5,638 results match your criteria Thrombotic Thrombocytopenic Purpura


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

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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http://dx.doi.org/10.1016/j.transci.2018.11.005DOI Listing
December 2018

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
2 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
1 Read

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

J Pediatr Hematol Oncol 2018 Dec 12. Epub 2018 Dec 12.

Thalassemia and Hemoglobinopathy Research Center, Research Institute of Health.

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
December 2018

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

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

J Clin Apher 2018 Dec 8. 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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http://dx.doi.org/10.1002/jca.21673DOI Listing
December 2018

Acquired autoimmune thrombotic thrombocytopenic purpura.

Authors:
Vincent M Vacca

Nursing 2018 Dec 10. Epub 2018 Dec 10.

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
December 2018

TTP: long-term outcomes following recovery.

Authors:
James N George

Hematology Am Soc Hematol Educ Program 2018 Nov;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 Nov;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
4 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
2 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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December 2018
2 Reads

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

Am J Hematol 2018 Nov 27. Epub 2018 Nov 27.

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

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http://dx.doi.org/10.1002/ajh.25363DOI Listing
November 2018

Pharmacokinetics of plasma infusion in congenital thrombotic thrombocytopenic purpura.

J Thromb Haemost 2018 Nov 26. Epub 2018 Nov 26.

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

Background: Congenital thrombotic thrombocytopenic purpura (TTP) is defined by persistent severe deficiency of ADAMTS-13 in the absence of anti-ADAMTS-13 inhibitory antibodies, confirmed by mutational analysis. Replacement of the missing protease prevents disease relapse, primarily using plasma infusion (PI).

Objectives, Patients And Methods: There is scant evidence regarding optimal dose and frequency of treatment, tending to be empirically guided. Read More

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http://dx.doi.org/10.1111/jth.14345DOI Listing
November 2018
1 Read

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
1 Read

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

Thromb Res 2018 Nov 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
November 2018
3 Reads

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

J Clin Apher 2018 Nov 23. 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
November 2018
12 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
7 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
4 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
1 Read

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
3 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
10 Reads

A Case of Recurrent Thrombotic Microangiopathy Caused by Hypertensive Urgency.

Cureus 2018 Aug 30;10(8):e3235. Epub 2018 Aug 30.

Internal Medicine, West Virginia University/Charleston Area Medical Center, Charleston, USA.

A 26-year-old man presented to the emergency room with abdominal pain, nausea, and vomiting for four days. His medical history was significant for hypertension and end-stage renal disease managed with hemodialysis. He had been noncompliant with the antihypertensive regimen which included nifedipine, hydralazine, and spironolactone. Read More

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http://dx.doi.org/10.7759/cureus.3235DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207490PMC
August 2018
4 Reads

ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome.

J Clin Apher 2018 Nov 5. Epub 2018 Nov 5.

Blood Centre, University Hospital Ostrava, Ostrava, Czech Republic.

Background: Hereditary thrombotic thrombocytopenic purpura, also called Upshaw-Schulman syndrome (USS), is a rare disease caused by genetic mutations in the ADAMTS13 gene, which severely decrease the activity of ADAMTS13, a metalloprotease that cleaves von Willebrand factor multimers (VWF). Genotypically identical patients can show great phenotypic diversity.

Objectives: Comparison of selected laboratory parameters and ADAMTS13 pharmacokinetics among patients with USS was performed. Read More

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http://dx.doi.org/10.1002/jca.21664DOI Listing
November 2018
1 Read

Impact of a multidisciplinary team for the management of thrombotic microangiopathy.

PLoS One 2018 2;13(11):e0206558. Epub 2018 Nov 2.

Department of Gynecology and Obstetrics. Son Espases University Hospital, Palma de Mallorca, Spain.

Background: Thrombotic microangiopathy (TMA) is an important complication associated with several diseases that are rare and life-threatening. TMA is common to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). TTP is defined by a severe deficiency of ADAMTS13, and early treatment is associated with good prognosis. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0206558PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214549PMC
November 2018
16 Reads

Thrombotic thrombocytopenic purpura and hemophagocytic lymphohistiocytosis in an elderly man: A case report.

Medicine (Baltimore) 2018 Nov;97(44):e13025

Hematology Unit, Medical Specialty Center, Bangkok Hospital Hat Yai, Bangkok Dusit Medical Services, Hat Yai, Songkhla, Thailand.

Rationale: Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients. Read More

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http://dx.doi.org/10.1097/MD.0000000000013025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221720PMC
November 2018
1 Read
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Adult onset haemophagocytic lymphohistiocytosis prognosis is affected by underlying disease: analysis of a single-institution series of 174 patients.

Swiss Med Wkly 2018 09 23;148:w14641. Epub 2018 Sep 23.

Senior Department of Haematology, The First Affiliated Hospital, Medical School of Zhejiang University, Hangzhou, Zhejiang Province, China.

Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterised by activation of the mononuclear phagocytic system, and often leads to progressive multiple organ failure. The diagnosis of HLH is made late by most physicians.

Methods: To confirm the diagnosis of acquired HLH made in a single-institution series of adult patients with HLH-04 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome. Read More

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http://dx.doi.org/10.4414/smw.2018.14641DOI Listing
September 2018
3 Reads

A Rare Case of Thrombotic Thrombocytopenic Purpura Caused by Pancreatitis and Clopidogrel.

Am J Case Rep 2018 Oct 30;19:1288-1291. Epub 2018 Oct 30.

Department of Internal Medicine, Charleston Area Medical Center, Charleston, WV, USA.

BACKGROUND Thrombotic thrombocytopenic purpura is mostly characterized by symptoms and signs of hemolytic anemia, thrombocytopenia, renal impairment, fever and neurologic dysfunction. It is not always necessary to have all 5 characteristic symptoms, and presentations can vary. It can be congenital or acquired by any etiology that causes deficiency or dysfunction of ADAMST13 enzyme. Read More

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https://www.amjcaserep.com/abstract/index/idArt/911679
Publisher Site
http://dx.doi.org/10.12659/AJCR.911679DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6223198PMC
October 2018
3 Reads

Bone Marrow Necrosis in Sickle Cell-Beta Thalassemia Patient Mimicking Thrombotic Thrombocytopenic Purpura.

Ann Clin Lab Sci 2018 Sep;48(5):670-673

Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX, USA

We describe a case of bone marrow necrosis (BMN) with subsequent fat embolism precipitated by high altitudes in a young healthy man with previously undiagnosed sickle cell-beta thalassemia. This case report adds to the growing literature on the complexity of diagnosing bone marrow necrosis given that the clinical and laboratory features mimic thrombotic thrombocytopenic purpura. This BMN with fat embolism syndrome (FES) is novel in that it was precipitated by high altitudes and the patient was treated successfully with a series of therapeutic plasma exchanges and red blood cell transfusions alone, without any adverse long-term complications to the patient. Read More

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September 2018
1 Read

Acute Hepatitis Caused by Genotype 4 HCV Presenting with Microangiopathic Hemolytic Anemia.

ACG Case Rep J 2018 17;5:e73. Epub 2018 Oct 17.

Department of Clinical Pathology, Alexandria University, Alexandria, Egypt.

Many cases have been reported about the role of chronic hepatitis C and interferon therapy in the development and recurrence of thrombotic thrombocytopenic purpura (TTP), but to our knowledge there is no previous report about the association between acute hepatitis C and any microangiopathic hemolytic anemia (MAHA) including TTP. We report a case of acute hepatitis C that presented with MAHA resembling TTP, which resolved with spontaneous clearance of viral infection. Read More

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http://dx.doi.org/10.14309/crj.2018.73DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6195645PMC
October 2018
2 Reads

[The diagnostic value of PLASMIC score in the discrimination between thrombotic thrombocytopenic purpura and disseminated intravascular coagulation].

Zhonghua Xue Ye Xue Za Zhi 2018 Oct;39(10):812-816

Jiangsu Institute of Hematology, MOH Key Lab of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.

PLASMIC score was evaluated its value in differential diagnosis between the patients with thrombotic thrombocytopenic purpura (TTP) and those with disseminated intravascular coagulation (DIC) . Twenty-four patients with TTP and 41 cases with DIC were retrospectively analyzed in this study. The platelet count, average red blood cell volume, indirect bilirubin, creatinine and prothrombin time international normalised ratio were collected, and then PLASMIC scores were calculated. Read More

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October 2018
1 Read

No More Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome Please.

Authors:
Yeong-Hau H Lien

Am J Med 2018 Oct 24. Epub 2018 Oct 24.

Department of Medicine, College of Medicine, University of Arizona, Tucson; Arizona Kidney Disease and Hypertension Center, Tucson. Electronic address:

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http://dx.doi.org/10.1016/j.amjmed.2018.10.009DOI Listing
October 2018
2 Reads

A pediatric neurologic assessment score may drive the eculizumab-based treatment of Escherichia coli-related hemolytic uremic syndrome with neurological involvement.

Pediatr Nephrol 2018 Oct 25. Epub 2018 Oct 25.

Pediatric Nephrology and Dialysis Unit, Pediatric Hospital "Giovanni XXIII", Bari, Italy.

Background: Thrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC).

Methods: TMA mainly affects the kidney, but extra-renal complications are frequently described. Read More

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http://link.springer.com/10.1007/s00467-018-4112-2
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http://dx.doi.org/10.1007/s00467-018-4112-2DOI Listing
October 2018
7 Reads

Thrombotic Thrombocytopenic Purpura Associated with Dermatomyositis.

Cureus 2018 Aug 20;10(8):e3161. Epub 2018 Aug 20.

Internal Medicine, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, New York, USA.

Dermatomyositis and thrombotic thrombocytopenic purpura (TTP) are both rare diseases. TTP is a blood abnormality in which blood clots form in blood vessels leading to fatal outcomes. Dermatomyositis is an inflammatory myopathy which causes a distinctive skin rash and muscle weakness. Read More

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http://dx.doi.org/10.7759/cureus.3161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6197508PMC
August 2018
1 Read

Relevance of antiphospholipid antibody profile in the clinical outcome of ITP: a single-centre study.

Hematology 2019 Dec 25;24(1):134-138. Epub 2018 Oct 25.

a Department of Medicine , Padova University School of Medicine , Padova , Italy.

Objectives: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown.

Methods: In this study, we report our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment. Read More

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http://dx.doi.org/10.1080/10245332.2018.1532649DOI Listing
December 2019

Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.

Arterioscler Thromb Vasc Biol 2018 Nov;38(11):2731-2743

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

Objective- ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats-13) cleaves VWF (von Willebrand factor). This process is essential for hemostasis. Severe deficiency of plasma ADAMTS13 activity, most commonly resulting from autoantibodies against ADAMTS13, causes thrombotic thrombocytopenic purpura. Read More

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https://www.ahajournals.org/doi/10.1161/ATVBAHA.118.311407
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http://dx.doi.org/10.1161/ATVBAHA.118.311407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214616PMC
November 2018
5 Reads

Platelets Can Soak It Up and Then Spit It Out.

Arterioscler Thromb Vasc Biol 2018 Nov;38(11):2544-2545

From the Division of Hematology, Children's Hospital of Philadelphia, PA (K.G., D.F.F., M.P.).

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http://dx.doi.org/10.1161/ATVBAHA.118.311863DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6226015PMC
November 2018
1 Read

Thrombotic thrombocytopenic purpura-like syndrome associated with arcanobacterium pyogenes endocarditis in a post-transplant patient: A case report.

Med J Malaysia 2018 Oct;73(5):344-346

Sarawak General Hospital, Department of Internal Medicine, Haematology Unit, Sarawak, Malaysia.

Systemic Arcanobacterium pyogenes is a rare bacterial infection in humans.1The diagnosis of thrombotic thrombocytopenic purpura (TTP)-like syndrome and infective endocarditis (IE) is often elusive. We report a case of TTP-like syndrome associated with A. Read More

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October 2018
6 Reads

Evaluation of a semi-automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers.

Res Pract Thromb Haemost 2018 Oct 12;2(4):790-799. Epub 2018 Aug 12.

Department of Coagulation Sheffield Haemophilia and Thrombosis Centre Sheffield UK.

Background: The phenotypic diagnosis of von Willebrand disease (VWD) is a multistep process with classification dependent on the quantification of von Willebrand factor (VWF) multimeric structure. VWF multimer analysis is a technically challenging, lengthy and non-standardised assay, usually performed in specialist laboratories. Recently, a new semi-automated multimer assay, the Hydragel 5 von Willebrand multimers (H5VWM) has become available. Read More

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http://doi.wiley.com/10.1002/rth2.12141
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http://dx.doi.org/10.1002/rth2.12141DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6178608PMC
October 2018
3 Reads

Validation of the PLASMIC score, a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis, in Chinese patients.

Thromb Res 2018 Dec 12;172:9-13. Epub 2018 Oct 12.

Department of Clinical Laboratory, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China. Electronic address:

Background: The recently published PLASMIC score was shown to be an excellent diagnostic model to identify patients with severe ADAMTS-13 deficiency. However, it is unclear if this score is suitable for Chinese patients with suspected thrombotic thrombocytopenic purpura (TTP).

Methods: We retrospectively studied a Chinese cohort of 134 consecutively hospitalized patients with thrombotic microangiopathy, ADAMTS-13 test results, and clinical information between January 2015 and June 2018, the PLASMIC score's distinguishing ability in this cohort was evaluated. Read More

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http://dx.doi.org/10.1016/j.thromres.2018.10.010DOI Listing
December 2018
1 Read

Clinical presentation in thrombotic thrombocytopenic purpura: Real-world data from two Mexican institutions.

J Clin Apher 2018 Dec 15;33(6):645-653. Epub 2018 Oct 15.

Hematology Service, Department of Internal Medicine, Hospital Universitario "Dr. José Eleuterio González", Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, Mexico.

Background: Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP.

Study Design And Methods: We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. Read More

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http://dx.doi.org/10.1002/jca.21659DOI Listing
December 2018
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Thrombotic thrombocytopenic purpura associated with Hodgkin lymphoma and non-Hodgkin lymphoma.

Pathology 2018 Dec 9;50(7):776-777. Epub 2018 Oct 9.

Sunshine Coast University Hospital, Birtinya, Qld, Australia.

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http://dx.doi.org/10.1016/j.pathol.2018.05.011DOI Listing
December 2018
1 Read

Caplacizumab: First Global Approval.

Authors:
Sean Duggan

Drugs 2018 Oct;78(15):1639-1642

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Ablynx, a Sanofi Company, has developed the anti-von Willebrand factor Nanobody caplacizumab (Cablivi™) for the treatment of acquired thrombotic thrombocytopenic purpura (aTTP). Based on positive results in phase II and III trials in patients with aTTP, caplacizumab was recently approved in the EU for the treatment of adults experiencing an episode of aTTP, in conjunction with plasma exchange and immunosuppression. This article summarizes the milestones in the development of caplacizumab leading to this first approval. Read More

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http://link.springer.com/10.1007/s40265-018-0989-0
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http://dx.doi.org/10.1007/s40265-018-0989-0DOI Listing
October 2018
8 Reads

Panel sequencing distinguishes monogenic forms of nephritis from nephrosis in children.

Nephrol Dial Transplant 2018 Mar 21. Epub 2018 Mar 21.

Department of Medicine, Division of Nephrology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Background: Alport syndrome (AS) and atypical hemolytic-uremic syndrome (aHUS) are rare forms of chronic kidney disease (CKD) that can lead to a severe decline of renal function. Steroid-resistant nephrotic syndrome (SRNS) is more common than AS and aHUS and causes 10% of childhood-onset CKD. In recent years, multiple monogenic causes of AS, aHUS and SRNS have been identified, but their relative prevalence has yet to be studied together in a typical pediatric cohort of children with proteinuria and hematuria. Read More

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https://academic.oup.com/ndt/advance-article/doi/10.1093/ndt
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http://dx.doi.org/10.1093/ndt/gfy050DOI Listing
March 2018
6 Reads

Vitamin B deficiency-induced pseudothrombotic microangiopathy without macrocytosis presenting with acute renal failure: a case report.

J Med Case Rep 2018 Oct 3;12(1):296. Epub 2018 Oct 3.

Divisione di Clinica Medica, Ospedale San Gerardo, Monza, Italy.

Background: Vitamin B deficiency-induced thrombotic microangiopathy, known as pseudothrombotic microangiopathy, is a rare condition which resembles the clinical features of thrombotic thrombocytopenic purpura but requires a markedly different treatment. Most cases of vitamin B deficiency have only mild hematological findings, but in approximately 10% of patients life-threatening conditions have been reported.

Case Presentation: We report a case of a 46-year-old Moroccan man presenting with severe hemolytic anemia, thrombocytopenia, and renal failure in absence of macrocytosis, thus mimicking a genuine thrombotic thrombocytopenic purpura. Read More

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http://dx.doi.org/10.1186/s13256-018-1815-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6169071PMC
October 2018
2 Reads

Thrombotic thrombocytopenic purpura relapse induced by acute hepatitis E transmitted by cryosupernatant plasma and successfully controlled with ribavirin.

Transfusion 2018 Nov 4;58(11):2501-2505. Epub 2018 Oct 4.

Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain.

Background: Hepatitis E virus (HEV) can be transmitted by transfusion of any type of blood component, but there are few data on the potential risk of transmitting this virus and the associated complications. We provide evidence that HEV can be transmitted by cryosupernatant plasma, and that HEV infection can act as a trigger for thrombotic thrombocytopenic purpura (TTP).

Study Design And Methods: A patient with a history of TTP treated with plasmapheresis 2 months previously developed jaundice and a TTP exacerbation with purpura, thrombocytopenia, schistocytes, and undetectable ADAMTS-13 activity. Read More

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http://dx.doi.org/10.1111/trf.14831DOI Listing
November 2018
6 Reads

Ehrlichiosis masquerading as thrombotic thrombocytopenic purpura.

BMJ Case Rep 2018 Oct 2;2018. Epub 2018 Oct 2.

Department of Pulmonary and Critical Care Medicine, University of Louisville, Louisville, Kentucky, USA.

Ehrlichiosis is a rare tickborne illness that can manifest from an asymptomatic, self-limiting disease to a severe presentation with encephalopathy and renal failure. Ehrlichiosis is diagnosed largely based on patient history with confirmatory tests including peripheral blood smear, serology and PCR. Empiric treatment is warranted in patients with suspected tick bites as a delay in treatment can result in multiorgan failure. Read More

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http://dx.doi.org/10.1136/bcr-2018-226665DOI Listing
October 2018
2 Reads