with microangiopathic hemolytic anemia. A 62-year-old female was transferred from an outside hospital for rapidly worsening mental status and severe thrombocytopenia. Laboratory studies were significant for reduced hemoglobin and platelet count along with raised blood urea nitrogen, creatinine, and serum lactate dehydrogenase levels. Read More

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