6,667 results match your criteria Thrombotic Thrombocytopenic Purpura


Aspirin Prophylaxis for Hereditary and Acquired Thrombotic Thrombocytopenic Purpura?

Am J Hematol 2022 May 20. Epub 2022 May 20.

Cardiovascular Biology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK.

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Naturally Occurring Anti-Idiotypic Antibodies Portray a Largely Private Repertoire in Immune-Mediated Thrombotic Thrombocytopenic Purpura.

J Immunol 2022 May 18. Epub 2022 May 18.

Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland; and Department for BioMedical Research, University of Bern, Bern, Switzerland

Rare immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening disease resulting from a severe autoantibody-mediated ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motifs, member 13) deficiency. Acute iTTP episodes are medical emergencies, but when treated appropriately >95% of patients survive. However, at least half of survivors will eventually experience a relapse. Read More

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Thrombocytopenia: the good, the bad and the ugly.

Clin Med (Lond) 2022 May;22(3):214-217

University Hospitals Sussex NHS Foundation Trust, Brighton, UK and Brighton and Sussex Medical School, Falmer, UK.

New thrombocytopenia may be associated with a variety of conditions and diagnosis can be challenging. Presentation can vary from life-threatening bleeding or thrombosis to an incidental finding in an asymptomatic patient. New thrombocytopenia requires urgent investigation. Read More

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Cardiovascular and Hematologic Complications of COVID-19 Vaccines.

Cardiol Rev 2022 May 16. Epub 2022 May 16.

Departments of Medicine and Cardiology, New York Medical College/Westchester Medical Center, Valhalla, NY.

COVID-19 is a pro-thrombotic and cardiac-damaging disease. There are four vaccines against COVID-19 currently approved in North America, including the mRNA vaccines by Pfizer and Moderna, and the adenovirus vector vaccines by Johnson and Johnson and AstraZeneca. These vaccines have been proven effective in preventing morbidity and mortality in patients who were exposed to COVID-19 infection, but the vaccines have also been associated with complications. Read More

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Paediatric Thrombosis: A Five-Year Experience From A Tertiary Care Center Of Pakistan.

J Ayub Med Coll Abbottabad 2022 Apr-Jun;34(2):263-268

CMH Medical College and Institute of Dentistry, Karachi, Pakistan.

Background: Advances in imaging techniques and longer survival of chronic medical conditions contribute to the increase in paediatric thrombosis. We aim to determine the incidence, underlying risk factors, management and clinical outcome of paediatric thrombosis at a multidisciplinary facility of Pakistan.

Methods: A retrospectively analysis of the medical records of patients in the paediatric age group admitted at the Aga Khan University hospital from January 2013-September 2018 was performed. Read More

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Gamma-Delta Large Granular Lymphocytic Leukemia: A Diagnostic Dilemma.

Cureus 2022 Apr 13;14(4):e24124. Epub 2022 Apr 13.

Department of Oncology, University of Florida College of Medicine - Jacksonville, Jacksonville, USA.

We report an initial diagnostic dilemma case of a 60-year-old male who presented with worsening hemolytic anemia, thrombocytopenia, and acute kidney injury requiring hemodialysis. His presentation was initially suspected to be secondary to thrombotic thrombocytopenic purpura (TTP) and he was treated with intravenous immunoglobulin (IVIG) and plasmapheresis. Despite treatment, he failed to improve during his admission leading to further workup revealing gamma-delta T-cell large granular lymphocytic (γδ T-LGL) leukemia. Read More

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Camelid Single-Domain Antibodies: Promises and Challenges as Lifesaving Treatments.

Int J Mol Sci 2022 Apr 30;23(9). Epub 2022 Apr 30.

Human Health Therapeutics Research Centre, National Research Council Canada, 100 Sussex Drive, Ottawa, ON K1A 0R6, Canada.

Since the discovery of camelid heavy-chain antibodies in 1993, there has been tremendous excitement for these antibody domains (VHHs/sdAbs/nanobodies) as research tools, diagnostics, and therapeutics. Commercially, several patents were granted to pioneering research groups in Belgium and the Netherlands between 1996-2001. Ablynx was established in 2001 with the aim of exploring the therapeutic applications and development of nanobody drugs. Read More

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Case Report: Rabies Vaccine-Induced Thrombotic Thrombocytopenic Purpura in a Patient With Systemic Lupus Erythematosus.

Front Immunol 2022 25;13:851316. Epub 2022 Apr 25.

Department of Rheumatology and Immunology, The Sixth Affiliated Hospital of Guangzhou Medical University, Qingyuan People's Hospital, Guangdong, China.

For patients with autoimmune diseases, vaccination is controversial. The use of vaccination in patients with autoimmune diseases is controversial. There are many reports of secondary thrombotic thrombocytopenic purpura cases after various vaccinations. Read More

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Complement Mediated Endothelial Damage in Thrombotic Microangiopathies.

Front Med (Lausanne) 2022 25;9:811504. Epub 2022 Apr 25.

Hematopathology Unit, Department of Pathology, Hospital Clínic of Barcelona, Biomedical Diagnosis Centre (CDB), Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.

Thrombotic microangiopathies (TMA) constitute a group of different disorders that have a common underlying mechanism: the endothelial damage. These disorders may exhibit different mechanisms of endothelial injury depending on the pathological trigger. However, over the last decades, the potential role of the complement system (CS) has gained prominence in their pathogenesis. Read More

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Thrombotic thrombocytopenic purpura (TTP) after COVID-19 vaccination: A systematic review of reported cases.

Thromb Res 2022 May 2;214:115-121. Epub 2022 May 2.

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, United States. Electronic address:

Introduction: With the advent of COVID-19 vaccines, hospitalization rates and progression to severe COVID-19 disease have reduced drastically. Most of the adverse events reported by the vaccine recipients were minor. However, autoimmune hematological complications such as vaccine-induced immune thrombotic thrombocytopenia (VITT), immune thrombocytopenic purpura (ITP) and TTP have also been reported post-COVID-19 vaccination. Read More

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Diagnostic dilemma in a patient presenting with thrombotic microangiopathy in the setting of pregnancy.

Arch Clin Cases 2022 6;9(1):24-28. Epub 2022 Apr 6.

CMH Lahore Medical College, Lahore, Punjab, Pakistan.

We report a case of thrombotic microangiopathy in a postpartum female for which considerable diagnostic uncertainty existed initially regarding the etiology. This case highlights the limitations surrounding PLASMIC scoring criteria for the diagnosis of thrombotic thrombocytopenic purpura (TTP). A 32-year-old woman presented to maternofetal medicine in her third trimester of pregnancy at 32 weeks for a routine follow up and was subsequently found to have elevated blood pressures with proteinuria, and was diagnosed with pre-eclampsia. Read More

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Response to belimumab in thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a case-based review.

Clin Rheumatol 2022 May 7. Epub 2022 May 7.

Department of Rheumatology and Immunology, Daping Hospital, Army Medical University (Third Military Medical University), Chongqing, 400042, China.

Thrombotic thrombocytopenic purpura (TTP), a life-threatening syndrome characterized by acute microangiopathic hemolytic anemia, thrombocytopenia, and visceral ischemia, can be classified as congenital TTP (inherited due to a mutation in ADAMTS13) and acquired TTP. The acquired TTP is further classified as idiopathic and secondary TTP. Systemic lupus erythematosus (SLE) is regarded as one of the most common causes of secondary TTP (SLE-TTP). Read More

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Case Report: Thrombotic-Thrombocytopenic Purpura Following Ipilimumab and Nivolumab Combination Immunotherapy for Metastatic Melanoma.

Front Immunol 2022 20;13:871217. Epub 2022 Apr 20.

Department of Medical Oncology, University Hospital Waterford, Waterford, Ireland.

A man in his early 50s presented with small bowel obstruction, requiring emergency laparoscopic small bowel resection for the metastatic melanoma of the jejunum with no identifiable primary lesion. One week after his first treatment with ipilimumab and nivolumab, he presented with diffuse abdominal pain, constipation, and fatigue. A computerized tomography scan did not identify a cause for his symptoms. Read More

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Pathogenesis of vaccine-induced immune thrombotic thrombocytopenia (VITT).

Semin Hematol 2022 Apr 23;59(2):97-107. Epub 2022 Feb 23.

Institute for Anatomy and Cell Biology, University Medicine Greifswald, Greifswald, Germany.

Vaccine-induced immune thrombotic thrombocytopenia (VITT; synonym, thrombosis with thrombocytopenia syndrome, is associated with high-titer immunoglobulin G antibodies directed against platelet factor 4 (PF4). These antibodies activate platelets via platelet FcγIIa receptors, with platelet activation greatly enhanced by PF4. Here we summarize the current concepts in the pathogenesis of VITT. Read More

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Treatment of vaccine-induced immune thrombotic thrombocytopenia (VITT).

Semin Hematol 2022 Apr 7;59(2):89-96. Epub 2022 Mar 7.

Department of Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada; McMaster Center for Transfusion Research, McMaster University, Hamilton, Ontario, Canada; Service of Benign Hematology, Department of Medicine, Hamilton Health Sciences, Hamilton, Ontario Canada; Department of Pathology and Molecular Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada; Transfusion Medicine, Hamilton Regional Laboratory Medicine Program, Hamilton, Ontario, Canada. Electronic address:

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a novel prothrombotic disorder characterized by thrombosis, thrombocytopenia, and disseminated intravascular coagulation identified in hundreds of recipients of ChAdOx1 nCoV-19 (Oxford/AstraZeneca), an adenovirus vector coronavirus disease 2019 (COVID-19) vaccine. VITT resembles heparin-induced thrombocytopenia (HIT) in that patients have platelet-activating anti-platelet factor 4 antibodies; however, whereas heparin typically enhances platelet activation by HIT antibodies, VITT antibody-induced platelet activation is often inhibited in vitro by pharmacological concentrations of heparin. Further, the thrombotic complications in VITT feature much higher frequencies of atypical thrombosis, most notably cerebral vein thrombosis and splanchnic vein thrombosis, compared with HIT. Read More

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Laboratory testing for VITT antibodies.

Semin Hematol 2022 Apr 7;59(2):80-88. Epub 2022 Mar 7.

Institute for Transfusion Medicine, University Medicine Greifswald, Greifswald, Germany.

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a highly prothrombotic disorder that like heparin-induced thrombocytopenia (HIT) is caused by platelet-activating antibodies that recognize platelet factor 4 (PF4). However, unlike HIT-where heparin at low concentrations (0.1-0. Read More

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Clinical picture of VITT.

Semin Hematol 2022 Apr 8;59(2):76-79. Epub 2022 Feb 8.

Department Hematology, Oxford University Hospitals NHS Foundation Trust, Oxfordshire. Electronic address:

This chapter explores the clinical features of vaccine-induced immune thrombotic thrombocytopenia, also called vaccine-induced immune thrombocytopenia and thrombosis (VITT). Whilst the etiology is distinct from other causes of thrombotic thrombocytopenia syndrome (TTS), presentation may be similar and hence the need for strict diagnostic criteria to ensure accurate and prompt diagnosis and early treatment. Studies have identified prognostic markers of the disease, directing therapy and management pathways, and mortality and morbidity from this rare but life-threatening and potentially disabling consequence of the ChAdOx1 nCov-19 vaccine has declined. Read More

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Epidemiology of VITT.

Authors:
Menaka Pai

Semin Hematol 2022 Apr 8;59(2):72-75. Epub 2022 Feb 8.

McMaster University, Hamilton Health Sciences, Hamilton Regional Laboratory Medicine Program, Hamilton, Ontario, Canada. Electronic address:

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a life-threatening syndrome of aggressive thrombosis, often profound thrombocytopenia, and frequently overt disseminated intravascular coagulation. It has been associated with 2 adenovirus vector COVID-19 vaccines: ChAdOx1 nCoV-19 (AstraZeneca) and Ad26.COV2. Read More

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Platelet-activating anti-PF4 disorders: An overview.

Semin Hematol 2022 Apr 20;59(2):59-71. Epub 2022 Feb 20.

Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada; Department of Medicine, McMaster University, Hamilton, ON, Canada; Transfusion Medicine, Hamilton Regional Laboratory Medicine Program, Hamilton, ON, Canada; Service of Benign Hematology, Hamilton Health Sciences, Hamilton General Hospital, Hamilton, ON, Canada; McMaster Center for Transfusion Research, McMaster University, Hamilton, ON, Canada. Electronic address:

Platelet factor 4 (PF4) is a highly cationic tetrameric protein that can be targeted by platelet-activating anti-PF4 antibodies of immunoglobulin G (IgG) class. Certain features of PF4, including its multivalent nature (duplicate antigen sites per tetramer), the ability of many PF4 tetramers to undergo close approximation through charge neutralization, and the dimeric binding of IgG molecules, results in formation of IgG-containing immune complexes in situ on platelets, neutrophils, and monocytes, resulting in Fcγ receptor-mediated pancellular activation that also activates hemostasis (potential for disseminated intravascular coagulation). This review discusses 4 anti-PF4 disorders: classic heparin-induced thrombocytopenia ([HIT]; triggered by heparin and certain other polyanionic pharmaceuticals, featuring predominantly heparin-dependent antibodies), autoimmune HIT (aHIT; severe subtype of HIT that features both heparin-dependent and heparin-independent platelet-activating antibodies), and spontaneous HIT (non-heparin triggers such as knee replacement surgery and infection; predominantly heparin-independent platelet-activating antibodies). Read More

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Vaccine-induced immune thrombotic thrombocytopenia (VITT).

Semin Hematol 2022 Apr 8;59(2):57-58. Epub 2022 Mar 8.

Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada; Department of Medicine, McMaster University, Hamilton, Ontario, Canada; Transfusion Medicine, Hamilton Regional Laboratory Medicine Program, Hamilton, Ontario, Canada; Service of Benign Hematology, Hamilton Health Sciences, Hamilton General Hospital, Hamilton, Ontario, Canada; McMaster Center for Transfusion Research, McMaster University, Hamilton, Ontario, Canada; McMaster Centre for Transfusion Research, McMaster University, Hamilton, Ontario. Electronic address:

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The psychiatric and psychological sequelae of vaccine-induced immune thrombotic thrombocytopenia (VITT).

Semin Hematol 2022 Apr 18;59(2):115-119. Epub 2022 Feb 18.

Department Hematology, Oxford University Hospitals NHS Foundation Trust, UK.

This review paper explores the potential psychiatric and psychological sequelae of vaccine-induced immune thrombotic thrombocytopenia, also called vaccine-induced immune thrombocytopenia, and thrombosis (VITT). In the absence of any literature to date we have extrapolated data from similar conditions, particularly data pertaining to the critical care population. We discuss both the direct and indirect effects of thrombosis, likely psychiatric and psychological challenges during recovery, and ethical issues around vaccination. Read More

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Longitudinal Aspects of VITT.

Semin Hematol 2022 Apr 7;59(2):108-114. Epub 2022 Mar 7.

Department of Transfusion Medicine, Institute of Immunology and Transfusion Medicine, Universitätsmedizin Greifswald, Greifswald, Germany. Electronic address:

In hundreds of patients worldwide, vaccination against COVID-19 with adenovirus vector vaccines (ChAdOx1 nCoV-19; Ad26.COV2.S) triggered platelet-activating anti-platelet factor 4 (PF4) antibodies inducing vaccine-induced immune thrombotic thrombocytopenia (VITT). Read More

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Relapse of immune-mediated thrombotic thrombocytopenic purpura following mRNA COVID-19 vaccination: a prospective cohort study.

Haematologica 2022 May 5. Epub 2022 May 5.

Division of Hematology, AOU "Policlinico G. Rodolico-San Marco", Catania, Italy; Postgraduate School of Hematology, University of Catania, Catania.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening disease. Vaccination has been reported to be a trigger of onset and relapse of autoimmune diseases. We evaluated after mRNA COVID-19 vaccination 32 adult patients previously diagnosed with iTTP by means of weekly monitoring of complete blood count and ADAMTS13 testing. Read More

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Refractory immune TTP following Pfizer-BioNTech COVID-19 vaccine successfully salvaged with caplacizumab.

J Thromb Haemost 2022 May 5. Epub 2022 May 5.

Charles-Lemoyne Hospital, CISSS of Montérégie-Centre, University of Sherbrooke, Greenfield Park, Quebec, Canada.

Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy caused by antibodies against ADAMTS13. We report a young, healthy female who developed hematuria, vomiting, and hematemesis 3 weeks after her first dose of Pfizer Bio-NTech COVID-19 vaccine. Investigations confirmed iTTP with undetectable ADAMTS13 activity and a positive antibody assay. Read More

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A Case of Thrombotic Microangiopathy Secondary to Hypertensive Emergency.

Cureus 2022 Apr 18;14(4):e24237. Epub 2022 Apr 18.

Internal Medicine, Baton Rouge General Medical Center, Baton Rouge, USA.

Hypertension causing thrombotic microangiopathy (TMA) is one of the several etiologies of TMA, which causes endothelial damage and thrombosis of microvasculature, leading to hemolytic anemia, thrombocytopenia, and ischemic organ damage. Renal microvasculature involvement leading to renal dysfunction is most frequently seen in TMA but the degree of dysfunction varies with etiology. If left untreated, TMA carries a risk of high mortality, so it is extremely important for early identification of the cause of TMA. Read More

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Alterations in B- and circulating T-follicular helper cell subsets in immune thrombotic thrombocytopenic purpura.

Blood Adv 2022 May 4. Epub 2022 May 4.

5National Institute for Health Research Cardiometabolic Programme, UCLH/UCL Cardiovascular BRC, London, UK, United Kingdom.

T follicular helper (Tfh) cells regulate development of antigen-specific B-cell immunity. We prospectively investigated B-cell and cTfh subsets in 45 immune TTP patients at presentation and longitudinally after rituximab (RTX). B-cell phenotype was altered at acute iTTP presentation with decreased transitional cells and postgerminal centre (post-GC) memory B cells and increased plasmablasts compared to healthy controls. Read More

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Evaluation of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies: Lessons learned from a 14-year retrospective study.

Ther Apher Dial 2022 May 2. Epub 2022 May 2.

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a clinical thrombotic microangiopathy (TMA) syndrome defined by the pentad of symptoms. Therapeutic plasma exchange with plasma replacement is an ASFA Category I modality that can reduce morbidity and mortality if initiated early. We describe a 14-year review of patients referred for plasma exchange with a suspected diagnosis of TTP. Read More

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Mitomycin-Induced Thrombotic Thrombocytopenic Purpura Treated Successfully With Plasmapheresis and Steroid: A Case Report.

Cureus 2022 Mar 26;14(3):e23525. Epub 2022 Mar 26.

Pulmonary and Critical Care, Icahn School of Medicine at Mount Sinai/BronxCare Health System, New York City, USA.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by severely reduced ADAMTS13 or the von Willebrand factor-cleaving protease (VWFCP) enzyme resulting in low platelet and red blood cell counts along with severe renal, cardiac, and neurological dysfunction. Plasmapheresis is the treatment of choice. Mitomycin, a widely used chemotherapeutic agent for gastrointestinal (GI) cancers anal and breast cancers, has been reported to occasionally cause severe TTP and hemolytic uremic syndrome (HUS) cases. Read More

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[Immune-mediated thrombotic thrombocytopenic purpura and susceptible HLA alleles].

Authors:
Kazuya Sakai

Rinsho Ketsueki 2022 ;63(4):277-285

Department of Blood Transfusion Medicine, Nara Medical University.

Thrombotic thrombocytopenic purpura (TTP) is an extremely rare and fatal thrombotic disorder characterized by impaired enzyme activity of von Willebrand factor cleaving protease, also known as ADAMTS13. Immune-mediated TTP (iTTP) is an acquired form of TTP caused by the production of auto-antibodies against ADAMTS13. The pathophysiology of autoimmune disorders is multifactorial, with several human leukocyte antigen (HLA) alleles identified as a genetic risk factor for autoimmune diseases known as susceptible HLA. Read More

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