3,084 results match your criteria Hemophilia Acquired


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

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

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

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

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

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

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http://doi.wiley.com/10.1111/jth.14381
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http://dx.doi.org/10.1111/jth.14381DOI Listing
January 2019
2 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
1 Read

Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.

N Engl J Med 2019 Jan 9. 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

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
4 Reads

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

Haemophilia 2019 Jan 3. 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
January 2019

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

[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
January 2018
1 Read

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

[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
5 Reads

Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A.

Haemophilia 2018 Dec 18. 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
December 2018
9 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 2018 Dec 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
December 2018
1 Read

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

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
5 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

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
1 Read

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

Semin Hematol 2018 Oct 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 2018 Dec 5:fj201801391R. 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
December 2018
6 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
2 Reads

Acquired hemophilia A in solid cancer: Two case reports and review of the literature.

World J Clin Cases 2018 Nov;6(14):781-785

Department of Internal Medicine, Health Sciences University of Hokkaido, Toubetsu 0610293, Japan.

Acquired hemophilia A (AHA) is a rare, hemorrhagic autoimmune disease, whose pathogenesis involves reduced coagulation factor VIII (FVIII) activity related to the appearance of inhibitors against FVIII. Common etiological factors include autoimmune diseases, malignancy, and pregnancy. We report two cases of AHA in solid cancer. Read More

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http://dx.doi.org/10.12998/wjcc.v6.i14.781DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6264993PMC
November 2018
1 Read

aPCC vs. rFVIIa for the treatment of bleeding in patients with acquired haemophilia - a cost-effectiveness model.

Vox Sang 2018 Nov 30. Epub 2018 Nov 30.

Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

Background: Acquired haemophilia A (AHA) is an autoimmune bleeding disorder with significant morbidity and mortality. Bleeding AHA patients with high titre inhibitors can be treated with either activated prothrombin complex concentrate (aPCC) or recombinant activated factor VII (rFVIIa). Given that both replacement therapies have inherent benefits and limitations, a cost-effectiveness analysis (CEA) was performed in this population to compare rFVIIa with aPCC. Read More

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http://dx.doi.org/10.1111/vox.12726DOI Listing
November 2018
2 Reads

Management of hydrocephalus in infants with severe hemophilia A: report of 2 cases.

J Neurosurg Pediatr 2018 Oct 1:1-5. Epub 2018 Oct 1.

1Department of Neurosurgery.

The authors report on the clinical course of two infants with severe hemophilia A (HA) and concomitant progressive hydrocephalus that required management with a ventriculoperitoneal shunt. The first child, with known HA, presented with a spontaneous intracranial hemorrhage and acquired hydrocephalus. He underwent cerebrospinal fluid diversion with a temporary external ventricular drain, followed by placement of a ventriculoperitoneal shunt. Read More

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https://thejns.org/view/journals/j-neurosurg-pediatr/aop/art
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http://dx.doi.org/10.3171/2018.8.PEDS18409DOI Listing
October 2018
6 Reads

Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A.

Case Rep Hematol 2018 24;2018:6208597. Epub 2018 Oct 24.

Senior Registrar, Department of Pathology, Faculty of Medical Sciences, University of Sri Jayewardenepura, Nugegoda, Sri Lanka.

Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. Read More

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https://www.hindawi.com/journals/crihem/2018/6208597/
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http://dx.doi.org/10.1155/2018/6208597DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6220402PMC
October 2018
9 Reads

Bleeding Disorders in Adolescents with Heavy Menstrual Bleeding: The Queensland Statewide Paediatric and Adolescent Gynaecology Service.

J Pediatr Adolesc Gynecol 2018 Nov 22. Epub 2018 Nov 22.

Statewide Paediatric and Adolescent Gynaecology Service, Royal Brisbane and Women's Hospital and Lady Cilento Children's Hospital, Brisbane, Queensland, Australia; University of Queensland, Faculty of Medicine, Brisbane, Queensland, Australia.

Study Objective: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.11.005DOI Listing
November 2018
1 Read

Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus.

Haemophilia 2018 Nov 8. Epub 2018 Nov 8.

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

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http://doi.wiley.com/10.1111/hae.13626
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http://dx.doi.org/10.1111/hae.13626DOI Listing
November 2018
9 Reads

Management of acquired hemophilia A: Review of current evidence.

Transfus Apher Sci 2018 Dec 30;57(6):717-720. Epub 2018 Oct 30.

Division of Hematology/Oncology, Hôpital Maisonneuve-Rosemont, Department of Medicine, Université de Montréal, Montréal, Qc, Canada. Electronic address:

Acquired hemophilia A (AHA) is a rare acquired bleeding disorder caused by autoantibodies against autologous factor VIII (FVIII). It is a disease that most commonly affects the elderly, but it has been described in children and during the post-partum period. It is idiopathic in 50% of cases and is associated with autoimmune disease, malignancy, pregnancy, infection or certain medications in the other 50%. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14730502183043
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http://dx.doi.org/10.1016/j.transci.2018.10.011DOI Listing
December 2018
14 Reads

Determining the cutoff value of the APTT mixing test for factor VIII inhibitor.

Clin Chem Lab Med 2018 Oct 23. Epub 2018 Oct 23.

Department of Clinical Laboratory, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, P.R. China, Phone: 00862783665469, Fax: 00862783663639.

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http://www.degruyter.com/view/j/cclm.ahead-of-print/cclm-201
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http://dx.doi.org/10.1515/cclm-2018-0794DOI Listing
October 2018
7 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

An update on the role of RANKL-RANK/osteoprotegerin and WNT-ß-catenin signaling pathways in pediatric diseases.

World J Pediatr 2018 Oct 20. Epub 2018 Oct 20.

Pediatric Section, Department of Biomedical Sciences and Human Oncology, University "A. Moro" of Bari, Piazza G. Cesare 11, 70124, Bari, Italy.

Background: Bone remodeling is a lifelong process due to the balanced activity of osteoclasts (OCs), the bone-reabsorbing cells, and osteoblasts (OBs), and the bone-forming cells. This equilibrium is regulated by numerous cytokines, but it has been largely demonstrated that the RANK/RANKL/osteoprotegerin and Wnt/β-catenin pathways play a key role in the control of osteoclastogenesis and osteoblastogenesis, respectively. The pro-osteoblastogenic activity of the Wnt/β-catenin can be inhibited by sclerostin and Dickkopf-1 (DKK-1). Read More

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http://link.springer.com/10.1007/s12519-018-0198-7
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http://dx.doi.org/10.1007/s12519-018-0198-7DOI Listing
October 2018
5 Reads

Development of acquired haemophilia A in a patient treated with alemtuzumab for multiple sclerosis.

BMJ Case Rep 2018 Oct 17;2018. Epub 2018 Oct 17.

Department of Haematology, Townsville Hospital, Townsville, Queensland, Australia.

This case illustrates a 36-year-old man who presented with a factor VIII (FVIII) inhibitor (acquired haemophilia A) with cutaneous bleeding and a significant thigh haematoma. No traditional risk factors for the development of a FVIII inhibitor were identified. However, previous treatment with alemtuzumab for multiple sclerosis was noted in the patient's history. Read More

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http://dx.doi.org/10.1136/bcr-2018-226588DOI Listing
October 2018
7 Reads

Prohemostatic Activity of Factor X in Combination With Activated Factor VII in Dilutional Coagulopathy.

Anesth Analg 2018 Oct 12. Epub 2018 Oct 12.

Department of Anesthesiology, University of Maryland School of Medicine, Baltimore, Maryland.

Background: Recombinant activated factor VII (rFVIIa) concentrate reduces allogeneic blood transfusions, but it may increase thromboembolic complications in complex cardiac surgery. The mixture of activated factor VII (FVIIa) and factor X (FX) (FVIIa/FX) (FVIIa:FX = 1:10) is a novel bypassing agent for hemophilia patients. We hypothesized that the combination of FX and FVIIa could improve thrombin generation (TG) in acquired multifactorial coagulation defects such as seen in cardiac surgery and conducted in vitro evaluation of FVIIa/FX in parallel with other coagulation factor concentrates using in vitro and in vivo diluted plasma samples. Read More

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http://dx.doi.org/10.1213/ANE.0000000000003858DOI Listing
October 2018
8 Reads

Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents.

Hematol Rep 2018 Sep 5;10(3):7453. Epub 2018 Sep 5.

Department of Internal Medicine, Health Sciences University of Hokkaido, Japan.

A 74-years-old male who was a smoker and received treatment for hypertension, dyslipidemia, peripheral arterial disease and idiopathic interstitial pneumonia complained of subcutaneous hemorrhage of the right lower thigh. Marked anemia (hemoglobin 5.5 g/dL) and prolonged activated partial thromboplastin time (≥130 seconds) were noted. Read More

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https://www.pagepress.org/journals/index.php/hr/article/view
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http://dx.doi.org/10.4081/hr.2018.7453DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6151351PMC
September 2018
2 Reads

Combined use of antifibrinolytics and activated prothrombin complex concentrate (aPCC) is not related to thromboembolic events in patients with acquired haemophilia A: data from FAIR Registry.

J Thromb Thrombolysis 2018 Sep 28. Epub 2018 Sep 28.

Haemophilia Centre, University Hospital of Padua, Via Giustiniani, 35128, Padua, Italy.

Antifibrinolytics combined with aPCC are not routinely administered to patients with acquired hemophilia A due to increased thrombotic risk. This association normalizes clot stability, and improves the efficacy of therapy, but can increase the risk of severe side effects. Due to these premises it has always raised doubts and perplexities in the clinics. Read More

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http://dx.doi.org/10.1007/s11239-018-1750-yDOI Listing
September 2018
8 Reads

Correction to: Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia.

Ann Hematol 2018 12;97(12):2531

Division of Emergency Medicine, McMaster University, Hamilton, Ontario, Canada.

The article Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia, written by Andreas Tiede and Andrew Worster, was originally published electronically on the publisher's internet portal (currently SpringerLink) on 26 May 2018 without open access. Read More

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http://link.springer.com/10.1007/s00277-018-3504-5
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http://dx.doi.org/10.1007/s00277-018-3504-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6208678PMC
December 2018
2 Reads

Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis: A case report and literature review.

Authors:
Fang Wei

Medicine (Baltimore) 2018 Sep;97(38):e12044

Introduction: Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII (FVIII). Spontaneous bleeding symptoms usually affect the skin, musco, muscle, and internal organs, while joint hemarthrosis in AHA is an extremely rare manifestation. AHA may have an autoimmune cause and is often associated with autoimmune disease, but no demonstrable platelet impairment was found in AHA patients. Read More

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http://dx.doi.org/10.1097/MD.0000000000012044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6160082PMC
September 2018
1 Read

Congenital and acquired bleeding disorders.

Authors:
Peter Salaj

Vnitr Lek 2018 ;64(5):547-558

Hemostasis can be characterized as an array of physiological mechanisms providing both blood fluidity in the intact blood vessels and hemostasis in the event of impaired continuity of the blood vessel wall. The impaired hemo-static balance may lead on the one hand to an increased tendency to bleed, either spontaneously or only in response to an external stimulus. At the opposite end of bleeding are thrombophilic conditions characterized by an increased tendency to blood coagulation and thereby to the development of venous or arterial thrombosis. Read More

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January 2018
1 Read

A Case Report of Anticoagulation Management in Acquired Hemophilia Associated With Levofloxacin.

J Pharm Pract 2018 Sep 6:897190018799186. Epub 2018 Sep 6.

1 Duke Regional Hospital, Durham, NC, USA.

Purpose: To report a case of acquired hemophilia secondary to levofloxacin and provide a guide for the use of anticoagulation in acute coronary syndrome.

Case Summary: A 75-year-old female treated with levofloxacin presented with spontaneous bruising of the upper extremities. Levofloxacin was discontinued and the symptoms resolved. Read More

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http://journals.sagepub.com/doi/10.1177/0897190018799186
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http://dx.doi.org/10.1177/0897190018799186DOI Listing
September 2018
16 Reads

A case of acquired hemophilia A in an elderly female.

J Community Hosp Intern Med Perspect 2018 23;8(4):237-240. Epub 2018 Aug 23.

Department of Hematology and Oncology, Saint Agnes Hospital, Baltimore, MD, USA.

Acquired Hemophilia A (AHA) is a bleeding diathesis related to the development of factor VIII inhibitor, which can frequently go undetected. It commonly manifests as spontaneous mucosal bleeds without any known history of a bleeding disorder, but has the potential to cause life threatening bleeding especially in elderly patients with underlying comorbidities. Here we describe a case of AHA in a 78 year old female presenting with spontaneous mucocutaneous bleeding as tongue hematoma and recurrent gastrointestinal (GI) bleeding. Read More

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http://dx.doi.org/10.1080/20009666.2018.1487246DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6116292PMC
August 2018
1 Read

Challenges in diagnosis and management of acquired factor XIII (FXIII) inhibitors.

Haemophilia 2018 Nov 24;24(6):e417-e420. Epub 2018 Aug 24.

Division of Hematology/Oncology, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

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http://doi.wiley.com/10.1111/hae.13603
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http://dx.doi.org/10.1111/hae.13603DOI Listing
November 2018
13 Reads

Acquired Hemophilia A: Experience of a Tertiary Care Institute from North India.

Indian J Hematol Blood Transfus 2018 Jul 18;34(3):524-529. Epub 2017 Sep 18.

2Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, 160012 India.

Acquired hemophilia A (AHA) is an uncommon bleeding disorder infrequently reported among Indians. The present retrospective data comprises eight cases of AHA over a period of 15 years. The mean age of patients was 59. Read More

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http://dx.doi.org/10.1007/s12288-017-0873-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081324PMC
July 2018
2 Reads

Retrospective review of Acquired Haemophilia A from the largest Canadian Haemophilia treatment centre.

Haemophilia 2018 Sep 16;24(5):e383-e387. Epub 2018 Aug 16.

Division of Hematology/Oncology, Department of Medicine, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1111/hae.13598DOI Listing
September 2018
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Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma.

Hamostaseologie 2018 Aug 15. Epub 2018 Aug 15.

Department of Internal Medicine, Jagiellonian University Medical College, Cracow, Poland.

Acquired haemophilia A (AHA) is a rare autoimmune disease caused by antibodies directed against clotting factor VIII. About half of cases are idiopathic, but AHA may also be secondary to autoimmune, dermatologic, or oncologic diseases. In approximately 10% of non-idiopathic cases, the disease occurs after or with the diagnosis of cancer as an extremely rare paraneoplastic syndrome. Read More

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http://dx.doi.org/10.1055/s-0038-1668570DOI Listing
August 2018
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Generalized pustular psoriasis as an unusual cause of acquired haemophilia A.

Eur J Dermatol 2018 Jun;28(3):417-418

Department of Dermatology, University Hospital, Brest, France.

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http://dx.doi.org/10.1684/ejd.2018.3271DOI Listing
June 2018
14 Reads

[Acquired hemophilia A revealing lung cancer].

Rev Mal Respir 2018 Sep 8;35(7):727-730. Epub 2018 Aug 8.

Service de pneumologie, hôpital de la Croix-Rousse, hospices civils de Lyon, 103, Grande rue de la Croix-Rousse, 69004 Lyon, France; Université Charles-Mérieux, 69004 Oullins, France. Electronic address:

Acquired hemophilia A (AHA) is a rare disease, defined by the production of anti-factor VIII antibodies causing disordered hemostasis. It is idiopathic in 50% of cases, but sometimes associated with solid tumors. We report a case where AHA led to the diagnosis of lung cancer. Read More

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http://dx.doi.org/10.1016/j.rmr.2017.08.005DOI Listing
September 2018
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Novel assays in the coagulation laboratory: a clinical and laboratory perspective.

Transfus Apher Sci 2018 Aug 20;57(4):480-484. Epub 2018 Jul 20.

University of Toronto, Department of Medicine, Toronto, Canada; St. Michael's Hospital Hemostasis and Thrombosis Laboratory, Toronto, Canada; Li Ka Shing Knowledge Institute, St. Michael's Hospital, Toronto, Canada.

The ability to monitor Factor VIII (FVIII) and Factor IX (FIX) levels is integral to the clinical management of hemophilia A and B patients, respectively. Factor activity levels are checked during regular follow-up, post-infusion of factor concentrates, during pre- and post-operative assessments, and when the presence of an inhibitor is suspected. However, the ability to accurately and reproducibly measure factor activity levels with standard coagulation assays has been challenging due to the emergence of recombinant factor concentrates with extended half-lives. Read More

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http://dx.doi.org/10.1016/j.transci.2018.07.008DOI Listing
August 2018
32 Reads

Treatment and prevention of bleeding in congenital hemophilia A patients with inhibitors.

Authors:
Jerome M Teitel

Transfus Apher Sci 2018 Aug 29;57(4):466-471. Epub 2018 Jul 29.

Division of Hematology and Oncology, St. Michael's Hospital, Toronto, and Department of Medicine, University of Toronto, Canada. Electronic address:

The treatment of bleeding in hemophilia A patients with persistent inhibitory antibodies to factor VIII is problematic. The current standard hemostatic agents for inhibitor patients are recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (APCC). These "inhibitor bypassing agents" are less reliably effective than are replacement therapies for patients without inhibitors, and there are no validated laboratory assays to monitor their efficacy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14730502183030
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http://dx.doi.org/10.1016/j.transci.2018.07.011DOI Listing
August 2018
27 Reads

Identification of a novel mutation in the factor VIII gene causing severe haemophilia A.

BMC Hematol 2018 31;18:17. Epub 2018 Jul 31.

1Department of Infectious Diseases, Aarhus University Hospital, Aarhus, Denmark.

Background: Deficiency in coagulation factor VIII encoded by results in the X-linked recessive bleeding disorder haemophilia A (HEMA). Here we describe the identification of a novel variant in the factor VIII gene, , in an adult male patient with severe haemophilia A.

Case Presentation: The patient was diagnosed in early childhood and subsequently co-infected with Hepatitis C and HIV acquired during early blood transfusion for haemophilia in the 1980ies. Read More

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http://dx.doi.org/10.1186/s12878-018-0113-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6069878PMC
July 2018
3 Reads

Acquired Hemophilia of Unknown Etiology in an Elderly Man: Case Report.

Am J Case Rep 2018 Jul 23;19:858-863. Epub 2018 Jul 23.

Jeffrey Cheah School of Medicine, Monash University, Johor Bahru, Malaysia.

BACKGROUND Acquired hemophilia is a rare but potentially dangerous bleeding disorder caused by autoantibodies against coagulation factors. It affects 1 to 1.5 per 1 million people each year. Read More

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http://dx.doi.org/10.12659/AJCR.909228DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6066981PMC