5,672 results match your criteria Thrombotic Thrombocytopenic Purpura


A case off TTP temporally associated with cocaine use: Implications for treatment of suspected DITMA.

J Clin Apher 2019 Feb 18. Epub 2019 Feb 18.

Department of Medicine, West Virginia University Hospital, Morgantown, West Virginia.

Thrombotic microangiopathies are rare diseases associated with significant morbidity and mortality. The treatment of thrombotic thrombocytopenic purpura (TTP) and drug-induced thrombotic microangiopathy (DITMA) remains a diagnostic dilemma as they present similarly but respond differently to standard treatment with plasma exchange. TTP is a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 resulting in von Willebrand factor aggregates. Read More

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http://doi.wiley.com/10.1002/jca.21687
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http://dx.doi.org/10.1002/jca.21687DOI Listing
February 2019
3 Reads

First Report of a Disease by Rhazes 10 Centuries Ago.

Int J Prev Med 2019 15;10. Epub 2019 Jan 15.

Department of Anesthesiology and Intensive Care Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.

Introduction: Abu Bakr Mohammad Ibn Zakariya Al-Razi (865-925 CE), who was known as "Rhazes" in the west, was a famous scientist of medieval ages. He has more than 200 books and treatises. His masterpiece on medicine "" contains around 900 case reports. Read More

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http://dx.doi.org/10.4103/ijpvm.IJPVM_216_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360843PMC
January 2019

Characterization and treatment of congenital thrombotic thrombocytopenic purpura.

Blood 2019 Feb 15. Epub 2019 Feb 15.

Department of Haematology, University College London Hospital, London, United Kingdom.

Congenital Thrombotic Thrombocytopenic Purpura (cTTP) is an ultra rare thrombomicroangiopathy caused by an inherited deficiency of ADAMTS13. There is limited data on the genotype-phenotype correlation and no consensus on treatment. We reviewed the largest cohort of cTTP cases, diagnosed in the UK, over the past 15 years. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-11
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http://dx.doi.org/10.1182/blood-2018-11-884700DOI Listing
February 2019
1 Read

Atypical Thrombotic Thrombocytopenic Purpura Presenting as Stroke.

Case Rep Hematol 2019 14;2019:7425320. Epub 2019 Jan 14.

Division of Hematology, Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, 6411 Fannin Street, Houston 77030, USA.

Here we report a case of atypical thrombotic thrombocytopenic purpura that presented as an ischemic cerebrovascular accident. A 56-year-old man with multiple cardiovascular risk factors presented with sudden left-sided weakness, slurred speech, and left facial droop. He showed mild improvement when he was treated with thrombolytic therapy according to the hospital stroke protocol. Read More

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http://dx.doi.org/10.1155/2019/7425320DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350572PMC
January 2019
2 Reads

Risk of diagnostic delay in congenital thrombotic thrombocytopenic purpura.

J Thromb Haemost 2019 Feb 14. Epub 2019 Feb 14.

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

Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening disorder characterized by multiple organ ischemia due to disseminated thrombi formation in the microvasculature. The congenital form of the disease (Upshaw-Schulman syndrome) is related to ADAMTS13 mutations. Adulthood-onset of TTP does not exclude the congenital form of the disease and a diagnostic delay may account for a great morbidity burden in these patients. Read More

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http://dx.doi.org/10.1111/jth.14409DOI Listing
February 2019
1 Read

Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome.

Curr Opin Rheumatol 2019 Feb 11. Epub 2019 Feb 11.

Division of Rheumatology, Department of Internal Medicine, University of Michigan.

Purpose Of Review: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding - and persistent knowledge gaps - through clinically oriented questions and answers.

Recent Findings: A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Read More

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http://dx.doi.org/10.1097/BOR.0000000000000595DOI Listing
February 2019
1 Read

Cerebral pontine infarctions during pregnancy - A case report and review of the literature.

Case Rep Womens Health 2019 Jan 23;21:e00097. Epub 2019 Jan 23.

University of South Alabama, Children's and Women's Hospital, Department of Obstetrics and Gynecology, 251 Cox Street, Mobile, AL 36604, United States.

Cerebrovascular disease is not uncommon during pregnancy as a result of either venous or arterial occlusion, or a hemorrhagic event, resulting in ischemia. Pregnancy may alter the prognosis of these neurologic disorders, with increased risks of morbidity and mortality for the mother and the developing fetus. Etiologies of stroke during pregnancy and the postpartum period include preeclampsia, eclampsia, HELLP syndrome, posterior reversible encephalopathy syndrome (PRES), amniotic fluid embolism, postpartum angiopathy, postpartum cardiomyopathy, thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), cerebral venous thrombosis, CNS infections, and maternal thrombophilia. Read More

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http://dx.doi.org/10.1016/j.crwh.2019.e00097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358547PMC
January 2019
2 Reads

[Successful management of primary immune thrombocytopenia with romiplostim during open heart surgery in a hemodialysis patient].

Rinsho Ketsueki 2019 ;60(1):28-32

Department of Hematology, Faculty of Medicine, University of Tsukuba.

A 66-year-old male undergoing maintenance hemodialysis presented with mild thrombocytopenia. He also had aortic valve stenosis and required aortic valve replacement. In addition, he required anticoagulation therapy with warfarin because of chronic subclavian artery occlusion. Read More

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http://dx.doi.org/10.11406/rinketsu.60.28DOI Listing
January 2019
2 Reads

Cell Membrane-Derived Microvesicles in Systemic Inflammatory Response.

Folia Biol (Praha) 2018 ;64(4):113-124

Institute of Pathological Physiology, First Faculty of Medicine, Charles University, Prague, Czech Republic.

Human body reacts to physical, chemical and biological insults with a complex inflammatory reaction. Crucial components and executors of this response are endothelial cells, platelets, white blood cells, plasmatic coagulation system, and complement. Endothelial injury and inflammation are associated with elevated blood levels of cell membrane-derived microvesicles. Read More

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January 2018
3 Reads

Thrombotic Microangiopathy, Hemolytic Uremic Syndrome, and Thrombotic Thrombocytopenic Purpura Following Hump-nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka.

Wilderness Environ Med 2019 Jan 30. Epub 2019 Jan 30.

Faculty of Medicine, University of Peradeniya, Peredeniya, Sri Lanka.

Thrombotic microangiopathy (TMA), which includes the spectrum of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, is an uncommon complication of hump-nosed pit viper envenomation. We describe 4 cases of TMA following hump-nosed pit viper (Hypnale spp) bites in Sri Lanka. The first case is a typical TMA that spontaneously resolved with supportive treatments. Read More

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http://dx.doi.org/10.1016/j.wem.2018.10.003DOI Listing
January 2019
1 Read

Association of Appendicitis, Helicobacter Pylori Positive Gastritis and Thrombotic Thrombocytopenic Purpura in an Adolescent.

Am J Case Rep 2019 Jan 31;20:131-133. Epub 2019 Jan 31.

Department of Nephrology, University Hospital in Split, Split, Croatia.

BACKGROUND Thrombotic thrombocytopenic purpura (TTP) in children is a rare life-threatening syndrome, characterized by microangiopathic hemolytic anemia, thrombocytopenia with renal dysfunction, neurologic symptoms, and fever. TTP is usually caused by deficient activity of von Willebrand factor cleaving protease (ADAMTS13), due to either gene mutations or acquired via anti-ADAMTS13 autoantibodies. It can be triggered by bone marrow or solid organ transplantation, cardiothoracic-, abdominal-, and orthopedic surgeries, infections including very rarely Helicobacter pylori infection. Read More

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http://dx.doi.org/10.12659/AJCR.913129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364441PMC
January 2019
2 Reads

AFM Imaging Reveals Multiple Conformational States of ADAMTS13.

J Biol Eng 2019 22;13. Epub 2019 Jan 22.

Institute of Biomechanics, School of Biosciences and Bioengineering, South China University of Technology, Guangzhou, 510006 China.

Background: ADAMTS13 (A disintegrin and metalloprotease with a thrombospondin type 1 motif 13) cleaves Von Willebrand factor (VWF) to regulate its size, thereby preventing aberrant platelet aggregation and thrombus. Deficiency of ADAMTS13 caused by either genetic mutations or by inhibitory autoantibodies against ADAMTS13 leads to thrombotic thrombocytopenic purpura (TTP). Recently, ADAMTS13 was reported to adopt a "closed" conformation with lower activity and an "open" one resulting from the engagements of VWF D4-CK domains or antibodies to the distal domains of ADAMTS13, or mutations in its spacer domain. Read More

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http://dx.doi.org/10.1186/s13036-018-0102-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343300PMC
January 2019
1 Read

Thrombotic Thrombocytopenic Purpura due to Checkpoint Inhibitors.

Case Rep Hematol 2018 20;2018:2464619. Epub 2018 Dec 20.

The T.W. Lewis Melanoma Center of Excellence, Banner MD Anderson Cancer Center, Gilbert, AZ, USA.

Ipilimumab is a monoclonal antibody that enhances the efficacy of the immune system by targeting a cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), which is a protein receptor that downregulates the immune system. Nivolumab is also a humanized monoclonal antibody that targets another protein receptor that prevents activated T cells from attacking the cancer; this receptor is called programmed cell death 1 (PD-1). The FDA approved ipilimumab combined with nivolumab as a frontline therapy for patients with metastatic melanoma or renal cell carcinoma and as a second-line therapy for patients with microsatellite instability-high (MSI-H) metastatic colon cancer. Read More

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http://dx.doi.org/10.1155/2018/2464619DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6317083PMC
December 2018
2 Reads

Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine.

Res Pract Thromb Haemost 2019 Jan 16;3(1):26-37. Epub 2018 Nov 16.

Departments of Epidemiology & Biostatistics, Medicine University of Oklahoma Health Sciences Center Oklahoma City Oklahoma.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by severe congenital or immune-mediated deficiency in ADAMTS13, the enzyme that cleaves von Willebrand factor multimers. This rare condition leads invariably and rapidly to a fatal outcome in the absence of treatment, and therefore raises multiple diagnostic and therapeutic challenges. The novel concepts and mechanisms identified in the laboratory for this disease have been rapidly and successfully translated into the clinic for the benefit of patients, making TTP an archetypal disease that has benefited from targeted therapies. Read More

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http://dx.doi.org/10.1002/rth2.12160DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332733PMC
January 2019
3 Reads

Malignant benign hematology.

Res Pract Thromb Haemost 2019 Jan 12;3(1):15-17. Epub 2018 Dec 12.

Departments of Medicine and Laboratory Medicine and Pathobiology St. Michael's Hospital Li Ka Shing Knowledge Institute University of Toronto Toronto Ontario Canada.

When faced with a life-threatening non-cancerous blood disorder, the term "benign" is a misnomer. Devastating diseases like catastrophic antiphospholipid antibody syndrome, acquired hemophilia, and severe immune thrombocytopenia present a challenge to the hematologist. They are often difficult to treat and lack appropriately powered, unbiased evidence to support management. Read More

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http://dx.doi.org/10.1002/rth2.12170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332750PMC
January 2019
5 Reads

Myelophthisic Anemia in a Patient with Lobular Breast Carcinoma Metastasized to the Bone Marrow.

Cureus 2018 Nov 4;10(11):e3541. Epub 2018 Nov 4.

Hematology / Oncology, The Brooklyn Hospital Center, Brooklyn, USA.

Breast tumors have a predilection for metastasizing to the bone leading to cells being displaced by the cancer cells subsequently producing immature leukocytes and erythrocytes in the peripheral blood. We present a case of a 57-year-old female who was found to have myelophthisic anemia secondary to stage four lobular breast carcinoma metastasized to the bone marrow after being misdiagnosed as having thrombotic thrombocytopenia purpura. Diagnosis of myelophthisic anemia requires a thorough workup and treatment is based upon secondary management of the malignancy. Read More

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http://dx.doi.org/10.7759/cureus.3541DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6324862PMC
November 2018
2 Reads

[Analysis of 5 children with congenital thrombotic thrombocytopenic purpura].

Zhonghua Er Ke Za Zhi 2019 Jan;57(1):50-54

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

To explore the clinical features and points of diagnosis and treatment for congenital thrombotic thrombocytopenic purpura (TTP) in children. The clinical manifestations, laboratory tests, genetic analysis and treatments of 5 children with congenital TTP hospitalized in Beijing Children's Hospital, Capital Medical University from February 2015 to July 2017 were analyzed retrospectively. Among the 5 children with congenital TTP diagnosed by genetic monitoring and enzymology, there were 1 male and 4 females, 3 cases had suspicious positive family history, the age of onset was several hours after birth (range several hours after birth to 28 months). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.01.012DOI Listing
January 2019
5 Reads

Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series.

J Thromb Haemost 2019 Jan 10. Epub 2019 Jan 10.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

Background: Severe ADAMTS13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions including surgery may influence the levels of ultra large Von Willebrand factor and ADAMTS13, acting as a trigger for an acute TTP event.

Objectives: To report our experience for management of six patients affected with acquired TTP who underwent elective surgery after a prophylactic treatment to restore ADAMTS13 activity levels. Read More

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http://doi.wiley.com/10.1111/jth.14381
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http://dx.doi.org/10.1111/jth.14381DOI Listing
January 2019
9 Reads

Thrombotic Thrombocytopenic Purpura Following Aortic Valve Replacement with St. Jude Medical Trifecta Bio-Prosthesis.

Cardiol Res 2018 Dec 7;9(6):392-394. Epub 2018 Dec 7.

Oxford Heart Centre, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.

Thrombocytopenia is a recognized complication following aortic valve replacement (AVR). While post-operative thrombotic thrombocytopenic purpura (TTP) is less common than heparin-induced thrombocytopenia (HIT), it is associated with high mortality and morbidity and prompt diagnosis and treatment is vital. In this case report, we describe the first reported case of TTP after AVR using the trifecta bio-prosthesis. Read More

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http://dx.doi.org/10.14740/cr780wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306118PMC
December 2018
15 Reads

Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.

N Engl J Med 2019 01 9;380(4):335-346. Epub 2019 Jan 9.

From the Department of Haematology, University College London Hospitals, Cardiometabolic Program, National Institute for Health Research UCLH-UCL Biomedical Research Center, London (M.S.); the Division of Hematology, Department of Internal Medicine, Ohio State University, Columbus (S.R.C.); Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, and the Department of Pathophysiology and Transplantation, University of Milan, Milan (F.P.); the Department of Hematology, Saint-Antoine University Hospital, Paris (P.C.); the Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna (P.K.); the Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland (J.A.K.H.); the Division of Hematology, Duke University School of Medicine, Durham, NC (A.M.); the Hematology Department, Universidad Católica de Valencia Hospital Dr. Peset, Valencia, Spain (J.R.); the Departments of Medicine and Laboratory Medicine, St. Michael's Hospital and University of Toronto, Toronto (K.P.); and Clinical Development, Ablynx, Zwijnaarde, Belgium (F.C., D.B., H.D.W., R.K.Z.).

Background: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

Methods: In this double-blind, controlled trial, we randomly assigned 145 patients with TTP to receive caplacizumab (10-mg intravenous loading bolus, followed by 10 mg daily subcutaneously) or placebo during plasma exchange and for 30 days thereafter. Read More

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http://dx.doi.org/10.1056/NEJMoa1806311DOI Listing
January 2019
3 Reads

Cardiac implications of thrombotic thrombocytopenic purpura.

World J Cardiol 2018 Dec;10(12):254-266

Division of Cardiology, Lang Research Center, New York Presbyterian Medical Group - Queens Hospital, Flushing, NY 11355, United States.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that essentially can affect any organ in the human body. The hallmark of the pathogenesis in TTP is the large von Willebrand factor multimers on platelet-mediated micro-thrombi formation, leading to microvascular thrombosis. Autopsy studies showed that cardiac arrest and myocardial infarction are the most common immediate causes of death in these patients. Read More

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https://www.wjgnet.com/1949-8462/full/v10/i12/254.htm
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http://dx.doi.org/10.4330/wjc.v10.i12.254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314883PMC
December 2018
5 Reads

von Willebrand factor/ADAMTS-13 interactions at birth: implications for thrombosis in the neonatal period.

J Thromb Haemost 2018 Dec 29. Epub 2018 Dec 29.

Hemostasis Branch, Division of Plasma Protein Therapeutics, Office of Tissues and Advanced Therapies, Center for Biologics Evaluation and Research, US FDA, Silver Spring, MD, USA.

von Willebrand factor (VWF) and its cleaving protease ADAMTS-13 (A Disintegrin and Metalloproteinase with Thrombospondin type 1 motif, member 13) are essential components to hemostasis. These plasma proteins have also been implicated in a number of disease states, including those affecting children. The best described abnormality is the congenital form of thrombotic thrombocytopenic purpura (TTP) resulting from germline mutations in the ADAMTS-13 gene. Read More

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http://doi.wiley.com/10.1111/jth.14374
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http://dx.doi.org/10.1111/jth.14374DOI Listing
December 2018
8 Reads

Acquired autoimmune thrombotic thrombocytopenic purpura.

Authors:

Nursing 2019 Jan;49(1):29-30

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http://dx.doi.org/10.1097/01.NURSE.0000552822.05671.65DOI Listing
January 2019
2 Reads

Leptospirosis complicated with Guillain Barre syndrome, papillitis and thrombotic thrombocytopenic Purpura; a case report.

BMC Infect Dis 2018 Dec 22;18(1):691. Epub 2018 Dec 22.

National Hospital of Sri Lanka, Colombo 10, Sri Lanka.

Background: Leptospirosis is a zoonosis commonly prevalent in tropical countries. Clinical course of leptospirosis varies from mild to severe disease. Here we present a case of leptospirosis complicated with Guillain-Barre Syndrome (GBS), papillitis, and Thrombotic Thrombocytopenic Purpura(TTP). Read More

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http://dx.doi.org/10.1186/s12879-018-3616-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6303948PMC
December 2018
2 Reads

A child with anemia, thrombocytopenia, renal failure and elevated amylase, and lipase enzymes: Questions.

Pediatr Nephrol 2018 Dec 19. Epub 2018 Dec 19.

Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey.

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http://dx.doi.org/10.1007/s00467-018-4159-0DOI Listing
December 2018
2 Reads

Thrombotic thrombocytopenic purpura (TTP)-like syndrome in the HIV era.

Thromb J 2018 13;16:35. Epub 2018 Dec 13.

Department of Molecular Medicine and Haematology, Faculty of Health Sciences, University of Witwatersrand and National Health Laboratory Service, Office 3B20, 7 York Road, Parktown, Johannesburg, 2191 South Africa.

Background: The thrombotic microangiopathies (TMAs) is a heterogeneous group of relatively uncommon but serious disorders presenting with thrombocytopenia and microangiopathic haemolysis. Thrombotic thrombocytopenic purpura (TTP) is one of these microangiopathic processes. HIV infection is an acquired cause of TTP but the pathogenesis is poorly understood. Read More

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https://thrombosisjournal.biomedcentral.com/articles/10.1186
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http://dx.doi.org/10.1186/s12959-018-0189-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291936PMC
December 2018
8 Reads

Comparison of ADAMTS13 and Von Willebrand factor levels and activities, and plasminogen levels, in plasma products currently available for the treatment of thrombotic thrombocytopenic purpura in South Africa.

Transfus Apher Sci 2018 Dec 5. Epub 2018 Dec 5.

Department of Haematology and Cell Biology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa; National Health Laboratory Service (NHLS), Universitas Hospital Haematology Academic Laboratories, Bloemfontein, South Africa.

Objective: Thrombotic thrombocytopenic purpura (TTP) results from a deficiency in the Von Willebrand factor (VWF) cleaving protease, ADAMTS13. Treatment involves plasma exchange (PEX) therapy with either fresh frozen plasma (FFP), cryosupernatant (CSP) or solvent/detergent-treated plasma (SDP), available in South Africa as Bioplasma FDP. The aim of the study was to generate in vitro data on these products, and to explore possible differences between the products that may offer treatment advantages. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14730502183027
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http://dx.doi.org/10.1016/j.transci.2018.11.005DOI Listing
December 2018
8 Reads

Maintenance rituximab for relapsing thrombotic thrombocytopenic purpura: a case report.

Transfusion 2018 Dec 12. Epub 2018 Dec 12.

Department of Internal Medicine, College of Medicine, Department of Biostatistics & Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma.

Background: Appropriate management to prevent relapses of acquired, autoimmune thrombotic thrombocytopenic purpura (TTP) is not clear. Rituximab (375 mg/m /week × 4) is effective treatment for acute episodes but it is not consistently effective for prevention of relapses. Maintenance rituximab, 375 mg/m /3 months for 2 years, is commonly used to prevent progression of follicular lymphoma, but the outcome of maintenance rituximab to prevent TTP relapses has been rarely reported. Read More

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http://dx.doi.org/10.1111/trf.15093DOI Listing
December 2018
9 Reads

Kidney injury following envenoming by hump-nosed pit viper (Genus: Hypnale) in Sri Lanka: proven and probable cases.

Trans R Soc Trop Med Hyg 2018 Dec 13. Epub 2018 Dec 13.

Department of Medicine, Faculty of Medicine, University of Peradeniya, Sri Lanka.

Background: Hump-nosed pit vipers (Hypnale hypnale) are highly venomous snakes found in Sri Lanka and the Western Ghats region of India. They are the most common cause of venomous snakebites in Sri Lanka, mainly causing local envenoming leading to pain, swelling and necrosis. Systemic envenoming causing acute kidney injury and coagulopathy are more commonly recognized following their bites. Read More

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http://fdslive.oup.com/www.oup.com/pdf/production_in_progres
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http://dx.doi.org/10.1093/trstmh/try120DOI Listing
December 2018
11 Reads

The Association Between Human Leukocyte Antigens and ITP, TTP, and HIT.

J Pediatr Hematol Oncol 2019 Mar;41(2):81-86

Thalassemia and Hemoglobinopathy Research Center, Research Institute of Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Background: Autoimmune thrombocytopenia in immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura (TTP), and heparin-induced thrombocytopenia (HIT) is associated with immunologic degradation of platelets and reduced platelet counts in patients, leading to bleeding risk in patients. Considering the role of human leukocyte antigens (HLA) in the development of immune response, in this review, we examine the relationship between HLA and pathogenesis of the above-mentioned diseases.

Methods: Relevant English-language literature was searched and retrieved from Google Scholar search engine and PubMed database (1979 to 2018). Read More

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http://dx.doi.org/10.1097/MPH.0000000000001381DOI Listing

Generation of anti-idiotypic antibodies to detect anti-spacer antibody idiotopes in acute thrombotic thrombocytopenic purpura patients.

Haematologica 2018 Dec 6. Epub 2018 Dec 6.

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

In autoantibody-mediated autoimmune diseases, autoantibody profiling allows to stratify patients and link autoantibodies with disease severity and outcome. However, in immune-mediated thrombotic thrombocytopenic purpura patients, stratification according to antibody profiles and their clinical relevance has not been fully explored. We aimed at developing a new type of autoantibody profiling assay for immune-mediated thrombotic thrombocytopenic purpura based on the use of anti-idiotypic antibodies. Read More

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http://dx.doi.org/10.3324/haematol.2018.205666DOI Listing
December 2018
1 Read

Thrombotic thrombocytopenic purpura: A 5-year tertiary care centre experience.

J Clin Apher 2019 Feb 8;34(1):44-50. Epub 2018 Dec 8.

Department of Molecular Medicine and Haematology, Faculty of Health Sciences, National Health Laboratory Service, University of the Witwatersrand, Johannesburg, South Africa.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is associated with high mortality if not managed timeously with therapeutic plasma exchange (TPE). TTP secondary to human immunodeficiency virus (HIV) infection is unique to sub-Saharan Africa. The management and outcome of TTP in the era of improved access to therapy has not been described. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/jca.21673
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http://dx.doi.org/10.1002/jca.21673DOI Listing
February 2019
3 Reads

Acquired autoimmune thrombotic thrombocytopenic purpura.

Authors:
Vincent M Vacca

Nursing 2019 Jan;49(1):22-29

Vincent M. Vacca, Jr., is an adjunct faculty member at Massachusetts College of Pharmacy and Health Sciences in Boston, Mass.

Acquired autoimmune thrombotic thrombocytopenic purpura (TTP)-the most common form of TTP-is a life-threatening hematologic disease characterized by hemolytic anemia and thrombocytopenia. Acquired autoimmune TTP can cause signs and symptoms of neurologic and other organ involvement, with mortality approaching 90% if the disease is not promptly recognized and treated. Since the introduction of plasma exchange in 1991, the acquired autoimmune TTP survival rate has increased to 78%. Read More

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http://dx.doi.org/10.1097/01.NURSE.0000549721.69197.4dDOI Listing
January 2019
1 Read

TTP: long-term outcomes following recovery.

Authors:
James N George

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):548-552

Hematology-Oncology Section, Department of Medicine, College of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK.

Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.548DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246011PMC
November 2018
1 Read

Beyond plasma exchange: novel therapies for thrombotic thrombocytopenic purpura.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):539-547

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

The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246029PMC
November 2018
11 Reads

Clinical and laboratory diagnosis of TTP: an integrated approach.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):530-538

Department of Medicine and Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.530DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246034PMC
November 2018
11 Reads

Atypical Hemolytic Uremic Syndrome Presenting as Pre-eclampsia in a 24-year-old Woman with Chronic Kidney Disease: Pathogenesis and Genetics.

Cureus 2018 Sep 25;10(9):e3358. Epub 2018 Sep 25.

Internal Medicine, Brookdale University Hospital, New York, USA.

Atypical hemolytic uremic syndrome (aHUS) is a kidney disorder that is frequently unrecognized during its progression, and misdiagnosed with more common etiologies of microangiopathic hemolytic anemia (MAHA): hemolytic uremic syndrome, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura (TTP). During pregnancy, the diagnosis of aHUS is furthermore challenging. The clinical presentation of aHUS may mimic pre-eclampsia as it occurred to the patient described in the case report. Read More

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http://dx.doi.org/10.7759/cureus.3358DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257492PMC
September 2018
3 Reads

[The 466th case:myasthenia,fever,blurred vision and pancytopenia].

Zhonghua Nei Ke Za Zhi 2018 Dec;57(12):942-944

Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1426.2018.12.015DOI Listing
December 2018
3 Reads

Fat embolism syndrome due to bone marrow necrosis in patients with hemoglobinopathies: A life-threatening complication mimicking thrombotic thrombocytopenic purpura.

Am J Hematol 2019 Feb 10;94(2):E64-E66. Epub 2018 Dec 10.

Division of Laboratory Medicine, Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama.

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https://onlinelibrary.wiley.com/doi/abs/10.1002/ajh.25363
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http://dx.doi.org/10.1002/ajh.25363DOI Listing
February 2019
4 Reads

Pharmacokinetics of plasma infusion in congenital thrombotic thrombocytopenic purpura.

J Thromb Haemost 2019 Jan 31;17(1):88-98. Epub 2018 Dec 31.

Department of Haematology, UCLH and Cardiometabolic Programme-NIHR UCLH/UC BRC London, London, UK.

Essentials Congenital thrombotic thrombocytopenic purpura (TTP) is primarily treated with plasma infusion. We present a pharmacokinetic analysis of ADAMTS-13 in six patients following plasma infusion. A median half-life of 130 h was demonstrated, ranging between 82. Read More

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http://dx.doi.org/10.1111/jth.14345DOI Listing
January 2019
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Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome.

Transfus Apher Sci 2018 Dec 16;57(6):790-792. Epub 2018 Nov 16.

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Molecular Endocrinology and Metabolism, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum. We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. Read More

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http://dx.doi.org/10.1016/j.transci.2018.10.023DOI Listing
December 2018
7 Reads

Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission.

Thromb Res 2019 01 12;173:42-47. Epub 2018 Nov 12.

Department of Medicine, University of Padua, Padua, Italy.

Introduction: ADAMTS13 deficiency results in unusually large von Willebrand factor (ULVWF) multimers in the circulation and a higher risk of microthrombi due to high shear stress. In patients treated for acquired thrombotic thrombocytopenic purpura (TTP), a persistently severe ADAMTS13 deficiency (<10%) in remission is associated with more relapses. A reduced plasma ADAMTS13 activity and increased VWF levels are associated with a higher risk of myocardial infarction. Read More

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http://dx.doi.org/10.1016/j.thromres.2018.11.005DOI Listing
January 2019
8 Reads

Low titer A plasma in three AB patients for therapeutic plasma exchange.

J Clin Apher 2019 Feb 23;34(1):7-12. Epub 2018 Nov 23.

Institute for Transfusion Medicine, Pittsburgh, Pennsylvania.

Background: Currently, low titer A plasma is used on a routine basis in bleeding trauma patients of unknown AB type. Three AB non-bleeding apheresis patients are presented here who safely received a combination of AB and low titer A plasma during therapeutic plasma exchange (TPE). One control AB patient received AB plasma only. Read More

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http://dx.doi.org/10.1002/jca.21656DOI Listing
February 2019
26 Reads

Chest pain with increased troponin level; not always a cardiology issue.

Rom J Intern Med 2018 Nov 15. Epub 2018 Nov 15.

Hematology Unit,General Hospital of Trikala, Thessaly,Greece.

Thrombotic Thrombocytopenic Purpura (TTP) is a thrombotic microangiopathy syndrome resulting from decrease or absence of "a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13" (ADAMTS13). TTP has been characterized by the classical pentad of thrombocytopenia, hemolysis, fever, renal injury and neurological deficits, yet the patient may present with any atypical symptom related to microthrombi formation in the microcirculation. Here we present a rare case of a young patient with retrosternal chest pain and myocardial injury as the first manifestation of TTP. Read More

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http://dx.doi.org/10.2478/rjim-2018-0037DOI Listing
November 2018
15 Reads

Multidisciplinary team approach for an atypical presentation of postpartum thrombotic thrombocytopenic purpura and severe preeclampsia in the Intensive Care Unit.

Saudi J Anaesth 2018 Oct-Dec;12(4):640-642

Division of Critical Care, Department of Anesthesia, Pain Management and Perioperative Medicine, Henry Ford Hospital, Detroit, Michigan, USA.

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic syndrome during pregnancy with overlapping features of severe preeclampsia and is associated with high morbidity and mortality. We present a case of postpartum TTP, associated with severe preeclampsia. Therapeutic approach for this case included corticosteroids, plasma exchange therapy, and immunomodulatory therapy. Read More

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http://dx.doi.org/10.4103/sja.SJA_204_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6180678PMC
November 2018
10 Reads

Postoperative Thrombotic Thrombocytopenic Purpura after Total Hip Arthroplasty: A Case Report and Review of the Literature.

Case Rep Orthop 2018 23;2018:9716170. Epub 2018 Oct 23.

Department of Orthopaedic Surgery, Emory University School of Medicine, Atlanta, GA 30329, USA.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon and potentially lethal microangiopathy that carries a >90% mortality rate if not treated urgently and appropriately. Postoperative TTP after orthopaedic procedures is particularly rare with only four case reports existing in the literature. We present the case of postoperative TTP in a 57-year-old female who underwent elective total hip arthroplasty. Read More

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http://dx.doi.org/10.1155/2018/9716170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218747PMC
October 2018
2 Reads

Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab.

Case Rep Nephrol 2018 23;2018:6051083. Epub 2018 Oct 23.

Medical student, Javeriana University, Bogota, Colombia.

We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. Read More

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https://www.hindawi.com/journals/crin/2018/6051083/
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http://dx.doi.org/10.1155/2018/6051083DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218791PMC
October 2018
8 Reads

Relapsed/refractory acquired thrombotic thrombocytopenic purpura in a patient with Sjögren syndrome: Case report and review of the literature.

Medicine (Baltimore) 2018 Oct;97(43):e12989

Department of Immunology and Rheumatology.

Rationale: Thrombotic thrombocytopenic purpura (TTP) is a rare, fatal disorder which could be caused by autoimmune diseases. However, TTP secondary to Sjögren syndrome (SS) is extremely rare.

Patient Concerns: A 47-year- old woman with an 8-year history of SS was admitted due to skin ecchymosis and bleeding gums. Read More

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http://dx.doi.org/10.1097/MD.0000000000012989DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221612PMC
October 2018
13 Reads