3,238 results match your criteria Hemophilia Acquired


Recovery from COVID-19 following Hepatitis C, Human Immunodeficiency Virus Infection and Liver Transplantation.

Am J Transplant 2020 Jun 3. Epub 2020 Jun 3.

General, Visceral and Transplant Surgery, Dept. of Surgery, Medical University of Graz.

Immunosuppression and frequent comorbidities in transplant recipients potentially increase the risk of fatal outcomes of pandemic coronavirus disease 2019 (COVID-19) [1]. A 1965 born male had suffered from haemophilia A. In the nineteen-seventies, he acquired hepatitis C virus (HCV) infection, probably via factor VIII supplementation, and in 1985 human immunodeficiency virus (HIV) infection. Read More

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http://dx.doi.org/10.1111/ajt.16107DOI Listing

Acquired bleeding disorders.

Haemophilia 2020 Jun 1. Epub 2020 Jun 1.

Surgical Research Laboratory and Section of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Acquired bleeding disorders can accompany hematological, neoplastic, autoimmune, cardiovascular or liver diseases, but can sometimes also arise spontaneously. They can manifest as single factor deficiencies or as complex hemostatic abnormalities. This review addresses (a) acquired hemophilia A, an autoimmune disorder characterized by inhibitory autoantibodies against coagulation factor VIII; (b) acquired von Willebrand syndrome in patients with cardiovascular disorders, where shear stress abnormalities result in destruction of von Willebrand factor; and (c) liver function disorders that comprise complex changes in pro- and anti-hemostatic factors, whose clinical implications are often difficult to predict. Read More

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http://dx.doi.org/10.1111/hae.14033DOI Listing

Predictors of response and outcome of patients with acquired haemophilia A.

Haemophilia 2020 May 29. Epub 2020 May 29.

Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, MI, USA.

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http://dx.doi.org/10.1111/hae.14054DOI Listing

Acquired hemophilia A revealed by spontaneous bleeding in an 80-year-old man: a marginal diagnosis?

Acta Clin Belg 2020 May 27:1-3. Epub 2020 May 27.

Department of Hematology, Hôpital Erasme, Université Libre De Bruxelles , Brussels, Belgium.

This case report illustrates the difficulty associated with diagnosing acquired hemophilia A by reviewing the case of an 80-year-old man admitted to the hospital for anemia. A prolonged activated partial thromboplastin time (aPTT) was not noticed until the patient developed a severe hemorrhagic syndrome. Read More

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http://dx.doi.org/10.1080/17843286.2020.1768735DOI Listing

Postoperative hemorrhage secondary to acquired hemophilia A.

JAAPA 2020 Jun;33(6):24-26

At the University of Colorado School of Medicine in Aurora, Colo., Zachary P. Asher practices in the Department of Surgery, Tyler W. Buckner practices in the Department of Medicine, and Robert C. McIntyre, Jr., practices in the Department of Surgery. The authors have disclosed no potential conflicts of interest, financial or otherwise.

Acquired hemophilia A in postoperative patients can cause major bleeding and an accurate diagnosis is required for effective treatment. Standard treatment is costly, difficult to obtain, and takes 4 to 6 weeks to be effective. This article describes a patient successfully treated with recombinant factor VIIa, porcine factor VIII, plasmapheresis, rituximab, and high-dose corticosteroids. Read More

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http://dx.doi.org/10.1097/01.JAA.0000660136.56989.efDOI Listing

International recommendations on the diagnosis and treatment of acquired hemophilia A.

Haematologica 2020 May 7. Epub 2020 May 7.

Green Templeton College, University of Oxford, Oxford, UK.

Acquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time (APTT) due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Read More

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http://dx.doi.org/10.3324/haematol.2019.230771DOI Listing

Utilization of Emicizumab in Acquired Factor VIII Deficiency.

Am J Case Rep 2020 May 7;21:e922326. Epub 2020 May 7.

Department of Hematology and Oncology, Lehigh Valley Health Network, Allentown, PA, USA.

BACKGROUND Acquired hemophilia A (AHA) is a rare autoimmune disease caused by immunoglobulins that bind and inactive factor VIII, thereby predisposing to life-threatening bleeding. Bleeding is typically stabilized by utilizing bypassing agents, such as recombinant factor VIIa (rVIIa). Select case reports have demonstrated the success of alternative prophylaxis for clearance of factor VIII inhibitors through the use of emicizumab, a current FDA approved medication for treatment of congenital hemophilia A. Read More

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http://dx.doi.org/10.12659/AJCR.922326DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7237794PMC

Acute airway compromise and coagulopathy: a rare presentation of acquired haemophilia A.

BMJ Case Rep 2020 May 4;13(5). Epub 2020 May 4.

Department of Otolaryngology, Head & Neck Surgery, Gosford Hospital, Gosford, New South Wales, Australia.

Acquired haemophilia A is a rare but important diagnosis, carrying a mortality rate of 22%. Life-threatening sequalae of this diagnosis includes airway compromise, which can rapidly lead to demise of the patient if left untreated. Our case examines an 80-year-old man presenting with a supraglottic haematoma resulting from acquired haemophilia A causing airway compromise and necessitating definitive airway control. Read More

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http://dx.doi.org/10.1136/bcr-2019-233345DOI Listing

A hemophilia A mouse model for the in vivo assessment of Emicizumab function.

Blood 2020 May 5. Epub 2020 May 5.

INSERM, Le Kremlin Bicetre, France.

The bispecific antibody Emicizumab is increasingly used for hemophilia A-treatment. However, its specificity for human factors IX and X (FIX, FX) has limited its in vivo functional analysis to primate models of acquired hemophilia. Here we describe a novel mouse model allowing to examine Emicizumab function. Read More

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http://dx.doi.org/10.1182/blood.2019004334DOI Listing

Clinical and laboratorial description of the differential diagnoses of hemostatic disorders in the horse.

Iran J Vet Res 2020 ;21(1):1-8

Department of Animal Medicine and Surgery, Faculty of Veterinary Medicine, University of Córdoba, Córdoba, Spain.

The process of fibrin clot formation is a series of complex and well-regulated reactions involving blood vessels, platelets, procoagulant plasma proteins, natural inhibitors, and fibrinolytic enzymes. Vasculitis can be caused by a variety of different agents as bacteria, viruses, protozoal, rickettsial organisms, toxic, drugs, medications, and neoplasms. The most common cause of vasculitis is the purpura hemorrhagica, which is associated with exposure to ssp. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7183377PMC
January 2020

Therapeutic doses of recombinant factor VIIa in hemophilia generates thrombin in platelet-dependent and -independent mechanisms.

J Thromb Haemost 2020 May 2. Epub 2020 May 2.

Department of Cellular and Molecular Biology, The University of Texas Health Science Center at Tyler, Texas, USA.

Background: In hemophilia bypass therapy, a platelet-dependent mechanism is believed to be primarily responsible for recombinant factor VIIa (rFVIIa)'s hemostatic effect. rFVIIa may also possibly interact with other cells through its binding to endothelial cell protein C receptor (EPCR) or cell surface phospholipids.

Objectives: We aim to investigate the relative contribution of platelet-dependent and platelet-independent mechanisms in rFVIIa-mediated thrombin generation in hemophilic conditions at the injury site. Read More

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http://dx.doi.org/10.1111/jth.14881DOI Listing

Mechanistic Insights into Factor VIII Immune Tolerance Induction via Prenatal Cell Therapy in Hemophilia A.

Curr Stem Cell Rep 2019 Dec 20;5(4):145-161. Epub 2019 Nov 20.

Wake Forest Institute for Regenerative Medicine, Wake Forest School of Medicine, Winston-Salem, NC, USA.

Purpose Of Review: Prenatal stem cell and gene therapy approaches are amongst the few therapies that can promise the birth of a healthy infant with specific known genetic diseases. This review describes fetal immune cell signaling and its potential influence on donor cell engraftment, and summarizes mechanisms of central T cell tolerance to peripherally-acquired antigen in the context of prenatal therapies for Hemophilia A.

Recent Findings: During early gestation, different subsets of antigen presenting cells take up peripherally-acquired, non-inherited antigens and induce the deletion of antigen-reactive T-cell precursors in the thymus, demonstrating the potential for using prenatal cell and gene therapies to induce central tolerance to FVIII in the context of prenatal diagnosis/therapy of Hemophilia A. Read More

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http://dx.doi.org/10.1007/s40778-019-00165-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7189775PMC
December 2019

Short term efficacy of recombinant porcine factor VIII in patients with factor VIII inhibitors.

Haemophilia 2020 Apr 27. Epub 2020 Apr 27.

Case Western Reserve University, Cleveland, OH.

Background: Antibodies against factor VIII (FVIII), seen in acquired (AHA) and congenital haemophilia A, lead to severe bleeding diatheses. Current first-line treatment includes bypass agents. Recombinant porcine sequence FVIII (rpFVIII) was developed as an alternative therapy. Read More

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http://dx.doi.org/10.1111/hae.14014DOI Listing

The never ending success story of tranexamic acid in acquired bleeding.

Haematologica 2020 May 26;105(5):1201-1205. Epub 2020 Mar 26.

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

Tranexamic acid (TXA) is an anti-fibrinolytic agent that acts by inhibiting plasminogen activation and fibrinolysis. Although its first clinical use dates back more than 50 years, this hemostatic agent is still the object of intense clinical and developmental research. In particular, renewed interest in TXA has arisen following evidence that it has a beneficial effect in reducing blood loss in a variety of medical and surgical conditions at increased risk of bleeding. Read More

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http://dx.doi.org/10.3324/haematol.2020.250720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7193503PMC

Acquired haemophilia A after alemtuzumab treatment of multiple sclerosis.

Br J Haematol 2020 Apr 16. Epub 2020 Apr 16.

Department of Internal Medicine, Maasstad Hospital, Rotterdam, The Netherlands.

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http://dx.doi.org/10.1111/bjh.16644DOI Listing

Advances in managing rare acquired bleeding disorders.

Expert Rev Hematol 2020 Apr 23:1-8. Epub 2020 Apr 23.

Italian National Blood Centre, National Institute of Health, Rome, Italy.

: Rare acquired bleeding disorders include a wide spectrum of coagulopathies characterized by spontaneous or post-trauma and post-surgery hemorrhages in patients without a previous personal or family history of bleeding.: This review, based on a Medline/PubMed search during the last 20 years, will focus mainly on rare acquired bleeding disorders caused by autoantibodies against coagulation factors, including autoantibodies against factor VIII (acquired hemophilia A), von Willebrand factor (acquired von Willebrand syndrome) and other coagulation factors (factors V, X, XI, and XIII). The pathogenic, laboratory, and clinical features of these rare hemorrhagic conditions will be discussed, with particular attention to their management. Read More

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http://dx.doi.org/10.1080/17474086.2020.1756259DOI Listing

A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review.

World J Oncol 2020 Apr 29;11(2):72-75. Epub 2020 Mar 29.

Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ 07753, USA.

Hemophilia is a bleeding diathesis that is most commonly congenital and causes a tendency for significant bleeding during procedures and often manifests as hemarthrosis. However, more rarely, hemophilia can be acquired. Our paper focuses on acquired hemophilia A (AHA), which is caused by the development of an autoantibody (an inhibitor) to factor VIII. Read More

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http://dx.doi.org/10.14740/wjon1260DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141160PMC

Femoral pseudotumor secondary to injury in a patient with idiopathic thrombocytopenic purpura: Case report.

Medicine (Baltimore) 2020 Apr;99(15):e19788

Department of Radiology, The Third Hospital of Hebei Medical University, Shijiazhuang Hebei Province, China.

Rationale: Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count of unknown causes and is a poorly understood acquired hemorrhagic disease involving destruction of platelets in the reticuloendothelial system induced by antiplatelet antibodies. Patients with ITP can have traumatic intra-articular, intraosseous or soft tissue hemorrhage which may present as a rare intraosseous pseudotumor on medical imaging.

Patient Concerns: A 30-year old male patient had complaint of pain in the right leg for 1 year. Read More

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http://dx.doi.org/10.1097/MD.0000000000019788DOI Listing
April 2020
5.723 Impact Factor

Effect of different methods for outlier detection and rejection when calculating cut off values for diagnosis of lupus anticoagulants.

Thromb Res 2020 Jun 26;190:20-25. Epub 2020 Mar 26.

Foundation IRCCS Ca' Granda Maggiore Policlinico Hospital, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milano, Italy. Electronic address:

Background: Lupus anticoagulant (LA)-detection is performed by testing plasma with activated partial thromboplastin time (APTT)-derived and dilute-Russell-viper-venom (dRVV)-derived tests. Results are interpreted by comparison to cut-off values determined by testing plasma from healthy-subjects. Several issues are concerned with the determination of LA cut-offs. Read More

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http://dx.doi.org/10.1016/j.thromres.2020.03.018DOI Listing

Bleeding and Response to Hemostatic Therapy in Acquired Hemophilia A (AHA): Results from the GTH-AH 01/2010 Study.

Blood 2020 Apr 8. Epub 2020 Apr 8.

Hannover Medical School, Hannover, Germany.

Acquired hemophilia A (AHA) is due to autoantibodies against coagulation factor VIII (FVIII) and most often presents with unexpected bleeding. In contrast to congenital hemophilia, the patient's residual FVIII activity does not seem to correlate with the risk of bleeding as suggested from previous studies. Risk factors for bleeding have not been described. Read More

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http://dx.doi.org/10.1182/blood.2019003639DOI Listing

Acquired Hemophilia A, Hemolytic Anemia, Type 1 Diabetes Mellitus, and Autoimmune Hypothyroidism in a Systemic Lupus Erythematosus Patient.

J Clin Rheumatol 2020 Apr 2. Epub 2020 Apr 2.

Rheumatology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal Young Adult and Pediatric Rheumatology, Unit / Centro Hospitalar e Universitário, do Hospital de São João, Porto, Portugal Rheumatology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal.

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http://dx.doi.org/10.1097/RHU.0000000000001383DOI Listing

Acquired platelet dysfunction with eosinophilia in two patients.

Pediatr Neonatol 2020 Jun 12;61(3):346-347. Epub 2020 Mar 12.

Center for Rare Disease and Hemophilia, Taichung Veterans General Hospital, Taichung, Taiwan; Department of Pediatrics, Taichung Veterans General Hospital, Taichung, Taiwan; Department of Industrial Engineering and Enterprise Information, Tunghai University, Taichung, Taiwan. Electronic address:

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http://dx.doi.org/10.1016/j.pedneo.2020.03.004DOI Listing

Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study.

J Thromb Thrombolysis 2020 Mar 26. Epub 2020 Mar 26.

Department of Hemostaseology and Hemophilia Center, University Hospital Frankfurt, Theodor Stern Kai 7, 60596, Frankfurt, Germany.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severely reduced activity of the von-Willebrand factor-cleaving protease ADAMTS13, mainly caused by anti-ADAMTS-13 antibodies. Although several test systems for ADAMTS13 measurement exist, long turn-around times hamper the usability in daily practice. We performed a method comparison study for two commercially available ADAMTS13 assays and evaluated the agreement between the fully-automated rapid turn-over HemosIL AcuStar ADAMTS13 Activity assay and the manually performed TECHNOZYM ADAMTS-13 Activity assay. Read More

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http://dx.doi.org/10.1007/s11239-020-02086-8DOI Listing

Prevalence of the age-related diseases in older patients with acquired thrombotic thrombocytopenic purpura.

Eur J Intern Med 2020 May 20;75:79-83. Epub 2020 Mar 20.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano,Via Pace 9, Milan, 20122, Italy; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, and Fondazione Luigi Villa, Milan, Italy. Electronic address:

Background: The prevalence of older patients with acquired thrombotic thrombocytopenic purpura (TTP) is increasing. There is scarce information on the prevalence of multimorbidity, polypharmacy and age-related diseases in aging TTP patients. This study aimed to evaluate the prevalence of multimorbidity and polypharmacy in a population of acquired TTP patients aged 65 years or more compared with a group of age-matched controls. Read More

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http://dx.doi.org/10.1016/j.ejim.2020.01.024DOI Listing

Effects of performing dual tasks on postural sway and postural control complexity in people with haemophilic arthropathy.

Haemophilia 2020 May 20;26(3):e81-e87. Epub 2020 Mar 20.

Physiotherapy in Motion Multispeciality Research Group (PTinMOTION), Department of Physiotherapy, University of Valencia, Valencia, Spain.

Introduction: People with haemophilic arthropathy (PWHA) have impairments in postural control. However, little is known about the effects of demanding conditions, including the unipedal stance and dual tasks, on postural control in PWHA.

Aim: Determine the effects of performing dual tasks while in the one-leg stance on postural sway and postural control complexity in PWHA vs. Read More

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http://dx.doi.org/10.1111/hae.13955DOI Listing

A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack.

Acta Haematol 2020 Mar 10:1-3. Epub 2020 Mar 10.

Division of Angiology and Haemostasis, Geneva University Hospitals and Faculty of Medicine, Geneva, Switzerland,

Acquired hemophilia A (AHA) is a rare but serious condition, usually associated with significant spontaneous or traumatic bleeding and a high mortality rate. In this report, we describe the case of an elderly patient presenting a transient ischemic attack concurrently with AHA. A thrombotic event in AHA is occasionally associated with the use of bypassing agents for treatment, but a spontaneous thrombotic event has not ever been described. Read More

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http://dx.doi.org/10.1159/000506273DOI Listing

Full-genome analysis of hepatitis C virus in HIV-coinfected hemophiliac Japanese patients.

Hepatol Res 2020 Mar 9. Epub 2020 Mar 9.

AIDS Clinical Center, National Center for Global Health and Medicine, Tokyo, Japan.

Aim: More than 1400 Japanese hemophiliacs acquired HIV infection around 1983 through contaminated blood products imported from the USA, most of whom also acquired hepatitis C virus (HCV) infection. To delineate the HCV genetic relations in HIV-coinfected hemophiliacs, we analyzed stocked plasma samples of the patients seen at the largest referral center for HIV care in Japan.

Methods: Hepatitis C virus full-genome sequences were amplified and determined using next-generation sequencing, and genotyping and phylogenetic analyses of these sequences were carried out. Read More

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http://dx.doi.org/10.1111/hepr.13498DOI Listing

Acquired haemophilia A treated with recombinant porcine factor VIII: a single centre UK experience.

Br J Haematol 2020 May 6;189(4):e130-e133. Epub 2020 Mar 6.

Oxford Haemophilia and Thrombosis Centre, Oxford University Hospitals NHS Foundation Trust, NIHR Oxford Biomedical Research Centre, Oxford, UK.

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http://dx.doi.org/10.1111/bjh.16556DOI Listing

Essential thrombocythemia A retrospective case series.

Pediatr Blood Cancer 2020 May 2;67(5):e28183. Epub 2020 Mar 2.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Background: Essential thrombocythemia (ET) is rare in children, and pediatric guidelines are lacking. Therefore, we aimed to evaluate ET diagnosis and treatment in a pediatric cohort.

Procedure: Data of patients with ET from three hospitals were reviewed. Read More

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http://dx.doi.org/10.1002/pbc.28183DOI Listing

Treatment of the Dental Patient with Bleeding Dyscrasias: Etiologies and Management Options for Surgical Success in Practice.

Dent Clin North Am 2020 04 31;64(2):411-434. Epub 2020 Jan 31.

Woodhull Medical and Mental Health Center, 760 Broadway, Brooklyn, NY 11206, USA.

The oral health care provider sees a significant number of patients in his or her practice who suffer from systemic diseases affecting the ability to clot. These medical issues can be acquired or inherited bleeding dyscrasias requiring pharmacologic therapy during the perioperative period. Patients with inherited or acquired bleeding disorders require careful attention with respect to the assessment of bleeding risk. Read More

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http://dx.doi.org/10.1016/j.cden.2019.12.010DOI Listing

Impaired platelet-dependent thrombin generation associated with thrombocytopenia is improved by prothrombin complex concentrates in vitro.

Res Pract Thromb Haemost 2020 Feb 14;4(2):334-342. Epub 2020 Feb 14.

School of Biosciences & Medicine University of Surrey Guildford UK.

Background: Impaired thrombin generation (TG) in patients with acquired coagulopathy, is due to low coagulation factors and thrombocytopenia. The latter is typically treated with platelet transfusions and the former with plasma and occasionally with prothrombin complex concentrates (PCCs). We hypothesized that manipulating the concentrations of coagulation factors might result in restoration of platelet-dependent TG over and above that of simple replacement therapy. Read More

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http://dx.doi.org/10.1002/rth2.12310DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7040546PMC
February 2020

Acquired factor VIII deficiency in prostate adenocarcinoma presenting as multiple hematomas and hemarthrosis.

SAGE Open Med Case Rep 2020 16;8:2050313X20906743. Epub 2020 Feb 16.

Department of Hematology and Oncology, SUNY Downstate Medical Center, Brooklyn, NY, USA.

Acquired hemophilia A or acquired factor VIII deficiency is a rare bleeding disorder due to the presence of autoantibodies to factor VIII. It has been associated with autoimmune conditions, certain medications, and malignancy. It has a high morbidity and mortality, and early diagnosis and treatment is critically important. Read More

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http://dx.doi.org/10.1177/2050313X20906743DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7026807PMC
February 2020

Acquired Hemophilia in an Elderly Patient with Carcinoma of the Ampulla of Vater.

Case Rep Oncol 2020 Jan-Apr;13(1):1-6. Epub 2020 Jan 13.

Department of Medical Oncology, University of Ioannina, Ioannina, Greece.

Acquired hemophilia is a rare autoimmune bleeding disorder related to the production of autoantibodies that inhibit clotting factor VIII or IX. The underlying cause can be autoimmune disease, malignancy, pregnancy, or medications, but it is most commonly idiopathic. Here we present the case of an 81-year-old patient with locoregionally relapsed periampullary carcinoma who presented with soft tissue hematoma and an abnormally elevated activated partial thromboplastin time (aPTT) in the presence of a normal prothrombin time. Read More

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http://dx.doi.org/10.1159/000504338DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7036531PMC
January 2020

Gene therapy for hemophilias: the end of phenotypic testing or the start of a new era?

Blood Coagul Fibrinolysis 2020 Jun;31(4):237-242

Department of Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, New South Wales, Australia.

: Hemophilia comprises two distinct genetic disorders caused by missing or defective clotting factor VIII (hemophilia A) or clotting factor IX (hemophilia B). The management of these conditions has been for long based on replacement therapies, but emerging evidence garnered from recent landmark studies suggests that a promising avenue toward routine use of gene therapy is clearly progressing forward, thus generating unavoidable consequences on laboratory hemostasis, especially as pertaining to phenotypic testing. Although it seems likely that widespread use of gene therapy will be associated with a relative decrease of hemostasis tests requests in this patient population due to the relatively stable effect of transgene delivery and persistent production of endogenous clotting factor, some important aspects persuade us that conventional laboratory diagnostics, especially encompassing activated partial thromboplastin time, as well as one-stage and two-stage clotting factor assays, will not be completely voided in the gene therapy era. Read More

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http://dx.doi.org/10.1097/MBC.0000000000000905DOI Listing

Radiosynoviorthesis in the treatment of posttraumatic joint bleedings of hemophilic patients (concerning hip, knee and ankle joints)-Hungarian experience.

Injury 2020 Feb 16. Epub 2020 Feb 16.

Department of Orthopaedics, Uzsoki Hospital, Uzsoki street 29, Budapest 1145, Hungary.

Introduction: The surgical, arthroscopic synovectomy and radiosynovectomy (radiosynoviorthesis, RSO) all have great practical importance, since they can eliminate the posttraumatic joint bleedings and prevent the further joint destructions in hemophilic patients. The aim of this study was to examine the role of RSO in the prevention of joint bleedings in hemophilic patients.

Methods: 54 out of 684 RSO patients were hemophiliacs. Read More

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http://dx.doi.org/10.1016/j.injury.2020.02.054DOI Listing
February 2020

Acquired Factor XIII Deficiency Inducing Recurrent and Fatal Bleeding, Description of a Case.

J Blood Med 2020 30;11:43-45. Epub 2020 Jan 30.

Department of Internal Medicine and Emergency Room, Fatebenefratelli Hospital of Naples, Naples, Italy.

Factor XIII deficiency may be inherited or acquired. Inherited deficiency is associated with signs and symptoms of minor bleeding from a young age, and possible major bleeding complications, in particular during pregnancy. On the other hand, acquired factor XIII deficiency is usually associated with severe symptoms of major bleeding, in particular during surgery. Read More

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http://dx.doi.org/10.2147/JBM.S232115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996541PMC
January 2020

Acquired hemophilia A.

Proc (Bayl Univ Med Cent) 2020 Jan 20;33(1):71-74. Epub 2019 Nov 20.

Department of Hematology and Oncology, University of Arkansas for Medical SciencesLittle RockArkansas.

Acquired inhibitors of coagulation are a group of rare but potentially life-threatening blood disorders characterized by the presence of autoantibodies directed against clotting factor. Autoantibody against factor VIII is the most common form of clotting factor inhibitor, a condition also known as acquired hemophilia A. We present a clinical series of nine patients diagnosed and treated for acquired hemophilia A at our institution. Read More

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http://dx.doi.org/10.1080/08998280.2019.1689024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6988643PMC
January 2020

Bilateral simultaneous orbital hemorrhage and compartment syndrome as the presenting sign of acquired hemophilia A in an 11-year-old girl.

J AAPOS 2020 Feb 12. Epub 2020 Feb 12.

Eye Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; Department of Ophthalmology, Lerner College of Medicine of Case Western University, Cleveland, Ohio. Electronic address:

A previously healthy 11-year-old girl presented with bilateral orbital compartment syndrome secondary to retrobulbar hemorrhages requiring emergency cantholysis. Four days earlier she had sustained head trauma without symptoms until her acute presentation. A basic hematologic profile was remarkable for a prolonged partial thromboplastin time, which did not completely correct with a 1:1 mixing study. Read More

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http://dx.doi.org/10.1016/j.jaapos.2020.01.002DOI Listing
February 2020

Acquired haemophilia and haemostatic control with recombinant porcine FVIII: case series.

Intern Med J 2020 Feb 11. Epub 2020 Feb 11.

Haemophilia Treatment Centre, Alfred Hospital, Melbourne, Victoria, Australia.

Background: Acquired haemophilia A (AHA) is a rare acquired bleeding disorder that can present with life threatening bleeding.

Objective: To describe recent Australian use of recombinant porcine FVIII (rpFVIII) replacement therapy as a haemostatic agent in patients with acquired haemophilia.

Patients/method: Four patients with acquired haemophilia treated in three different institutions around Australia in the last 12 (twelve) months were included in the study. Read More

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http://dx.doi.org/10.1111/imj.14773DOI Listing
February 2020

Yttrium-90 radiosynovectomy in knees and ankles (25 joints in 22 hemophilic patients). Short-term results.

Hematol Transfus Cell Ther 2020 Jan 30. Epub 2020 Jan 30.

Medical School of Ribeirão Preto, University of São Paulo, São Paulo, Brazil.

Introduction: The radiosynovectomy (RS) is one treatment option for recurrent hemarthrosis in patients with hemophilia (PWH). A prospective cohort study was designed to evaluate the effects of the RS on the synovial membrane volume in the ankles and knees of PWH and patient characteristics related to the RS outcome.

Methods: In a one-year follow-up, 25 joints of 22 PWH who presented 3 bleeds or more in the same joint over the last 6 months (target joints) were subjected to the RS. Read More

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http://dx.doi.org/10.1016/j.htct.2019.11.001DOI Listing
January 2020

Plasma-derived factors VIIa and X mixtures (Byclot) significantly improve impairment of coagulant potential ex vivo in plasmas from acquired hemophilia A patients.

Int J Hematol 2020 Jun 6;111(6):779-785. Epub 2020 Feb 6.

Department of Pediatrics, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

A combined product of plasma-derived factor (F)VIIa and FX (pd-FVIIa/FX; Byclot) is currently available for the hemostatic treatment of hemophilia A and B patients with inhibitors in Japan. Limited information is available, however, on its coagulant effect in acquired hemophilia A (AHA). In the present study, we assessed the coagulant effect of pd-FVIIa/FX on impairment of coagulation potentials in AHA. Read More

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http://dx.doi.org/10.1007/s12185-020-02837-6DOI Listing

Acquired hemophilia.

Gac Med Mex 2020 ;156(1):67-77

Unidad de Investigación Médica en Trombosis, Hemostasia y Aterogénesis. Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Ciudad de México, México.

Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. Read More

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http://dx.doi.org/10.24875/GMM.19005469DOI Listing
January 2020

[Bullous pemphigoid associated with acquired hemophilia A].

Ann Dermatol Venereol 2020 Jan 30. Epub 2020 Jan 30.

Service de dermatologie, hôpital Arrazi, CHU Mohammed VI, université Caddi Ayyad, faculté de médecine, BP2360 Principal, avenue Ibn Sina, Marrakech, Maroc.

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http://dx.doi.org/10.1016/j.annder.2020.01.004DOI Listing
January 2020

Development and application of global assays of hyper- and hypofibrinolysis.

Res Pract Thromb Haemost 2020 Jan 6;4(1):46-53. Epub 2019 Nov 6.

Department of Pathology and Laboratory Medicine University of North Carolina Chapel Hill North Carolina.

Numerous methods for evaluation of global fibrinolytic activity in whole blood or plasma have been proposed, with the majority based on tissue-type plasminogen activator (t-PA) addition to initiate fibrinolysis. We propose that such an approach is useful to reveal hypofibrinolysis, but t-PA concentrations should be kept to a minimum. In this paper, we describe a low-concentration t-PA plasma turbidity assay to evaluate several congenital factor deficiencies, including plasminogen activator inhibitor-1 (PAI-1) and plasminogen deficiency, as well as hemophilia A and B. Read More

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http://dx.doi.org/10.1002/rth2.12275DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6971323PMC
January 2020

Acquired Glanzmann's thrombasthenia: Diagnosis aided by platelet aggregation mixing study.

Haemophilia 2020 Mar 27;26(2):e41-e43. Epub 2020 Jan 27.

Department of Pathology, UT Southwestern Medical Center, Dallas, Texas.

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http://dx.doi.org/10.1111/hae.13914DOI Listing

An anti-factor IXa/factor X bispecific antibody, emicizumab, improves ex vivo coagulant potentials in plasma from patients with acquired hemophilia A.

J Thromb Haemost 2020 Apr 26;18(4):825-833. Epub 2020 Feb 26.

Department of Pediatrics, Nara Medical University, Kashihara, Japan.

Introduction: Acquired hemophilia A (AHA) is caused by autoantibodies against factor (F)VIII, and is characterized by severe, spontaneous bleeding, which can be life-threatening. Emicizumab, an anti-FIXa/FX bispecific antibody, significantly reduces bleeding events in congenital hemophilia A (HA) with and without inhibitors. The known pathophysiological mechanisms and current preclinical data in HA suggest that emicizumab could provide effective treatment for AHA, but the coagulation activities of emicizumab in these patients remain unknown. Read More

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http://dx.doi.org/10.1111/jth.14746DOI Listing

Reducing the effect of DOAC interference in laboratory testing for factor VIII and factor IX: A comparative study using DOAC Stop and andexanet alfa to neutralize rivaroxaban effects.

Haemophilia 2020 Mar 21;26(2):354-362. Epub 2020 Jan 21.

Western Australian Centre for Thrombosis and Haemostasis (WACTH), Murdoch University, Perth, WA, Australia.

Introduction: Investigation of factors (F) VIII and IX is common, with testing important for diagnosis or exclusion of haemophilia A or B, associated acquired conditions and factor inhibitors. Rivaroxaban, a common direct anti-Xa agent, causes significant interference in clotting assays, including substantial false reduction of factor levels.

Aim: To assess whether rivaroxaban-induced interference of FVIII and FIX testing could be neutralized. Read More

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http://dx.doi.org/10.1111/hae.13930DOI Listing