5,710 results match your criteria Thrombotic Thrombocytopenic Purpura


Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy?

Hematol Rep 2019 Feb 19;11(1):7904. Epub 2019 Mar 19.

Department of Medicine, University of Padua, Italy.

With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging cases. To define the characteristics of subjects who may benefit from splenectomy may ease clinical decision making. Read More

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http://dx.doi.org/10.4081/hr.2019.7904DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6444372PMC
February 2019

Thrombotic Thrombocytopenic Purpura.

N Engl J Med 2019 Apr;380(16):e23

University of Louisville, Louisville, KY

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http://dx.doi.org/10.1056/NEJMicm1813768DOI Listing
April 2019
1 Read

FAM-tastic phospho-regulation of von Willebrand factor activity.

J Thromb Haemost 2019 Apr 15. Epub 2019 Apr 15.

Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.

A recent investigation performed by Da et al. provides evidence that von Willebrand factor (VWF) can be phosphorylated in the A2 domain, a modification that has never before been observed [1]. VWF is a multimeric glycoprotein that mediates platelet adhesion to the endothelium upon vascular injury, a process critical for hemostasis. Read More

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http://dx.doi.org/10.1111/jth.14448DOI Listing
April 2019
2 Reads

[HELLP syndrome and hemolytic uremic syndrome during pregnancy: two disease entities, same causation. Case report and literature review].

G Ital Nefrol 2019 Apr;36(2)

Centro per la Cura e lo Studio della Sindrome Emolitico-Uremica. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano.

Thrombotic microangiopathies (TMA) are a group of diseases that can complicate pregnancy and threaten the lives of both the mother and the fetus. Several conditions can lead to TMA, including thrombotic thrombocytopenic purpura (TTP), HELLP syndrome and hemolytic uremic syndrome (HUS). We describe the case of a 39-year-old woman who presented a HELLP syndrome in the immediate postpartum period. Read More

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April 2019
3 Reads

Elevated plasma levels of soluble C-type lectin-like receptor 2 (CLEC2) in patients with thrombotic microangiopathy.

Thromb Res 2019 Mar 28;178:54-58. Epub 2019 Mar 28.

Deaprtment of Molecular and Laboratory Medicine, Mie University Hospital and Mie University Graduate School of Medicine, Tsu, Japan. Electronic address:

Background: Thrombotic microangiopathy (TMA) is caused by activated platelets. The plasma C-type lectin-like receptor 2 (CLEC2) levels in 58 patients with TMA were examined and compared with those in healthy volunteers and other diseases.

Materials And Methods: The plasma levels of soluble platelet surface glycoprotein VI (GPVI) and CLEC2 were measured in patients with TMA. Read More

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http://dx.doi.org/10.1016/j.thromres.2019.03.018DOI Listing
March 2019
3 Reads

Clinical pharmacology of caplacizumab for the treatment of patients with acquired thrombotic thrombocytopenic purpura.

Expert Rev Clin Pharmacol 2019 Apr 12. Epub 2019 Apr 12.

f Formerly Clinical Development , Ablynx NV , Ghent , Belgium.

Introduction: Caplacizumab is a humanized anti-von Willebrand Factor (vWF) Nanobody® for the treatment of acquired Thrombotic Thrombocytopenic Purpura (aTTP). Caplacizumab targets the A1-domain of vWF, inhibiting the interaction between vWF and platelets. Clinical studies conducted in aTTP patients confirmed the rapid and sustained complete suppression of the vWF activity using an initial intravenous dose of 10 mg, and a maintenance subcutaneous 10 mg daily dosing regimen, with corresponding favorable efficacy and safety profiles. Read More

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https://www.tandfonline.com/doi/full/10.1080/17512433.2019.1
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http://dx.doi.org/10.1080/17512433.2019.1607293DOI Listing
April 2019
6 Reads

A probable case of catastrophic antiphospholipid syndrome: Should high-dose steroids be given in the setting of polymicrobial sepsis?

SAGE Open Med Case Rep 2019 1;7:2050313X19839531. Epub 2019 Apr 1.

Department of Medicine, Weill Cornell Medicine/New York-Presbyterian Hospital, New York, NY, USA.

In this clinical vignette, we present a case of a 59-year-old woman with catastrophic antiphospholipid syndrome likely triggered by polymicrobial sepsis. The diagnostic criteria and clinical manifestations of catastrophic antiphospholipid syndrome are reviewed. We also compare diagnostic criteria and clinical manifestations with other clinical entities in the differential diagnosis, including thrombotic thrombocytopenic purpura-hemolytic-uremic syndrome, disseminated intravascular coagulation, sepsis, and inflammatory bowel disease. Read More

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http://dx.doi.org/10.1177/2050313X19839531DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6444769PMC
April 2019
1 Read

Unique case of autoantibody mediated inactivation of ADAMTS13 in an Indian TTP patient.

Blood Cells Mol Dis 2019 Mar 23;77:29-33. Epub 2019 Mar 23.

Disease Biology Laboratory, Regional Centre for Biotechnology, National Capital Region Biotech Science Cluster, Faridabad, India. Electronic address:

A young Indian female visited hospital as a suspected case of thrombotic thrombocytopenic purpura (TTP) with relapsed thrombotic complications with low platelet counts, infarct in middle cerebral artery and thrombi in microvessels. We first confirmed the deficiency of ADAMTS13 metalloprotease in this patient showing improper cleavage of vWF multimers by her plasma unlike her parents and brother. Although patient had very less ADAMTS13 antigen in plasma, but it did not appear to be the cause of deficiency of the enzyme, because her father had similarly low antigen level and he never had prothrombotic complications. Read More

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http://dx.doi.org/10.1016/j.bcmd.2019.03.003DOI Listing
March 2019
2 Reads

Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report.

J Immunother Cancer 2019 Apr 3;7(1):97. Epub 2019 Apr 3.

Department of Pathology, Sapporo Medical University, School of Medicine, South 1, West 17, Chuo-ku, Sapporo, Hokkaido, 060-8556, Japan.

Background: Immune checkpoint inhibitors (ICIs) have provided more options in the treatment of lung cancer. However, ICIs can cause several unfavorable reactions generally referred to as immune-related adverse effects.

Case Presentation: In this report, we present the case of a 52-year-old woman with successful regression of pleomorphic carcinoma of the lung following nivolumab therapy. Read More

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http://dx.doi.org/10.1186/s40425-019-0582-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448268PMC
April 2019
1 Read

Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review.

J Stroke Cerebrovasc Dis 2019 Mar 28. Epub 2019 Mar 28.

Department of Neurology, Bicêtre Hospital, AP-HP, University Paris Sud Saclay, Le Kremlin-Bicêtre, France.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). In this article, we describe the first case of a young male adult suffering from a hereditary TTP revealed by recurrent strokes, relapsing despite antiplatelet and anticoagulant therapy. Because of the persistent moderate thrombocytopenia, plasmatic ADAMTS13 activity was investigated and was found lower than 5% in the absence of anti-ADAMTS13 IgG. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.03.011DOI Listing
March 2019
2 Reads

Thrombotic Thrombocytopenic Purpura – beyond Empiricism and Plasma Exchange.

Authors:
Han-Mou Tsai

Am J Med 2019 Mar 27. Epub 2019 Mar 27.

Division of Hematology/Oncology, State University of New York Downstate Medical Center, 450 Clarkson Ave, Brooklyn, NY 11203. Electronic address:

For many years after its first description in 1924 thrombotic thrombocytopenic purpura was an intriguing puzzle for clinicians and researchers, not only for its unique pathology, perplexing changes in von Willebrand factor multimers and high rate of rapid fatality but also for its dramatic response to plasma infusion or exchange. The discovery of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat member 13) and its deficiency in thrombotic thrombocytopenic purpura, due to inhibitory autoantibodies or genetic mutations, provides a mechanistic scheme for understanding its pathogenesis. This new knowledge quickly led to the use of rituximab to promote its remission and prevent recurrence. Read More

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http://dx.doi.org/10.1016/j.amjmed.2019.03.009DOI Listing
March 2019
2 Reads

Use of caplacizumab in a child with refractory thrombotic thrombocytopenic purpura.

Pediatr Blood Cancer 2019 Mar 29:e27737. Epub 2019 Mar 29.

St. Jude Children's Research Hospital, Memphis, Tennessee.

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http://dx.doi.org/10.1002/pbc.27737DOI Listing
March 2019
1 Read

New monoclonal/bi-specific antibodies: Reshaping transfusion medicine beyond replacement.

Transfus Apher Sci 2019 Mar 14. Epub 2019 Mar 14.

Graduate Institute of Biomedical Materials and Tissue Engineering, College of Biomedical Engineering, Taipei Medical University, Taipei, Taiwan; International PhD Program in Biomedical Engineering, College of Biomedical Engineering, Taipei Medical University, Taipei, Taiwan. Electronic address:

Since the first successful transfusion in 1818, Transfusion Medicine has evolved significantly. The advent of plasma fractionation and availability of recombinant products allowed for precision replacement therapy to treat many hematological conditions, such as hemophilia, thrombotic thrombocytopenic purpura, and hereditary angioedema. A deeper understanding of the pathophysiology underlying those conditions along with the development of safer monoclonal and bispecific antibodies is now offering safe and effective alternatives to the simple conventional approach of replacing a missing plasma protein. Read More

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http://dx.doi.org/10.1016/j.transci.2019.03.011DOI Listing
March 2019
2 Reads

Comparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Haematology, UCLH NHS Foundation Trust, London, UK.

Immune thrombotic thrombocytopenic purpura (iTTP) is an acute, multisystem thrombotic microangiopathy mediated by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) autoantibodies. Immunosuppression with anti-CD20 therapy is the mainstay of treatment. MabThera's patent has now expired and biosimilars have been approved. Read More

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http://dx.doi.org/10.1111/bjh.15874DOI Listing
March 2019
2 Reads

A case for consideration by apheresis practitioners: Melanoma and PD-1 inhibitor treatment in a patient with multiple relapses of immune thrombotic thrombocytopenic purpura.

Transfus Apher Sci 2019 Mar 13. Epub 2019 Mar 13.

Departments of Medicine, St. Michael's Hospital and University of Toronto, Canada.

Thrombotic Thrombocytopenic Purpura (TTP) is a rare life-threatening disease caused by ADAMTS-13 deficiency. Up to forty percent of patients with TTP relapse, and most relapse within eight years of their first presentation. This case report describes a patient with an aggressive course of TTP who subsequently developed metastatic melanoma while receiving prophylactic rituximab. Read More

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http://dx.doi.org/10.1016/j.transci.2019.03.001DOI Listing
March 2019
2 Reads

Refractory Thrombotic Thrombocytopenic Purpura in a patient with Kaposi sarcoma.

Transfus Apher Sci 2019 Mar 19. Epub 2019 Mar 19.

University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Department of Hematology, Ankara, Turkey.

The case is here presented of a 70-year old male patient with rare coexistence of Kaposi Sarcoma and resistant Thrombotic Thrombocytopenic Purpura (TTP). The Kaposi lesions were determined before the diagnosis of TTP and were exacerbated after receiving TTP-associated immunosuppressive therapy, in particular associated with rituximab. TTP in this case was resistant to conventional therapies such as steroid and plasma exchange and current immunosuppressive (rituximab, cyclophosphamide, vincristin) treatments. Read More

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http://dx.doi.org/10.1016/j.transci.2019.02.003DOI Listing
March 2019
5 Reads

Outcome of Thrombotic Thrombocytopenic Purpura Patients: Single Center Experience

Turk J Haematol 2019 Mar 25. Epub 2019 Mar 25.

Necmettin Erbakan University, Meram Medicine Faculty, Konya, Turkey

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http://dx.doi.org/10.4274/tjh.galenos.2019.2019.0048DOI Listing
March 2019
1 Read

Successful use of TNFα blockade in a severe case of idiopathic non-granulomatous ulcerative jejunoileitis associated with thrombotic thrombocytopenic purpura.

BMJ Open Gastroenterol 2019 8;6(1):e000252. Epub 2019 Feb 8.

Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany.

We describe the case of 50-year-old female patient who presented with severe gastrointestinal symptoms and progressive weight loss of unknown origin. Shortly after admission, she developed an acute flare of thrombotic thrombocytopaenic purpura (TTP) that had to be treated by plasma exchange therapy and rituximab administration. While the signs of TTP subsided, the gastrointestinal symptoms worsened with abdominal cramps, massive gastric retention, malnourishment and a stenosis due to extensive inflammation and wall thickening of the small bowel. Read More

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http://dx.doi.org/10.1136/bmjgast-2018-000252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6398868PMC
February 2019
1 Read

Additional autoimmune disorders in patients with acquired autoimmune thrombotic thrombocytopenic purpura.

Am J Hematol 2019 Mar 21. Epub 2019 Mar 21.

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

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http://dx.doi.org/10.1002/ajh.25466DOI Listing
March 2019
3 Reads

Molecular suitability of the chacma baboon in human-targeted Von Willebrand factor directed studies.

J Med Primatol 2019 Mar 20. Epub 2019 Mar 20.

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

Background: Von Willebrand factor (VWF) has a central role in primary haemostasis and is a popular pharmaceutical target in the prevention and treatment of disorders such as acute coronary syndrome and thrombotic thrombocytopenic purpura. We have evaluated numerous possible treatments targeting VWF in the chacma baboon. Unfortunately, no data exist regarding the molecular similarity of the chacma baboon and human VWF protein, resulting in limited translatability of results. Read More

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http://dx.doi.org/10.1111/jmp.12407DOI Listing
March 2019
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Complement in Thrombotic Microangiopathies: Unraveling Ariadne's Thread Into the Labyrinth of Complement Therapeutics.

Front Immunol 2019 27;10:337. Epub 2019 Feb 27.

Division of Biodiagnostic Sciences and Technologies, INRASTES, National Center for Scientific Research Demokritos, Athens, Greece.

Thrombotic microangiopathies (TMAs) are a heterogeneous group of syndromes presenting with a distinct clinical triad: microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. We currently recognize two major entities with distinct pathophysiology: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Beyond them, differential diagnosis also includes TMAs associated with underlying conditions, such as drugs, malignancy, infections, scleroderma-associated renal crisis, systemic lupus erythematosus (SLE), malignant hypertension, transplantation, HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), and disseminated intravascular coagulation (DIC). Read More

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http://dx.doi.org/10.3389/fimmu.2019.00337DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413705PMC
February 2019
2 Reads

Impact of hypertensive emergency and complement rare variants on presentation and outcome of atypical hemolytic uremic syndrome.

Haematologica 2019 Mar 19. Epub 2019 Mar 19.

APHP, Hôpital Européen Georges Pompidou, laboratoire d'Immunologie, INSERM, UMR_S 1138.

Atypical hemolytic uremic syndrome is a prototypic thrombotic microangiopathy attributable to complement dysregulation. Hypertensive emergency, characterized by elevation of systolic (>180mmHg) or diastolic (>120mmHg) blood pressure together with end-organ damage, can cause thrombotic microangiopathy which may mimic atypical hemolytic uremic syndrome. We sought to retrospectively evaluate the clinical, biological and complement genetic characteristics of 76 and 61 atypical hemolytic uremic syndrome patients with and without hypertensive emergency, respectively. Read More

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http://dx.doi.org/10.3324/haematol.2019.216903DOI Listing
March 2019
4 Reads

Thrombotic thrombocytopenic purpura in a patient with metastatic lung cancer.

Med Clin (Barc) 2019 Mar 11. Epub 2019 Mar 11.

Servicio de Hematología, Hospital Virgen de las Nieves, Granada, España.

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http://dx.doi.org/10.1016/j.medcli.2019.01.020DOI Listing
March 2019
1 Read

[Blood Transfusion: a Guide to Clinical Decision Making].

Anasthesiol Intensivmed Notfallmed Schmerzther 2019 Mar 13;54(3):194-205. Epub 2019 Mar 13.

Nowadays, management of hemotherapy is regulated in Germany by the transfusion act and several guidelines while the transfusing physician is responsible for correct implementation at the bedside. Indications for blood products have to be carefully adapted to the patient's current clinical situation and pre-existing diseases have to be considered as well. Today, for most perioperative elective surgeries, evidence-based transfusion thresholds for packed red blood cell concentrates (RBC) have been defined and should be considered. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0593-4390
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http://dx.doi.org/10.1055/a-0593-4390DOI Listing
March 2019
9 Reads

Atypical hemolytic uremic syndrome and complement blockade: established and emerging uses of complement inhibition.

Curr Opin Nephrol Hypertens 2019 May;28(3):278-287

Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Cedars Sinai Medical Center, Los Angeles, California, USA.

Purpose Of Review: Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders.

Recent Findings: In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficiency. Read More

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http://dx.doi.org/10.1097/MNH.0000000000000499DOI Listing
May 2019
3 Reads

Etiology and Outcomes of Thrombotic Microangiopathies.

Clin J Am Soc Nephrol 2019 Apr 12;14(4):557-566. Epub 2019 Mar 12.

Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville,

Background And Objectives: Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown. Read More

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http://dx.doi.org/10.2215/CJN.11470918DOI Listing
April 2019
3 Reads

Elevated Systemic Pentraxin-3 Is Associated With Complement Consumption in the Acute Phase of Thrombotic Microangiopathies.

Front Immunol 2019 25;10:240. Epub 2019 Feb 25.

Research Laboratory, MTA-SE Research Group of Immunology and Hematology, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

Pentraxin-3 (PTX3) and C-reactive protein (CRP) have been shown to regulate complement activation , but their role has not been investigated in complement consumption . Thrombotic microangiopathies (TMA) are often accompanied by complement overactivation and consumption, therefore we analyzed the relation of the systemic pentraxin levels to the complement profile, laboratory parameters and clinical outcome of TMA patients. We determined the PTX3 and CRP levels, complement factor and activation product concentrations in blood samples of 171 subjects with the diagnosis of typical hemolytic uremic syndrome (STEC-HUS) ( = 34), atypical HUS (aHUS) ( = 44), secondary TMA ( = 63), thrombotic thrombocytopenic purpura (TTP) ( = 30) and 69 age-matched healthy individuals. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397851PMC
February 2019
4 Reads

Combination of Fresh Frozen Plasma and Cryosupernatant Plasma for Therapeutic Plasma Exchange in Thrombotic Thrombocytopenic Purpura: A Single Institution Experience.

Biomed Res Int 2019 30;2019:1756109. Epub 2019 Jan 30.

Department of Blood Transfusion, Fujian Medical University Union Hospital, Gulou District, Fuzhou City, Fujian Province 350001, China.

Purpose: To evaluate the impact of a combination of fresh frozen plasma (FFP) and cryosupernatant plasma (CP) as a replacement fluid in therapeutic plasma exchange (TPE) on early therapeutic response and long-term survival of patients with thrombotic thrombocytopenic purpura (TTP).

Materials And Methods: A total of 44 patients with suspected TTP were screened by Bentley and PLASMIC scores. Twenty-seven patients treated with TPE using the FFP and CP combination as the replacement fluid were enrolled and divided into two groups: 11 patients who received TPE with CP-dominant replacement fluid (FFP/CP<1) and 16 patients who received TPE with FFP-dominant replacement fluid (FFP/CP>1). Read More

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http://dx.doi.org/10.1155/2019/1756109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374794PMC
January 2019
3 Reads

[Thrombotic thrombocytopenic purpura complicated with acute erythropoiesis dysfunction: a case report and literature review].

Zhonghua Xue Ye Xue Za Zhi 2019 Feb;40(2):141-143

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

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.02.009DOI Listing
February 2019
1 Read

Treatment of Acute Kidney Injury in Hemolythic Uremic Syndrome (TTP).

Med Arch 2018 Dec;72(6):453-455

Clinic of Hemodialysis, Clinical Center University of Sarajevo, Bosnia and Herzegovina.

Introduction: Plasmapheresis is often used as a therapy in the treatment of thrombotic thrombocytopenic purpura (TTP). TTP is manifested in thrombotic microangiopathy, consumed thrombocytopenia, hemolytic anemia and acute kidney injury with HUS development, neurologic dysfunction, and fever.

Case Report: we will present a case of a patient with acute kidney injury and refractory TTP at the beginning of hospitalization, subsequently manifested in secondary nephrotic syndrome. Read More

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https://www.ejmanager.com/fulltextpdf.php?mno=302643874
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http://dx.doi.org/10.5455/medarh.2018.72.453-455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340615PMC
December 2018
16 Reads

Comparison of the Long-Term Remission of Rituximab and Conventional Treatment for Acquired Thrombotic Thrombocytopenic Purpura: A Systematic Review and Meta-Analysis.

Clin Appl Thromb Hemost 2019 Jan-Dec;25:1076029618825309

1 Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

The current systematic review and meta-analysis aimed to summarize the results of all available studies to compare the efficacies of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura (TTP). Three investigators independently searched studies in the MEDLINE and EMBASE databases published before December 11, 2018. To be included in the meta-analysis, studies needed to be randomized-controlled or cohort studies comparing the efficacies of rituximab and conventional therapy for TTP treatment. Read More

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http://dx.doi.org/10.1177/1076029618825309DOI Listing
February 2019
9 Reads
1.575 Impact Factor

Post-partum acute kidney injury: sorting placental and non-placental thrombotic microangiopathies using the trajectory of biomarkers.

Nephrol Dial Transplant 2019 Feb 25. Epub 2019 Feb 25.

Sorbonne Université, Urgences Néphrologiques et Transplantation Rénale, Assistance Publique-Hôpital de Paris (APHP), Hôpital Tenon, Paris, France.

Background: Among the severe complications of preeclampsia (PE), acute kidney injury (AKI) is problematic if features of thrombotic microangiopathy (TMA) are present. Although a haemolysis enzyme liver low-platelets syndrome is considerably more frequent, it is vital to rule out a flare of atypical haemolytic and uraemic syndrome (aHUS). Our objective was to improve differential diagnosis procedures in post-partum AKI. Read More

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http://dx.doi.org/10.1093/ndt/gfz025DOI Listing
February 2019
6 Reads

The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: Key findings at enrolment until 2017.

Haematologica 2019 Feb 21. Epub 2019 Feb 21.

Inselspital, Bern University Hospital;

Congenital thrombotic thrombocytopenic purpura is an autosomal recessive inherited disease with a clinically heterogeneous course and an incompletely understood genotype-phenotype correlation. In 2006, the Hereditary TTP Registry started recruitment with the purpose of improving the understanding of this ultra-rare disease. The objective of this study is to present characteristics of the cohort until the end of 2017 and to explore the relationship between overt disease onset and ADAMTS13 activity with emphasis on the recurring ADAMTS13 c. Read More

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http://dx.doi.org/10.3324/haematol.2019.216796DOI Listing
February 2019
1 Read

Spontaneous Tumor Lysis Syndrome as Presenting Sign of Metastatic Prostate Cancer.

Cureus 2018 Dec 8;10(12):e3706. Epub 2018 Dec 8.

Hematology, Thomas Jefferson University Hospital, Philadelphia, USA.

Spontaneous tumor lysis syndrome is an exceedingly rare manifestation of metastatic prostate cancer. It can masquerade as thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (HUS). These entities present with microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, and improve with the initiation of plasma exchange and steroids. Read More

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http://dx.doi.org/10.7759/cureus.3706DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373879PMC
December 2018

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
6 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
2 Reads

Characterization and treatment of congenital thrombotic thrombocytopenic purpura.

Blood 2019 Apr 15;133(15):1644-1651. Epub 2019 Feb 15.

Department of Haematology, University College London Hospital (UCLH), London, United Kingdom.

Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra-rare thrombomicroangiopathy caused by an inherited deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). There are limited data on genotype-phenotype correlation; there is no consensus on treatment. We reviewed the largest cohort of cTTP cases, diagnosed in the United Kingdom, 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
April 2019
10 Reads

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
5 Reads

Risk of diagnostic delay in congenital thrombotic thrombocytopenic purpura.

J Thromb Haemost 2019 Apr 6;17(4):666-669. Epub 2019 Mar 6.

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

Essentials Congenital thrombotic thrombocytopenic purpura (cTTP) is a very rare thrombotic microangiopathy. Its rarity and great phenotype heterogeneity may account for misdiagnosis. We report the history of a middle-aged woman with cTTP, misdiagnosed until adulthood. Read More

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

Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome.

Curr Opin Rheumatol 2019 May;31(3):231-240

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
May 2019
2 Reads

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
10 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
4 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
10 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 Mar 31;30(1):66-78. Epub 2019 Jan 31.

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
March 2019
4 Reads

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
3 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
2 Reads

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
3 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
6 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
14 Reads