3,116 results match your criteria Hemophilia Acquired


Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients.

Blood Coagul Fibrinolysis 2019 Apr;30(3):127-132

Hospital General Universitario, Alicante, Spain.

: We hypothesized that inhibitor specificity may predict the outcome of antifactor VIII autoantibodies eradication treatment in acquired hemophilia A. Our objective was to analyze the association between factor VIII domains recognized by inhibitors and outcome of the immunosuppressive therapies (ISTs) in a prospective, observational study. 16 patients were recruited. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000804DOI Listing
April 2019
2 Reads

A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia.

Case Rep Hematol 2019 27;2019:8612031. Epub 2019 Feb 27.

Department of Hematology and Oncology, Kita-Harima Medical Center, Hyogo, Japan.

A 67-year-old male, with a known diagnosis of myelodysplastic syndromes with multilineage dysplasia (MDS-MLD) was admitted to our hospital with a primary complaint of subcutaneous bleeding in his left thigh. Laboratory data showed anaemia and prolongation of activated partial thromboplastin time (85.8 s, normal range 24-39 s) without thrombocytopenia. Read More

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http://dx.doi.org/10.1155/2019/8612031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6415290PMC
February 2019
4 Reads

Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases.

Reumatismo 2019 Apr 1;71(1):37-41. Epub 2019 Apr 1.

Department of Internal Medicine, Pordenone General Hospital.

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported. Read More

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https://www.reumatismo.org/index.php/reuma/article/view/1041
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http://dx.doi.org/10.4081/reumatismo.2019.1041DOI Listing
April 2019
6 Reads

Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan.

Diabetes Ther 2019 Mar 29. Epub 2019 Mar 29.

Department of Hematology and Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Chuo-Ku, Fukuoka, 810-8563, Japan.

Herein we describe four patients with acquired hemophilia A (AHA) caused by factor VIII (FVIII) inhibitor and histories of dipeptidyl peptidase-4 inhibitor (DPP4-I) treatment for diabetes mellitus (DM). Drug exposure can cause a breakdown of immune tolerance to FVIII associated with CD4 T cells, resulting in the induction of autoantibodies against FVIII. In patient 1 in the present series, FVIII inhibitor disappeared after DPP4-I treatment. Read More

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http://dx.doi.org/10.1007/s13300-019-0609-3DOI Listing
March 2019
1 Read

[The issue of use parallelism factor method and index of circulating anticoagulant in diagnostic algorithm deficity of coagulation factors.]

Klin Lab Diagn 2019 ;64(2):83-88

CJS Company "Company Galen", 109044, Moscow, Russia.

The paper presents the comparative use of algorithms to identify the causes of deficiency of coagulation factors in patients with a prolonged APTT, including the definition of the index of circulating anticoagulant (ICA) and the factor-parallelism (FP) method. The results obtained in children with hereditary hemophilia and adults with acquired hemophilia. It is shown that ICA is an effective method for pre-selection of patients with hereditary hemophilia if you suspect an inhibitor to subsequent confirmation test Bethesda. Read More

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http://dx.doi.org/10.18821/0869-2084-2019-64-2-83-88DOI Listing
January 2019

[PREGNANCY RELATED ACQUIRED HEMOPHILIA A].

Harefuah 2019 Mar;158(3):184-186

Hematology Institute and Blood Bank.

Introduction: Acquired hemophilia A is a rare disease. The incidence has been estimated to be 1.3-1. Read More

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March 2019
1 Read

[ACQUIRED HEMOPHILIA A AND THE TIMING OF IMMUNOMODULATORY THERAPY].

Harefuah 2019 Mar;158(3):165-167

Thrombosis and Hemostasis Unit, Rambam Health Care Campus, Haifa, Israel.

Introduction: Acquired hemophilia A is an autoimmune disease affecting men and women equally and is idiopathic in 50% of the cases. As the mortality rate reaches 50%, prompt diagnosis and treatment are needed. Diagnosis is made in a patient with a bleeding manifestation and prolonged PTT (partial thromboplastin time) that is not corrected in a mixing study with normal plasma. Read More

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March 2019
4 Reads

[Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review].

Rev Med Interne 2019 Mar 20. Epub 2019 Mar 20.

Service d'hématologie, CHU Nîmes, 30029 Nîmes, France.

Introduction: Autoimmune acquired haemophilia is a rare autoimmune disease. The purpose of immunosuppressive therapy is to stop the production of autoantibodies that inhibit clotting factors VIII or IX. A corticosteroids-cyclophosphamide combination is recommanded as first-line therapy. Read More

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http://dx.doi.org/10.1016/j.revmed.2019.02.008DOI Listing
March 2019
1 Read

Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report.

Ann Biol Clin (Paris) 2019 Apr;77(2):179-183

Service d'hématologie-immunologie-transfusion, Hôpitaux Universitaires Paris Ile-de-France Ouest, CHU Ambroise Paré, Boulogne-Billancourt, France.

Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disorder caused by circulating autoantibodies directed against factor (F) VIII. Apart from idiopathic cases, AHA is associated with autoimmune diseases, cancers, use of medications, pregnancy and the post-partum period. We report the case of a 78-year-old a male patient presenting with symptoms of a hematoma after a fall three days previously. Read More

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http://dx.doi.org/10.1684/abc.2018.1405DOI Listing
April 2019
2 Reads

Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report.

Blood Coagul Fibrinolysis 2019 Apr;30(3):120-126

Division of Hematology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels.

: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the development of autoantibodies against clotting factor VIII. Although the cause of this disorder remains obscure, it is often linked to malignancies, drug administration, autoimmune diseases and pregnancy. In pregnancy-associated AHA, hemorrhagic symptoms usually present 1-4 months peripartum, however they may occur up to 1-year postpartum. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000799DOI Listing
April 2019
1 Read

Clinical and Laboratory Features of Patients with Acquired Thrombotic Thrombocytopaenic Purpura: Fourteen Years of the Milan TTP Registry.

Thromb Haemost 2019 Mar 12. Epub 2019 Mar 12.

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

Acquired thrombotic thrombocytopaenic purpura (TTP) is a rare thrombotic microangiopathy caused by the immune-mediated severe deficiency of ADAMTS13. We hereby report the demographic and disease-related data of acquired TTP patients recorded in the Milan TTP Registry (www.ttpdatabase. Read More

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http://dx.doi.org/10.1055/s-0039-1679907DOI Listing
March 2019
2 Reads

Hypercoagulability and the risk of recurrence in young women with myocardial infarction or ischaemic stroke: a cohort study.

BMC Cardiovasc Disord 2019 Mar 7;19(1):55. Epub 2019 Mar 7.

Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.

Background: We aimed to investigate the role of hypercoagulability on the risk of lifetime cardiovascular recurrences after myocardial infarction or ischaemic stroke.

Methods: Young women (< 50 years) with either myocardial infarction (n = 197) or ischaemic stroke (n = 107) were followed between 1995 and 2012 in the RATIO follow-up study. To determine whether hypercoagulability affects the risk or recurrence, a coagulation score based on acquired and inherited markers was compiled and used in a quartile analysis. Read More

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https://bmccardiovascdisord.biomedcentral.com/articles/10.11
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http://dx.doi.org/10.1186/s12872-019-1040-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407236PMC
March 2019
2 Reads

Acquired haemophilia A and concomitant factor XIII consumption.

Haemophilia 2019 Mar 7. Epub 2019 Mar 7.

Department of Laboratory Medicine and Pathobiology, St. Michael's Hospital, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1111/hae.13690DOI Listing
March 2019
1 Read

Summary and Review of the Abstracts on Hemostasis/Thrombosis Presented at Haematocon 2018, Kochi.

Authors:
Prashant Sharma

Indian J Hematol Blood Transfus 2019 Jan 24;35(1):32-36. Epub 2019 Jan 24.

Department of Hematology, Postgraduate Institute of Medical Education and Research, Level 5, Research Block A, Sector 12, Chandigarh, 160012 India.

The hemostatic pathways involve a complex interplay of a vast array of inherited and acquired factors. Derangements of these pathways lead to several disorders that are complex and challenging not just for laboratory and clinical hematologists but also of concern to public health officials and policy makers. The past decade has seen an increased focus on research in this area with several recent meetings focusing on the diagnosis and management of persons afflicted with these disorders. Read More

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http://link.springer.com/10.1007/s12288-019-01076-y
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http://dx.doi.org/10.1007/s12288-019-01076-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369065PMC
January 2019
8 Reads
0.234 Impact Factor

Determining the cut-off value of the APTT mixing test for factor VIII inhibitor: reply.

Clin Chem Lab Med 2019 Apr;57(5):e91-e93

Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, 1757-Kanazawa, Ishikari-Tobetsu, Hokkaido 061-0293, Japan.

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http://dx.doi.org/10.1515/cclm-2019-0093DOI Listing
April 2019
5 Reads

The use of new procoagulants in blunt and penetrating trauma.

Curr Opin Anaesthesiol 2019 Apr;32(2):200-205

Katharine Dormandy Haemophilia and Thrombosis Centre, Dept. of Haematology, Royal Free Hospital, London, UK.

Purpose Of Review: Uncontrolled bleeding in trauma secondary to a combination of surgical bleeding and trauma-induced complex coagulopathy is a leading cause of death. Prothrombin complex concentrates (PCCs), recombinant activated factor seven (rFVIIa) and recombinant human prothrombin act as procoagulants by increasing thrombin generation and fibrinogen concentrate aids stable clot formation. This review summarizes the current evidence for procoagulant use in the management of bleeding in trauma, and data and evidence gaps for routine clinical use. Read More

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http://dx.doi.org/10.1097/ACO.0000000000000696DOI Listing
April 2019
15 Reads

Acquired Hemophilia A: A Case Report.

J Pharm Pract 2019 Feb 6:897190019826474. Epub 2019 Feb 6.

3 Surgical Intensive Care Unit Attending Physician, Department of Surgery, NYU Winthrop Hospital, Mineola, NY, USA.

Hemophilia A, also known as factor VIII deficiency, is a rare disorder caused by an insufficient level of factor VIII, an essential clotting protein. Hemophilia A can be inherited or acquired. Inherited hemophilia A is caused by a mutation to the factor VIII gene on the X chromosome, which is commonly passed down from parents to children. Read More

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http://dx.doi.org/10.1177/0897190019826474DOI Listing
February 2019
7 Reads

Inhibitor eradication in refractory acquired hemophilia with lenalidomide.

Ann Hematol 2019 Feb 1. Epub 2019 Feb 1.

Division of Hemostaseology, University Hospital Leipzig, Liebigstr. 20, 04103, Leipzig, Germany.

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http://dx.doi.org/10.1007/s00277-019-03620-2DOI Listing
February 2019
1 Read

Literature review and clinical observation of acquired idiopathic hemophilia with a new missense mutation in the factor VIII gene (His2026Arg).

Ter Arkh 2018 Aug;90(7):118-122

National medical research center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.

The article provides review of possible mechanisms of inhibitor coagulopathies, in particular of acquired hemophilia A. This pathology is an extremely rare disease occurring in 1-2 cases in 1 million per year. In the present study we provide data for two clinical cases of hemophilia A in women. Read More

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http://dx.doi.org/10.26442/terarkh2018907118-122DOI Listing
August 2018
2 Reads

Relapse pattern and long-term outcomes in subjects with acquired haemophilia A.

Haemophilia 2019 Mar 29;25(2):252-257. Epub 2019 Jan 29.

Division of Hematology-Oncology, Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada.

Introduction: Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy.

Aim: Long-term follow-up data from the Quebec Reference Centre for Inhibitors (QRCI) were analysed to identify factors predictive of AHA relapse and the influence of relapse on survival. Read More

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http://doi.wiley.com/10.1111/hae.13685
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http://dx.doi.org/10.1111/hae.13685DOI Listing
March 2019
10 Reads

Expecting the unexpected: Acquired haemophilia A in a patient with homozygous factor V deficiency.

Haemophilia 2019 Mar 28;25(2):e101-e103. Epub 2019 Jan 28.

Department of Haematology, Manchester Royal Infirmary, Manchester University Hospitals NHS Foundation Trust, Manchester, UK.

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http://dx.doi.org/10.1111/hae.13669DOI Listing
March 2019
2 Reads

Thrombotic complications in adult patients with severe single coagulation factor or platelet defects - an overview.

Expert Rev Hematol 2019 Feb 28;12(2):119-128. Epub 2019 Jan 28.

a Section for Hematology, Department of Clinical Science , University of Bergen , Bergen , Norway.

Introduction: Even though thrombotic events are rare in patients with coagulation deficiencies, several cases of both arterial and venous thromboses have been reported in patients with single coagulation factor defects and platelet defects. Thromboses have been described both in hemophilia A and B, von Willebrand disease as well as in many other rare congenital coagulation factor and platelet defects. Thromboses may also occur in patients with acquired hemophilia and in patients with severe thrombocytopenia due to hematological malignancies or intensive chemotherapy. Read More

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http://dx.doi.org/10.1080/17474086.2019.1570126DOI Listing
February 2019
5 Reads

Spontaneous Spinal Epidural Haematoma Secondary to Autoimmune Acquired Haemophilia.

Case Rep Neurol 2018 Sep-Dec;10(3):353-356. Epub 2018 Dec 13.

Department of Neurosurgery, Birmingham University Hospital, Birmingham, United Kingdom.

Spontaneous spinal epidural haematoma is a rare entity associated with high morbidity. Although there are previous reports of spinal haematoma secondary to X-linked genetic haemophilia, there are no such cases secondary to acquired autoimmune haemophilia. We report the case of a 71-year-old patient who presented with sudden quadriplegia secondary to cervical (C2 to T1) epidural haematoma as a result of undiagnosed autoimmune acquired haemophilia A. Read More

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http://dx.doi.org/10.1159/000495028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341357PMC
December 2018
9 Reads

Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab.

Int J Hematol 2019 Jan 24. Epub 2019 Jan 24.

The Emergency and Critical Care Center, Mie University Hospital, 2-174, Edobashi, Tsu, Mie, 514-8507, Japan.

TAFRO syndrome and acquired hemophilia A (AHA) are rare, life-threatening diseases; however, the relationship between these two diseases is unknown. A 25-year-old man was transferred to our hospital because of bleeding tendency accompanied by multiple organ failure with generalized edema, massive pleural effusion, and ascites. He was diagnosed with AHA. Read More

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http://dx.doi.org/10.1007/s12185-019-02604-2DOI Listing
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
14 Reads

Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report.

Medicine (Baltimore) 2019 Jan;98(3):e14119

Department of Hematology, First Affiliated Hospital of Zhejiang, Chinese Medical University, Hangzhou, China.

Rationale: Acquired hemophilia A is a rare hemorrhagic disease in which the body produces specific antibodies that attack factor VIII, resulting in bleeding that is mainly mucocutaneous and associated with soft tissue and the gastrointestinal system. Approximately 50% of this disease derives from basic diseases, such as autoimmune diseases, cancer, and pregnancy.

Patient Concerns: We report a 35-year-old postpartum female with acquired hemophilia A who initially presented with pleural hemorrhage. Read More

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http://dx.doi.org/10.1097/MD.0000000000014119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370013PMC
January 2019
8 Reads

Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review.

Exp Ther Med 2019 Jan 29;17(1):633-638. Epub 2018 Nov 29.

Department of Emergency, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China.

Acquired hemophilia A (AHA) is a rare antibody-mediated condition in which autoantibodies form against a coagulation factor, most commonly factor VIII (FVIII), causing severe coagulopathy. Here the present report presents a case of AHA in a 35-year-old postpartum woman with continuous polyserous bloody effusions who was admitted to the First Affiliated Hospital of Zhejiang Chinese Medical University (Hangzhou, China) in October 2017 without a history of trauma, anticoagulation treatment or coagulopathy. At presentation, the patient's hemoglobin level was low (70 g/l; normal range: 115-150 g/l) g/l, blood pressure was 89/58 mmHg (normal range, 90-140/60-90 mmHg), and activated partial thromboplastin time was 68. Read More

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http://dx.doi.org/10.3892/etm.2018.7031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307365PMC
January 2019
4 Reads

The use of new procoagulants in blunt and penetrating trauma.

Curr Opin Anaesthesiol 2019 Jan 11. Epub 2019 Jan 11.

Katharine Dormandy Haemophilia and Thrombosis Centre, Dept. of Haematology, Royal Free Hospital, London, UK.

Purpose Of Review: Uncontrolled bleeding in trauma secondary to a combination of surgical bleeding and trauma-induced complex coagulopathy is a leading cause of death. Prothrombin complex concentrates (PCCs), recombinant activated factor seven (rFVIIa) and recombinant human prothrombin act as procoagulants by increasing thrombin generation and fibrinogen concentrate aids stable clot formation. This review summarizes current evidence for procoagulant use in the management of bleeding in trauma, and data and evidence gaps for routine clinical use. Read More

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http://dx.doi.org/10.1097/ACO.0000000000000696DOI Listing
January 2019
2 Reads

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

J Thromb Haemost 2019 Mar 25;17(3):492-498. Epub 2019 Feb 25.

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

Essentials Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. Surgery is a possible trigger of acute TTP episodes and no guidelines are available. Six patients with severe ADAMTS-13 deficiency during remission underwent elective surgery. 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
March 2019
21 Reads

A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury.

Case Rep Dent 2018 9;2018:7185263. Epub 2018 Dec 9.

Department of Maxillofacial Surgery, Tokyo Medical and Dental University, Tokyo, Japan.

Background: Acquired hemophilia A (AHA) is a rare disorder which results from the presence of autoantibodies against blood coagulation factor VIII. The initial diagnosis is based on the detection of an isolated prolongation of the activated partial thromboplastin time (aPTT) with negative personal and family history of bleeding disorder. Definitive diagnosis is the identification of reduced FVIII levels with evidence of FVIII neutralizing activity. Read More

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https://www.hindawi.com/journals/crid/2018/7185263/
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http://dx.doi.org/10.1155/2018/7185263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6304843PMC
December 2018
13 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
12 Reads

Current Understanding of How Extracorporeal Membrane Oxygenators Activate Haemostasis and Other Blood Components.

Front Med (Lausanne) 2018 12;5:352. Epub 2018 Dec 12.

Thrombosis and Haemophilia Centre, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom.

Extracorporeal membrane oxygenators are used in critical care for the management of severe respiratory and cardiac failure. Activation of the coagulation system is initiated by the exposure of blood to synthetic surfaces and the shear stresses of the circuit, especially from device pumps. Initial fibrinogen deposition and subsequent activation of coagulation factors and complement allow platelets and leucocytes to adhere to oxygenator surfaces and enhance thrombin generation. Read More

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https://www.frontiersin.org/article/10.3389/fmed.2018.00352/
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http://dx.doi.org/10.3389/fmed.2018.00352DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299009PMC
December 2018
16 Reads

Treatment of acquired haemophilia with recombinant porcine factor VIII: A successful strategy in a patient with unstable angina.

Haemophilia 2019 Mar 3;25(2):e94-e97. Epub 2019 Jan 3.

Department of Haematology, Leeds Comprehensive Care Haemophilia Centre, St James's University Hospital, Leeds, UK.

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http://dx.doi.org/10.1111/hae.13644DOI Listing
March 2019
27 Reads

Acquired Hemophilia A Presenting as Intramuscular Hematoma.

J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709618817572. Epub 2018 Dec 6.

MedStar Washington Hospital Center, Washington, DC, USA.

Acquired hemophilia A poses a clinical and diagnostic challenge. Although rare, it is still the most common acquired factor deficiency. We present a case of acquired hemophilia A diagnosed in a 71-year-old female who presented with a right thigh hematoma of acute onset. Read More

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http://dx.doi.org/10.1177/2324709618817572DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299309PMC
December 2018
5 Reads

[A Pediatric Case of Acquired Hemophilia A: The Usefulness of the Activated Partial Thromboplastin Time (APTT) Cross-Mixing Test for Early Diagnosis].

J UOEH 2018;40(4):331-337

Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan.

Acquired hemophilia A (AHA), a bleeding disorder caused by autoantibodies against FVIII, has the potential for life-threatening bleeding. The annual onset rate is said to be one in 4 million people, but diagnosis examples increase in adults because a disorder concept penetrated. AHA is quite rare in children, with an incidence rate of 0. Read More

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http://dx.doi.org/10.7888/juoeh.40.331DOI Listing
March 2019
2 Reads

Acquired haemophilia presenting with tongue swelling and dysphagia.

BMJ Case Rep 2018 Nov 28;11(1). Epub 2018 Nov 28.

Otolaryngology, Head and Neck Surgery, Royal Cornwall Hospitals NHS Trust, Truro, UK.

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http://dx.doi.org/10.1136/bcr-2018-228282DOI Listing
November 2018
1 Read

[Systemic lupus erythematosus with acquired hemophilia A: a case report].

Authors:
F Yang Y S Zhou Y Jia

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1108-1111

Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. Read More

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December 2018
22 Reads

Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A.

Haemophilia 2019 Jan 18;25(1):e59-e65. Epub 2018 Dec 18.

Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.

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http://doi.wiley.com/10.1111/hae.13658
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http://dx.doi.org/10.1111/hae.13658DOI Listing
January 2019
11 Reads

Low dose of aPCC after the initial treatment in acquired haemophilia A is useful to reduce bleeding relapses: Data from the FAIR registry.

Thromb Res 2019 02 6;174:24-26. Epub 2018 Dec 6.

Haemophilia and Thrombosis Centre, S.Giovanni Bosco Hospital of Naples, Italy.

Background: Bypassing agents are the first line therapy in patients with acquired haemophilia A (AHA). Activated prothrombin complex concentrate (aPCC) proved to be effective as initial treatment, but 20% of patients (pts) had relapses. aPCC as short-term prophylaxis to reduce subsequent bleeds is still not clear. Read More

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http://dx.doi.org/10.1016/j.thromres.2018.12.006DOI Listing
February 2019
2 Reads

Hemizygosity for the gene encoding glycoprotein Ibβ is not responsible for macrothrombocytopenia and bleeding in patients with 22q11 deletion syndrome.

J Thromb Haemost 2019 Feb 22;17(2):295-305. Epub 2019 Jan 22.

Blood Research Institute, BloodCenter of Wisconsin - part of Versiti, Milwaukee, WI, USA.

Essentials How thrombocytopenia relates to bleeding in 22q11 deletion syndrome (22q11DS) is not clear. Bleeding severity, platelet count and volume, and GPIBB were examined in patients with 22q11DS. Macrothrombocytopenia and bleeding typified imperfectly overlapping subsets of 22q11DS patients. Read More

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http://dx.doi.org/10.1111/jth.14357DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410711PMC
February 2019

Acquired haemophilia A: a rare cause of postpartum haemorrhage.

N Z Med J 2018 Dec 14;131(1487):111-115. Epub 2018 Dec 14.

Obstetrician and Gynaecologist, Department of Obstetrics and Gynaecology, North Shore Hospital, Auckland.

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December 2018
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Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A.

Authors:
Paul Knöbl

Drugs 2018 Dec;78(18):1861-1872

Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies inhibiting the function of coagulation factor VIII. It is characterized by spontaneous bleeding in patients with no previous family or personal history of bleeding. Although several large registries have collected clinical data on AHA, limited information is available on the optimal management of AHA because controlled clinical trials are lacking. Read More

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http://link.springer.com/10.1007/s40265-018-1027-y
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http://dx.doi.org/10.1007/s40265-018-1027-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6294818PMC
December 2018
11 Reads

Management of Acquired Hemophilia A in Elderly Patients.

Case Rep Hematol 2018 13;2018:6757345. Epub 2018 Nov 13.

Division of Hematology, Takasago-Seibu Hospital, Takasago 676-0812, Japan.

This report describes six elderly patients with acquired hemophilia A (AHA), including four individuals aged ≥90 years. Bleeding symptoms were subcutaneous or intramuscular hemorrhage (=4), hematuria (=1), and hemorrhagic shock after tooth extraction (=1). Factor VIII (FVIII) activity ranged from <1. Read More

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http://dx.doi.org/10.1155/2018/6757345DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260550PMC
November 2018
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Exploratory evaluation of pharmacodynamics, pharmacokinetics and safety of rivaroxaban in children and adolescents: an EINSTEIN-Jr phase I study.

Thromb J 2018 4;16:31. Epub 2018 Dec 4.

11Clinical Development, Bayer AG, Pharmaceuticals, Wuppertal, Germany.

Background: The EINSTEIN-Jr program will evaluate rivaroxaban for the treatment of venous thromboembolism (VTE) in children, targeting exposures similar to the 20 mg once-daily dose for adults.

Methods: This was a multinational, single-dose, open-label, phase I study to describe the pharmacodynamics (PD), pharmacokinetics (PK) and safety of a single bodyweight-adjusted rivaroxaban dose in children aged 0.5-18 years. Read More

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http://dx.doi.org/10.1186/s12959-018-0186-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6278122PMC
December 2018
26 Reads

Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series.

Semin Hematol 2018 10 21;55(4):197-201. Epub 2018 Feb 21.

Faculty of Medicine, University of Reims Champagne-Ardenne, Reims, France; Department of Research and Public Health, University Hospitals of Reims, Robert Debré Hospital, Reims, France.

Acquired hemophilia A (AHA) is a rare disease that requires urgent management. Currently, there is no consensus regarding optimal management in aged people. This systematic review aimed to describe diagnosis, clinical features, management, and endpoints in population aged 65 years or over with AHA. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.02.004DOI Listing
October 2018

Therapeutic expression of human clotting factors IX and X following adeno-associated viral vector-mediated intrauterine gene transfer in early-gestation fetal macaques.

FASEB J 2019 Mar 5;33(3):3954-3967. Epub 2018 Dec 5.

Obstetrics and Gynaecology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore.

Adeno-associated viral vectors (AAVs) achieve stable therapeutic expression without long-term toxicity in adults with hemophilia. To avert irreversible complications in congenital disorders producing early pathogenesis, safety and efficacy of AAV-intrauterine gene transfer (IUGT) requires assessment. We therefore performed IUGT of AAV5 or -8 with liver-specific promoter-1 encoding either human coagulation factors IX (hFIX) or X (hFX) into Macaca fascicularis fetuses at ∼0. Read More

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https://www.fasebj.org/doi/10.1096/fj.201801391R
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http://dx.doi.org/10.1096/fj.201801391RDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6404563PMC
March 2019
18 Reads
5.043 Impact Factor

Acquired haemophilia A as a secondary autoimmune disease after alemtuzumab treatment in multiple sclerosis: A case report.

Mult Scler Relat Disord 2019 Jan 29;27:403-405. Epub 2018 Nov 29.

Department of Neurology, Division of Neuroscience, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy.

Alemtuzumab is a highly effective monoclonal antibody for the treatment of multiple sclerosis (MS). During the immune reconstitution following the use of this treatment severe secondary autoimmune diseases (SADs) can develop. We present the case of a patient affected by active MS who failed to achieve disease control with several disease-modifying drugs and was thereafter successfully treated with alemtuzumab, obtaining no evidence of disease activity and a high quality of life. Read More

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http://dx.doi.org/10.1016/j.msard.2018.11.029DOI Listing
January 2019
3 Reads