6,272 results match your criteria Thrombotic Thrombocytopenic Purpura

Plasma levels of bigEndothelin-1 are associated with renal insufficiency and in-hospital mortality of immune thrombotic thrombocytopenic purpura.

Thromb Haemost 2021 May 13. Epub 2021 May 13.

Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, United States.

Immune thrombotic thrombocytopenic purpura (iTTP) is caused by severe deficiency of plasma ADAMTS13 activity. Despite advances in early diagnosis and management, the mortality rate of acute iTTP remains high in a large part of world where access to some of the most novel therapies is limited. To determine the role of plasma bigEndothelin-1 (bigET-1) or its bioactive product ET-1 as a biomarker and/or a pathogenic factor in acute iTTP, plasma levels of bigET-1 were determined using an immunoassay in patients with iTTP on admission and during remission, as well as in healthy controls; moreover, the biological effect of ET-1 in thrombus formation was determined by a microfluidic assay. Read More

View Article and Full-Text PDF

Atypical posterior reversible encephalopathy syndrome in a case of thrombotic thrombocytopenic purpura with Sjogren's syndrome.

Radiol Case Rep 2021 Jun 19;16(6):1552-1556. Epub 2021 Apr 19.

Department of Radiology, Yale New Haven Health System, Bridgeport Hospital, Bridgeport, CT.

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder characterized by thrombotic microangiopathy. Neurologic symptoms are frequently seen in its presentation and the most common finding on neuroimaging of TTP is posterior reversible encephalopathy syndrome (PRES). Acute strokes, hemorrhages and atypical PRES are uncommonly seen. Read More

View Article and Full-Text PDF

Thrombotic Thrombocytopenic Purpura after Ad26.COV2-S Vaccination.

Am J Emerg Med 2021 May 4. Epub 2021 May 4.

Cleveland Clinic Akron General, Department of Emergency Medicine, Akron, OH, USA; Northeast Ohio Medical University, Rootstown, OH, USA. Electronic address:

The U.S. Food and Drug Administration (FDA) recently issued an Emergency Use Authorization (EUA) for two highly effective Sars-CoV-2 (COVID-19) vaccines from Pfizer-BioNTech and Moderna. Read More

View Article and Full-Text PDF

Fatal cerebral hemorrhage in a patient with thrombotic thrombocytopenic purpura with a normal platelet count during treatment with caplacizumab.

Platelets 2021 May 12:1-2. Epub 2021 May 12.

Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a thrombotic microangiopathy with a severe mortality and morbidity. Caplacizumab has recently been approved in the Netherlands as a new therapeutic option in patients with life-threatening organ failure due to aTTP. We describe the case of a 50 year old patient with aTTP who was referred to our hospital for treatment with caplacizumab. Read More

View Article and Full-Text PDF

Clinical characteristics and outcomes of adult patients with acquired thrombotic thrombocytopenic purpura: a single center retrospective study.

Ann Palliat Med 2021 May 11. Epub 2021 May 11.

Department of Intensive Care Medicine, The First Affiliated Hospital of Soochow University, Suzhou, China.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare disease and a potentially lifethreatening thrombotic microangiopathy. Although the diagnostic and therapeutic techniques have improved, it is still difficult for clinicians to identify early due to different initial clinical manifestations and the incidence and survival rate are reported inconsistently. This study investigated the clinical characteristics, treatment strategies, and treatment outcomes of adult patients with acquired TTP. Read More

View Article and Full-Text PDF

Humoral Acute Rejection in a Kidney Transplant Recipient with Idiopathic Thrombocytopenic Purpura.

Case Rep Transplant 2021 22;2021:9933354. Epub 2021 Apr 22.

Departamento de Nefrología y Trasplantes, UMAE, Hospital de Especialidades, CMNO, IMSS, Guadalajara, Jalisco, Mexico.

A 47-year-old male was diagnosed with chronic kidney disease (CKD) in 2011; idiopathic thrombocytopenic purpura (ITP) was also diagnosed in 2011 refractory to medical treatment and finally treated with splenectomy (2017) without relapses since that date, 5 blood transfusions, and 4 platelet apheresis in 2017. Renal transplant from a living related donor (brother), ABO compatible, crossmatch were negative, sharing 1 haplotype. Donor-specific anti-HLA antibody was negative. Read More

View Article and Full-Text PDF

Atypical Evolution of Secondary Hemolytic Uremic Syndrome Defined as Paraneoplastic Syndrome under Eculizumab and Palbociclib Therapies.

Case Rep Oncol 2021 Jan-Apr;14(1):676-680. Epub 2021 Apr 12.

Nephrology, Hemodialysis, Apheresis and Kidney Transplantation Department, Grenoble Alpes University Hospital, Grenoble, France.

Thrombotic microangiopathy (TMA) is most of the time caused by thrombotic thrombocytopenic purpura or hemolytic uremic syndrome. A 60-year-old female was diagnosed in 2014 with mammary breast adenocarcinoma treated by several-line therapy: mastectomy, docetaxel, cyclophosphamide, radiotherapy, doxorubicine, and capecitabine. By mid-November, the patient was admitted to the hospital with regenerative, mechanical, and hemolytic anemia, schistocytes at 3%, and thrombopenia (99 G/L), associated with high blood transfusion requirement. Read More

View Article and Full-Text PDF

Intracranial haemorrhage in immune thrombotic thrombocytopenic purpura treated with caplacizumab.

J Thromb Haemost 2021 May 11. Epub 2021 May 11.

Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening condition resulting from IgG autoantibodies to 'a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13' (ADAMTS13). Severe ADAMTS13 deficiency causes accumulation of ultra-large von Willebrand factor (VWF) multimers which bind platelets, leading to widespread microvascular thrombosis, compromise of critical organs and profound thrombocytopenia . In the absence of treatment, TTP is usually fatal. Read More

View Article and Full-Text PDF

Postpartum Thrombocytopenia Mimicking Thrombotic Thrombocytopenic Purpura May Prevent Appropriate Selection of Therapeutic Options.

Asian J Anesthesiol 2021 Apr 30:1-2. Epub 2021 Apr 30.

Department of Anesthesiology and Division of Intensive Care, Nara Medical University, Kashihara, Nara, Japan.

View Article and Full-Text PDF

A Novel Case of Cytomegalovirus Pneumonia in an Acquired Thrombotic Thrombocytopenic Purpura Patient Treated With Rituximab.

Cureus 2021 Mar 30;13(3):e14182. Epub 2021 Mar 30.

Internal Medicine and Nephrology, McLaren Health Care, Flint/MSU, Flint, USA.

Thrombotic thrombocytopenic purpura (TTP) is thrombotic microangiopathy that is universally fatal if not promptly recognized and treated. Standard treatment includes plasma exchange (PLEX) therapy and immunosuppression. We present a case of an 80 years old African American male with a past medical history significant for essential hypertension, chronic obstructive pulmonary disease, and a recent TTP diagnosis for which he was treated with PLEX, glucocorticoids, and rituximab. Read More

View Article and Full-Text PDF

Successful Treatment of NDM Sepsis and Intestinal Decolonization with Ceftazidime/Avibactam Plus Aztreonam Combination in a Patient with TTP Complicated by SARSCoV-2 Nosocomial Infection.

Medicina (Kaunas) 2021 Apr 28;57(5). Epub 2021 Apr 28.

Department of Anesthesia and Intensive Care Unit, Università degli Studi di Foggia, Azienda Ospedaliero Universitaria Ospedali Riuniti di Foggia, 1, 71122 Viale Pinto, FG, Italy.

Carbapenem-resistant (CRE) are a serious public health threat. Infections due to these organisms are associated with significant morbidity and mortality. Among them, metallo-β-lactamases (MBLs)-producing are of global concern today. Read More

View Article and Full-Text PDF

A Case of Thrombotic Thrombocytopenic Purpura without Pathognomonic Schistocytes.

Kevin Yu Min Yan

Clin Pract 2021 Apr 13;11(2):223-227. Epub 2021 Apr 13.

Department of Hematology and Oncology, Alta Bates Summit Medical Center, Summit Campus, Oakland, CA 94609, USA.

Patients diagnosed with thrombotic thrombocytopenic purpura (TTP) typically present with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia; these two clinical manifestations were often believed to be essential indicators of TTP. However, such indicators are not always present in every case. Here, we present a patient affected by TTP but showing no distinctive schistocytes on blood smear review. Read More

View Article and Full-Text PDF

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

View Article and Full-Text PDF

Burden of illness among Medicare and Non-Medicare US populations with acquired thrombotic thrombocytopenic purpura.

J Med Econ 2021 Apr 27. Epub 2021 Apr 27.

National Marrow Donor Program, Seattle, WA, USA.

Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare hematologic disorder that can lead to serious life-threatening medical complications.

Objective: The aim of this study was to describe aTTP-related hospital resource utilization, cost, complications, and overall survival among US Medicare and non-Medicare populations following aTTP episodes prior to the US approval of caplacizumab.

Methods: This retrospective study utilized administrative claims data for Medicare Fee-for-Service (FFS) beneficiaries (100% sample) and a sample of commercial, managed Medicaid [MM], Medicare Advantage [MA] plan members from the Inovalon MORE Registry. Read More

View Article and Full-Text PDF

COVID 19 infection associated with thrombotic thrombocytopenic purpura.

J Thromb Thrombolysis 2021 Apr 27. Epub 2021 Apr 27.

Medical Oncology Hematology, AIIMS Rishikesh, Rishikesh, India.

Thrombotic thrombocytopenic purpura (TTP) which can cause significant mortality is a thrombotic microangiopathy due to deficiency of VWF cleaving protease ADAMTS13 and as per medical literature there are examples that TTP can be caused by COVID 19 infection. A 35 years old female after admission with right sided weakness and slurring of speech was found to be COVID positive and diagnosed as a case of TTP. Patient had absent ADAMTS13 level on day 1. Read More

View Article and Full-Text PDF

Fatal Thrombotic Microangiopathy and Posterior Reversible Encephalopathy Syndrome in a Patient with anti-Melanoma Differentiation-associated Gene 5 Antibody-positive Dermatomyositis.

Intern Med 2021 Apr 26. Epub 2021 Apr 26.

Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Japan.

A 56-year-old woman presented with dermatomyositis positive for anti-melanoma differentiation-associated gene 5 antibody. No interstitial lung disease was detected. Despite treatment with methylprednisolone pulse therapy and cyclosporine, dysphagia developed. Read More

View Article and Full-Text PDF

An Early Unexpected Immune Thrombotic Thrombocytopenic Purpura Relapse Associated with SARS-CoV-2 Infection: A Case Report and Literature Review.

Acta Haematol 2021 Apr 23:1-5. Epub 2021 Apr 23.

Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. Read More

View Article and Full-Text PDF

Accurate accounting of caplacizumab cost effectiveness - Authors' reply.

Lancet Haematol 2021 05;8(5):e316

Centre for Health Technology Evaluation, National Institute for Health and Care Excellence, Manchester M1 4BT, UK; Oxford Centre for Diabetes Endocrinology and Metabolism, Oxford, UK. Electronic address:

View Article and Full-Text PDF

Accurate accounting of caplacizumab cost effectiveness.

Lancet Haematol 2021 05;8(5):e315

Division of Hematology and Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA; Division of Hemostasis and Thrombosis, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA; Harvard Medical School, Boston, MA, USA. Electronic address:

View Article and Full-Text PDF

Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.

Semin Thromb Hemost 2021 Apr 23. Epub 2021 Apr 23.

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

von Willebrand factor (VWF) is a large adhesive multimeric protein involved in hemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect of VWF can lead to von Willebrand disease (VWD) and cause bleeding. Read More

View Article and Full-Text PDF

Eltrombopag-related renal vein thromboembolism in a patient with immune thrombocytopenia: A case report.

World J Clin Cases 2021 Apr;9(11):2611-2618

Department of Pharmacy, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China.

Background: Eltrombopag is an orally administered thrombopoietin receptor agonist linked to a heightened risk of treatment-related thromboembolism. Both venous and arterial thromboses have been documented in the medical literature.

Case Summary: In the absence of nephropathy, a 48-year-old patient receiving eltrombopag for immune thrombocytopenia (ITP) developed renal vein thrombosis and pulmonary embolism. Read More

View Article and Full-Text PDF

Post-partum occurrence of Wunderlich syndrome and microangiopathic haemolytic anaemia (MAHA): a case report.

J Community Hosp Intern Med Perspect 2021 Mar 23;11(2):277-279. Epub 2021 Mar 23.

Department of Internal Medicine, Albert Einstein Medical Center, Philadelphia, PA, USA.

This is a case of a 27-year-old primigravida with monochorionic diamniotic twin gestation who was admitted to the hospital for induction of labour. Her postpartum course was complicated by microangiopathic haemolytic anemia (MAHA). The etiology for the MAHA was initially thought to be secondary to pre-eclampsia and vitamin B12/folate deficiency. Read More

View Article and Full-Text PDF

Relapse of thrombotic thrombocytopenic purpura after COVID-19 vaccine.

Transfus Apher Sci 2021 Apr 16:103145. Epub 2021 Apr 16.

Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy. Electronic address:

View Article and Full-Text PDF

The effects of streptokinase in a Chacma baboon (Papio ursinus) model of acquired thrombotic thrombocytopenic purpura.

Clin Exp Med 2021 Apr 22. Epub 2021 Apr 22.

Human Molecular Biology Unit, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa.

TTP is a life-threatening disorder with limited pharmaceutical treatment options. Recently, the potential of streptokinase in the treatment of acquired TTP was demonstrated in humans in vitro, and in vivo in a mouse model. We aimed to determine the in vitro and in vivo effects of streptokinase in an established Papio ursinus model of acquired TTP. Read More

View Article and Full-Text PDF

Continuous plasma exchange with dialysis for thrombotic microangiopathy in intensive care unit: Retrospective observational study.

Ther Apher Dial 2021 Apr 22. Epub 2021 Apr 22.

Department of Emergency and Critical Care Medicine, Akita University Graduate School of Medicine, Akita, Japan.

Continuous plasma exchange with dialysis is a novel method of blood purification and is a modification of the plasma exchange. Technically, this process suggests a reduction in adverse events, cost-effectiveness, and compatibility with hemodynamic instability. The therapeutic effect of plasma exchange on thrombotic microangiopathies has been established. Read More

View Article and Full-Text PDF

Caplacizumab prevents refractoriness and mortality in acquired thrombotic thrombocytopenic purpura: integrated analysis.

Blood Adv 2021 Apr;5(8):2137-2141

Medical Affairs, Sanofi, Diegem, Belgium.

The efficacy and safety of caplacizumab in individuals with acquired thrombotic thrombocytopenic purpura (aTTP) have been established in the phase 2 TITAN and phase 3 HERCULES trials. Integrated analysis of data from both trials was conducted to increase statistical power for assessing treatment differences in efficacy and safety outcomes. Caplacizumab was associated with a significant reduction in the number of deaths (0 vs 4; P < . Read More

View Article and Full-Text PDF

Educational Case: Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies and Potential Role of the Spleen.

Acad Pathol 2021 Jan-Dec;8:23742895211001312. Epub 2021 Mar 30.

Robert J. Tomsich Department of Pathology and Laboratory Medicine, Cleveland Clinic Foundation, OH, USA.

http://journals.sagepub.com/doi/10. Read More

View Article and Full-Text PDF

Post-cardiac surgery thrombotic thrombocytopenic purpura: presence of anti-ADAMTS13 autoantibodies at preoperative baseline.

Platelets 2021 Apr 14:1-5. Epub 2021 Apr 14.

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Thrombotic thrombocytopenic purpura (TTP) rarely complicates acute inflammatory conditions such as surgery, including post-cardiac surgery. Review of 32 previously-reported cases of post-cardiac surgery TTP indicates that this disorder often occurs as early as 2-3 days following surgery, which seems too soon to implicate new formation of anti-ADAMTS13 autoantibodies as a consequence of surgery itself. We diagnosed post-cardiac surgery TTP in a 60-year-old female that began approximately 3 days post-coronary artery bypass surgery in which anti-ADAMTS13 autoantibodies were implicated. Read More

View Article and Full-Text PDF

[Thrombotic thrombocytopenic purpura, in a HIV-infected patient. Case report].

Rev Chilena Infectol 2021 Feb;38(1):108-113

Fundación Cardio-infantil, Bogotá, Colombia.

Hematological manifestations for human immunodeficiency virus (HIV) infection are frequent and diverse due to its ability to affect almost all cell lines. Among these, thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathies syndromes, characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia with impaired renal function. Nowadays, the relationship between these two entities is rare given the current highly active antiretroviral therapy (HAART). Read More

View Article and Full-Text PDF
February 2021