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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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[Thrombotic thrombocytopenic purpura].

Rinsho Ketsueki 2021 ;62(5):430-434

Saitama Medical University Hospital.

In this article, I mainly review the molecular targeted therapy for thrombotic thrombocytopenic purpura (TTP). TTP is one of thrombotic microangiopathies (TMA), which demonstrate hemolytic anemia with red blood cell destruction and thrombocytopenia. Another TTP, hemolytic uremic anemia (HUS) reveals bloody stood and acute kidney failure. Read More

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[Recurrent thrombocytopenia with hemolytic anemia in a boy aged 3 years].

Zhongguo Dang Dai Er Ke Za Zhi 2021 May;23(5):524-529

Department of Pediatric Hematologic Oncology, Hunan Provincial People's Hospital/Children's Medical Center of First Affiliated Hospital of Hunan Normal University, Changsha 410005, China.

A boy, aged 3 years and 8 months, had recurrent thrombocytopenia with hemolytic anemia for more than 3 years. The physical examination showed no enlargement of the liver, spleen, and lymph nodes or finger deformities. Laboratory results showed a negative result of the direct antiglobulin test, normal coagulation function, and increases in bilirubin, lactate dehydrogenase and reticulocytes. 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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Double positivity of anti-β-glycoprotein I domain I and anti-phosphatidylserine/prothrombin antibodies enhances both thrombosis and positivity of anti-ADAMTS13 antibody.

J Thromb Thrombolysis 2021 Apr 29. Epub 2021 Apr 29.

Department of Laboratory Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.

Although a few antiphospholipid syndrome (APS) occurs with acquired thrombotic thrombocytopenic purpura (TTP), the relationship between antiphospholipid antibodies (aPL) and anti-ADAMTS13 (anti-a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13) antibody remains uncertain. We investigated the relationship between high-risk thrombotic aPL and anti-ADAMTS13 antibody. Two hundred and thirty-seven patients with positive lupus anticoagulant and/or anticardiolipin antibody were included. Read More

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[Successful treatment with cyclosporine in a patient with rituximab-refractory thrombocytopenic purpura].

Rinsho Ketsueki 2021 ;62(3):176-179

Department of Hematology and Oncology, Mie University Hospital.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening systemic thrombotic microangiopathy characterized by the presence of anti-ADAMTS13 antibodies (inhibitor). Here we report the case of a patient with refractory aTTP successfully treated with cyclosporine. A 69-year-old man presenting with hematuria and petechiae was referred to our hospital; he was disoriented and febrile. Read More

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Evaluation of Immuno-Rolling Circle Amplification for Multiplex Detection and Profiling of Antigen-Specific Antibody Isotypes.

Anal Chem 2021 04 7;93(15):6169-6177. Epub 2021 Apr 7.

Department of Biosystems, Biosensors Group, KU Leuven, Willem De Croylaan 42, Heverlee B-3001, Belgium.

Antibody characterization is essential for understanding the immune system and development of diagnostics and therapeutics. Current technologies are mainly focusing on the detection of antigen-specific immunoglobulin G (IgG) using bulk singleplex measurements, which lack information on other isotypes and specificity of individual antibodies. Digital immunoassays based on nucleic acid amplification have demonstrated superior performance by allowing the detection of single molecules in a multiplex and sensitive manner. Read More

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A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy.

J Obstet Gynaecol Res 2021 May 9;47(5):1892-1897. Epub 2021 Mar 9.

Department of Obstetrics and Gynecology, Niigata University Medical and Dental Hospital, Niigata City, Japan.

We herein report the case of a 28-year-old pregnant woman with an acute presentation of remarkable petechiae on her lower extremities and severe thrombocytopenia (16 000/mm ) at the 35th week of gestation. Although idiopathic thrombocytopenic purpura was initially suspected, subsequent examinations revealed that her ADAMTS13 (a Disintegrin And Metalloprotease, with ThromboSpondin type 1 repeats, member 13) titer was extremely decreased, while she was negative for antibodies against ADAMTS13. Infusion of fresh frozen plasma was immediately performed, and the platelet count was observed to increase. Read More

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[Diagnosis of thrombotic thrombocytopenic purpura].

Ter Arkh 2020 Dec 15;92(12):207-217. Epub 2020 Dec 15.

National Research Center for Hematology.

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Read More

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December 2020

Thrombotic thrombocytopenic Purpura: A nurse's perspective on a decade of treatment in Sheffield, United Kingdom.

Transfus Apher Sci 2021 Apr 1;60(2):103090. Epub 2021 Mar 1.

Advanced Nurse Practitioner in Haemophilia & Other Haemostasis Disorders, Sheffield Haemophilia & Thrombosis Centre, Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF, United Kingdom. Electronic address:

TTP is a rare, life threatening condition, with an annual incidence of 3-11 cases per million people. A deficiency of a vWF multimer cleaving protein, ADAMTS13 is the cause of the condition. Quick & accurate diagnosis is crucial in the safe & effective treatment of individuals presenting with this condition. Read More

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[Analysis of five children with acquired thrombotic thrombocytopenic purpura].

Zhonghua Er Ke Za Zhi 2021 Mar;59(3):212-217

Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

To investigate the clinical characteristics, treatment and prognosis of children with acquired thrombotic thrombocytopenic purpura (TTP). The clinical manifestations, laboratory examination, treatment and prognosis of 5 children with acquired TTP hospitalized in Beijing Children's Hospital, Capital Medical University from January 2016 to July 2019 were analyzed retrospectively. There were 5 children with acquired TTP including 2 males and 3 females, with the onset age of 8. Read More

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Platelet-activating immune complexes identified in critically ill COVID-19 patients suspected of heparin-induced thrombocytopenia.

J Thromb Haemost 2021 05 14;19(5):1342-1347. Epub 2021 Mar 14.

Department of Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, ON, Canada.

Background: Thrombocytopenia and thrombosis are prominent in coronavirus disease 2019 (COVID-19), particularly among critically ill patients; however, the mechanism is unclear. Such critically ill COVID-19 patients may be suspected of heparin-induced thrombocytopenia (HIT), given similar clinical features.

Objectives: We investigated the presence of platelet-activating anti-platelet-factor 4 (PF4)/heparin antibodies in critically ill COVID-19 patients suspected of HIT. Read More

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Identification of Biomarkers in Patients with Thrombotic Thrombocytopenic Purpura Presenting with Large and Small Ischemic Stroke.

Cerebrovasc Dis Extra 2021 18;11(1):29-36. Epub 2021 Feb 18.

Department of Pathology, The University of Alabama at Birmingham, Birmingham, Alabama, USA.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder resulting in organ damage including ischemic strokes. We sought to characterize the neuroimaging patterns of stroke in a large cohort of patients with immune-mediated TTP (iTTP) and determined their associations with clinical and laboratory parameters and outcomes.

Methods: We analyzed the Alabama TTP Registry who had laboratory confirmation of acute iTTP. Read More

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Counting the cost of caplacizumab.

Blood 2021 02;137(7):871-872

Johns Hopkins University School of Medicine.

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February 2021

Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.

J Clin Apher 2021 Jun 18;36(3):499-504. Epub 2021 Jan 18.

Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.

Management of aTTP in patients who refuse or are intolerant to plasma remains challenging, but new drugs can be implemented with success. A 39-year-old woman presented to the Emergency department for bruises at the upper and lower limbs together with worsening anemia and thrombocytopenia; PLASMIC score was seven, indicative of high risk to have a thrombotic microangiopathy due to severe ADAMTS-13 deficiency: indeed, it was 1.4%. Read More

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[Endothelial dysfunction in thrombotic thrombocytopenic purpura: therapeutic perspectives].

Rev Med Interne 2021 Mar 14;42(3):202-209. Epub 2021 Jan 14.

Centre de Référence des Microangiopathies Thrombotiques (CNR-MAT, www.cnr-mat.fr), Paris, France; Service d'Hématologie, Centre de Référence des Microangiopathies Thrombotiques (CNR-MAT, www.cnr-mat.fr), AP-HP.6, Paris, France. Electronic address:

Immune Thrombotic Thrombocytopenic Purpura (iTTP) is a rare but severe disease with a mortality rate of almost 100 % in the absence of adequate treatment. iTTP is caused by a severe deficiency in ADAMTS13 activity due to the production of inhibitory antibodies. Age has been shown to be a major prognostic factor. Read More

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A Case Report of Congenital Thrombotic Thrombocytopenic Purpura: The Peripheral Blood Smear Lights the Diagnosis.

J Pediatr Hematol Oncol 2020 Dec 9. Epub 2020 Dec 9.

Department of Pediatric Hematology-Oncology, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, Turkey.

We report on a 12-year-old boy with congenital thrombotic thrombocytopenic purpura, on who had an erroneous diagnosis as chronic immune thrombocytopenia. The patient presented with complaints of jaundice and skin rash. Laboratory analysis showed nonimmune hemolytic anemia and severe thrombocytopenia. Read More

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December 2020

Effective and safe off-label use of caplacizumab treatment in a middle-aged obese male.

Transfus Clin Biol 2021 Feb 5;28(1):89-91. Epub 2020 Dec 5.

Thrombosis and Haemostasis Unit, Fondazione IRCCS "Casa Sollievo della Sofferenza", San Giovanni Rotondo (Foggia), Italy; Ob/Gyn Department of the First I.M. Sechenov Moscow State Medical University, Moscow, Russian Federation. Electronic address:

This study shows clinical efficacy and safety profile of an off-label use of caplacizumab for the treatment of immune-mediated thrombotic thrombocytopenic purpura in a middle-aged obese male patient manifesting aphasia, weakness and unconsciousness. Routine blood tests revealed haemolytic anaemia, severe thrombocytopenia (platelet count=20×10/L) and moderate creatinine increase. Diagnosis was based on the clinical judgement and laboratory determinations (undetectable ADAMTS13 activity and presence of anti-ADAMTS13 antibodies). Read More

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February 2021

Should all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab?

J Thromb Haemost 2021 01 17;19(1):58-67. Epub 2020 Dec 17.

Centre National de Référence des MicroAngiopathies Thrombotiques, Paris, France.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disease that causes systemic platelet-rich microthrombi with multiorgan damage. The historical treatment is based on therapeutic plasma exchange (TPE) and immunosuppression. Despite survival rates exceeding 85%, unfavorable outcomes including refractoriness, death, and exacerbations of the disease during treatment still calls for a better management strategy. Read More

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January 2021

Refractory Auto-Immune Thrombotic Thrombocytopenic Pupura Successfully Treated With Caplacizumab.

Front Med (Lausanne) 2020 22;7:549931. Epub 2020 Oct 22.

Service de Néphrologie-Dialyse-Transplantation, Centre Hospitalier Universitaire Angers, Angers, France.

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by mechanical hemolytic anemia, profound thrombocytopenia, and neurological manifestations. Acquired auto-immune TTP, the most prevalent cause of TTP, is induced by the presence of inhibitory anti-ADAMTS13 auto-antibodies. Modern treatment of acquired TTP relies on plasma exchange, rituximab, and steroids. Read More

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October 2020

Immunogenic hotspots in the spacer domain of ADAMTS13 in immune-mediated thrombotic thrombocytopenic purpura.

J Thromb Haemost 2021 02 31;19(2):478-488. Epub 2020 Dec 31.

Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.

Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is caused by anti-ADAMTS13 autoantibodies inducing a severe deficiency of ADAMTS13. Epitope mapping studies on samples obtained during acute iTTP episodes have shown that the iTTP immune response is polyclonal, with almost all patients having autoantibodies targeting the spacer domain of ADAMTS13.

Objectives: To identify the immunogenic hotspots in the spacer domain of ADAMTS13. Read More

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February 2021

[Frontline clinical practice for thrombotic thrombocytopenic purpura].

Rinsho Ketsueki 2020 ;61(9):1331-1337

Division of Hematology, Department of General Internal Medicine, Saitama Medical University Hospital.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by ADAMTS13 deficiency. Although the name of TTP is well known, most hematologists find its diagnosis and treatment difficult because it is ultrarare. TTP is an acute-onset and fatal disorder. Read More

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January 2021

A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP.

Blood 2021 02;137(6):733-742

Centre de Référence des Microangiopathies Thrombotiques, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

The anti-von Willebrand factor nanobody caplacizumab was licensed for adults with immune-mediated thrombotic thrombocytopenic purpura (iTTP) based on prospective controlled trials. However, few data are available on postmarketing surveillance. We treated 90 iTTP patients with a compassionate frontline triplet regimen associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab. Read More

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February 2021

Modifying ADAMTS13 to modulate binding of pathogenic autoantibodies of patients with acquired thrombotic thrombocytopenic purpura.

Haematologica 2020 11 1;105(11):2619-2630. Epub 2020 Nov 1.

Department of Molecular and Cellular Hemostasis, Sanquin-Academic Medical Center, The Netherlands.

Antibodies that develop in patients with immune thrombotic thrombocytopenic purpura (iTTP) commonly target the spacer epitope R568/F592/R660/Y661/Y665 (RFRYY). In this study we present a detailed contribution of each residue in this epitope for autoantibody binding. Different panels of mutations were introduced here to create a large collection of full-length ADAMTS13 variants comprising conservative (Y←→F), semi-conservative (Y/F→L), non-conservative (Y/F→N) or alanine (Y/F/R→A) substitutions. Read More

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November 2020

Transforming the major autoantibody site on ADAMTS13: spacer domain variants retaining von Willebrand factor cleavage activity.

Authors:
Marie Scully

Haematologica 2020 11 1;105(11):2510-2512. Epub 2020 Nov 1.

Department of Haematology and National Institute for Health Research Cardiometabolic Programme, UCLH/UCL BRC, London, UK.

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November 2020

Inhibitory anti ADAMTS13 antibodies with a new rapid fully automated CLiA assay.

Int J Lab Hematol 2021 Apr 23;43(2):298-304. Epub 2020 Oct 23.

S.C. Laboratory Analysis, AO Ordine Mauriziano, Torino, Italy.

Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by severe ADAMTS13 deficiency. The acquired form is associated with autoantibodies directed against ADAMTS13. Both noninhibitory and inhibitory autoantibodies can be detected by ELISA assay, while only inhibitory autoantibodies are detected by Bethesda assay. Read More

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Humanized anti CD-20 as an alternative in chronic management of relapsing thrombotic thrombocytopenic microangiopathy resistant to rituximab due to anti chimeric antibody.

Int J Hematol 2021 Mar 16;113(3):456-460. Epub 2020 Oct 16.

Department of Nephrology, Dialysis and Apheresis, University Hospital Caremeau, Nimes, France.

Acquired Immune thrombotic thrombocytopenic purpura (iTTP) is considered among clinical situations that needs not only urgent treatment in acute setting but also long term management to prevent relapses. Important progresses have been made in management of these patients that are definitely associated with reduced mortality and relapse rate. However, there are still noticeable percentage of patients that may relapse despite application of modern treatment strategies including preemptive rituximab infusions. Read More

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