3,368 results match your criteria Hemophilia Acquired


Coexistence of Acquired Hemophilia and Antiphospholipid Serology in Monoclonal Gammopathy Patient.

Int Med Case Rep J 2021 28;14:261-264. Epub 2021 Apr 28.

Department of Internal Medicine, Groupe Hospitalier Sud-Ile de France, Melun, 77000, France.

Acquired hemophilia is a rare coagulopathy with hemorrhage into the skin, muscle, or soft tissues and mucous membranes and caused by inhibitor antibodies, mainly against FVIII. We report a case of acquired hemophilia presenting with diffuse cutaneous hemorrhage and hemothorax. The patient was found to have acquired an FVIII inhibitor and a high titer of anti β2 glycoprotein 1 IgG and IgM, and anticardiolipin IgM in the context of IgA kappa-type monoclonal gammopathy. Read More

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Laparoscopic Hepatectomy for the Patient with Hemophilia A with High Titer Factor VIII Inhibitor.

Acta Med Okayama 2021 Apr;75(2):199-204

Department of Gastroenterological Surgery,Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences.

We present the first case of laparoscopic left lateral segmentectomy for hepatocellular carcinoma (HCC) in a patient with hemophilia A, acquired hepatitis C, and high-titer factor VIII inhibitor, which was confirmed by preoperative diagnosis. He underwent laparoscopic left lateral segmentectomy with the administration of recombinant activated factor VII. Surgery could be performed with reduced intraoperative hemorrhage. Read More

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[How I diagnose and treat acquired hemophilia A].

Authors:
S J Wang

Zhonghua Xue Ye Xue Za Zhi 2021 Mar;42(3):193-198

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China.

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Daratumumab as a novel treatment for refractory acquired von Willebrand syndrome associated with monoclonal gammopathy.

Haemophilia 2021 Apr 28. Epub 2021 Apr 28.

Division of Hematology and Hematologic Malignancies, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.

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[Acquired hemophilia as the initial manifestation of colorectal cancer's recurrence].

Medicina (B Aires) 2021 ;81(2):286-288

Service de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, Francia.

We report the case of an 86-year-old man presenting with a spontaneous hematoma in the left iliac muscle and previous diagnosis of colon cancer in 1998 (stage pT3N0M0) treated with transverse colectomy and considered in complete remission. After a complete study, it was possible to identify the presence of Factor VIII inhibitors antibodies that confirmed the presence of acquired hemophilia. During hospitalization the patient presented a lower gastrointestinal bleeding leading to the diagnosis of recurrence of a previously treated colorectal adenocarcinoma. Read More

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Caplacizumab prevents refractoriness and mortality in acquired thrombotic thrombocytopenic purpura: integrated analysis.

Blood Adv 2021 Apr;5(8):2137-2141

Medical Affairs, Sanofi, Diegem, Belgium.

The efficacy and safety of caplacizumab in individuals with acquired thrombotic thrombocytopenic purpura (aTTP) have been established in the phase 2 TITAN and phase 3 HERCULES trials. Integrated analysis of data from both trials was conducted to increase statistical power for assessing treatment differences in efficacy and safety outcomes. Caplacizumab was associated with a significant reduction in the number of deaths (0 vs 4; P < . Read More

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Acquired hemophilia A associated with Epstein-Barr-virus-associated T/natural killer-cell lymphoproliferative disease: A case report.

Medicine (Baltimore) 2021 Apr;100(16):e25518

Department of Medicine, Division of Gastroenterology and Hematology/Oncology, Asahikawa Medical University.

Introduction: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Hematological malignancies, especially lymphoid malignancies, are known to be underlying causes of AHA; however, thus far, there is no report of AHA associated with Epstein-Barr-virus-associated T/natural killer-cell lymphoproliferative disease (EBV-T/NK-LPD). Here, we present a case of AHA that developed during treatment for EBV-T/NK-LPD. Read More

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Acquired haemophilia A in a patient with limited cutaneous systemic sclerosis.

Rheumatology (Oxford) 2021 Apr 20. Epub 2021 Apr 20.

Department of Rheumatology, Haga Hospital, the Hague, the Netherlands.

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Acquired haemophilia in patients with malignant disease: A case report.

World J Clin Cases 2021 Apr;9(10):2409-2418

Medical Faculty, University of Ljubljana, Ljubljana 1000, Slovenia.

Background: Acquired haemophilia is a rare coagulation disorder characterized by autoantibodies against coagulation factor VIII leading to severe and potentially life-threatening haemorrhages. The underlying disorder causing the development of an autoimmune phenomenon is not always known, but 10%-15% could be linked to malignancies. Patients with cancer who require surgical resection represent a treatment challenge not solely due to increased risk of bleeding but also due to adverse events of immunosuppressive therapy. Read More

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Pathological coagulation parameters in as many as 54 patients with autoimmune acquired factor XIII deficiency due to anti-factor XIII autoantibodies.

Haemophilia 2021 Apr 12. Epub 2021 Apr 12.

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan.

Introduction: Autoimmune factor XIII (FXIII) deficiency (AiF13D) due to anti-FXIII autoantibodies is an extremely rare, life-threatening bleeding disorder that mostly occurs in the elderly. The number of patients diagnosed with AiF13D has been increasing in Japan, probably because of the nationwide survey on AiF13D supported by the Japanese Ministry of Health, Labour and Welfare.

Aim: To explore the pathologic characteristics of coagulation parameters in AiF13D. Read More

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[Acquired hemophilia A with reappearance of inhibitor 10 years after the completion of immunosuppressive therapy].

Rinsho Ketsueki 2021 ;62(3):186-189

Department of Pediatrics, Suikokai Munakata General Hospital.

Acquired hemophilia A (AHA) is a disease that causes severe bleeding with the appearance of an inhibitor (INH) against blood coagulation factor VIII (FVIII). The prevalence of this condition is low; it occurs in only one in one to four million people per year; however, the number of diagnosed cases has increased in recent years owing to the greater awareness of the disease. It is noteworthy that this is a hemorrhagic disease that suddenly develops in the elderly. Read More

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Consensus on hemophilia in Mexico.

Gac Med Mex 2021 ;157(Suppl 1):S1-S35

Hematology department, Centro Médico Nacional 20 de Noviembre, ISSSTE, Mexico City. Mexico.

Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles, and elbows). Read More

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January 2021

Autoimmune Complications in Hematologic Neoplasms.

Cancers (Basel) 2021 Mar 26;13(7). Epub 2021 Mar 26.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. Read More

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Assessing Plasmin Generation in Health and Disease.

Int J Mol Sci 2021 Mar 9;22(5). Epub 2021 Mar 9.

Synapse Research Institute, 6217 KD Maastricht, The Netherlands.

Fibrinolysis is an important process in hemostasis responsible for dissolving the clot during wound healing. Plasmin is a central enzyme in this process via its capacity to cleave fibrin. The kinetics of plasmin generation (PG) and inhibition during fibrinolysis have been poorly understood until the recent development of assays to quantify these metrics. Read More

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The concept of rebalanced hemostasis in patients with liver disease: Communication from the ISTH SSC working group on hemostatic management of patients with liver disease.

J Thromb Haemost 2021 04;19(4):1116-1122

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

Patients with liver diseases acquire complex alterations in their hemostatic system that may lead to abnormalities in routine diagnostic test of hemostasis. Thrombocytopenia, prolongations in the prothrombin time and activated partial thromboplastin time, and decreased plasma fibrinogen are common in patients with advanced liver disease. Historically, liver diseases therefore have been classified as an acquired bleeding disorder. Read More

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A Case Report of Acquired Haemophilia following COVID-19 Vaccine.

J Thromb Haemost 2021 Mar 30. Epub 2021 Mar 30.

Department of Anesthesia and Critical Care, King AbdulAziz University Hospital, King Abdulaziz University, Jeddah, Saudi Arabia.

Acquires haemophilia A (AHA) is rare bleeding condition commonly associated with malignancy, autoimmune disease, or pregnancy. We report a case of a 69-year-old gentleman who developed bleeding symptoms after receiving COVID-19 vaccine. Laboratory testing showed isolated prolongation of the activated partial thromboplastin time, and normal Von-Willebrand factor. Read More

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Successful Treatment of Acquired Hemophilia A with Bortezomib Plus Low Dose Rituximab in a Patient with Renal Failure and Severe Pneumonia.

Indian J Hematol Blood Transfus 2021 Jan 3;37(1):171-172. Epub 2020 Jun 3.

Department of Hematology, Peking Union Medical College Hospital, Beijing, China.

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January 2021

Hematological immune related adverse events after treatment with immune checkpoint inhibitors.

Eur J Cancer 2021 Apr 9;147:170-181. Epub 2021 Mar 9.

Department of Dermatology, University Hospital Kiel, Kiel, Germany.

Introduction: With the increasing use of checkpoint inhibitors, rare immune-related adverse events (irAE) are being identified. Haematological irAE (hem-irAE) are difficult to treat and have shown high mortality rates. In order to improve side-effect management for these potentially life-threatening events, we analysed frequency, severity and outcomes. Read More

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Two case reports of acquired haemophilia A as complications of alemtuzumab treatment for multiple sclerosis.

BMJ Neurol Open 2021 18;3(1):e000095. Epub 2021 Jan 18.

Neurology, Royal Hobart Hospital, Hobart, Tasmania, Australia.

Objective: To describe the case histories of two patients who developed acquired haemophilia A following treatment with alemtuzumab for multiple sclerosis.

Results: Two patients, a 48-year-old woman and a 31-year-old woman, developed acquired haemophilia A 21 months after their second doses of alemtuzumab. Both presented with spontaneous bruising, and the second case reported menorrhagia. Read More

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January 2021

Activated Prothrombin Complex Concentrate-Induced Atypical Hemolytic Uremic Syndrome Treated with Eculizumab.

Am J Case Rep 2021 Mar 8;22:e929616. Epub 2021 Mar 8.

Department of Hematology and Oncology, Veterans Affairs Loma Linda Healthcare System, Loma Linda, CA, USA.

BACKGROUND Atypical hemolytic uremic syndrome (aHUS) is a set of heterogenous disorders of thrombotic microangiopathy defined by thrombocytopenia, hemolytic anemia, and acute renal failure that is not mediated by shiga toxin. Factor Eight Inhibitor Bypassing Activity (FEIBA) is a concentrate of inactivated and activated coagulation factors that is approved for use to establish hemostasis in patients with hemophilia or acquired factor inhibitors. However, it has recently been used off-label as an anticoagulant reversal therapy among the general population. Read More

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von Willebrand disease: what does the future hold?

Blood 2021 Apr;137(17):2299-2306

Laboratory for Hemostasis, Inflammation & Thrombosis, Unité Mixed de Recherche S1176, Institut National de la Santé et de la Recherche Médicale, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

von Willebrand disease (VWD) is characterized by its heterogeneous clinical manifestation, which complicates its diagnosis and management. The clinical management of VWD has remained essentially unchanged over the last 30 years or so, using von Willebrand factor (VWF) concentrates, desmopressin, and anti-fibrinolytic agents as main tools to control bleeding. This is in contrast to hemophilia A, for which a continuous innovative path has led to novel treatment modalities. Read More

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Acquired Hemophilia A in Wiskott-Aldrich Syndrome.

J Clin Immunol 2021 Mar 3. Epub 2021 Mar 3.

Department of Rheumatology and Immunology, Shenzhen Children's Hospital, China Medical University, 7019 Yitian Road, Shenzhen, 518038, China.

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Hemostatic therapy as a management strategy for acquired hemophilia: what does the future hold?

Expert Rev Hematol 2021 Mar 3;14(3):263-270. Epub 2021 Mar 3.

Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy.

: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies that bind and inactivate factor VIII (FVIII), predisposing to a potentially life-threatening bleeding.: The main epidemiological, clinical, laboratory and therapeutic features of AHA are critically discussed. In particular, we focus on the hemostatic management of AHA patients analyzing the currently available treatment options and showing the latest data on the innovative hemostatic agents still under investigation. Read More

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Factor VIII/IX inhibitor testing practices in the United Kingdom: Results of a UKHCDO and UKNEQAS national survey.

Haemophilia 2021 Mar 2. Epub 2021 Mar 2.

The Royal London Hospital Haemophilia Centre, Barts and The London School of Medicine and Dentistry, QMUL, London, UK.

Introduction: Inhibitor formation is the greatest challenge facing persons with haemophilia treated with factor concentrates. The gold standard testing methodologies are the Nijmegen-Bethesda assay (NBA) for FVIII and Bethesda assay (BA) for FIX inhibitors, which are affected by pre-analytical and inter-laboratory variability.

Aims: To evaluate inhibitor testing methodology and assess correlation between self-reported and actual methodology. Read More

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Potential Clues to the Pathogenesis of Acquired Hemophilia.

Authors:
Jecko Thachil

Semin Thromb Hemost 2021 Mar 1. Epub 2021 Mar 1.

Department of Haematology, Manchester University Hospitals, Manchester, United Kingdom.

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Ex Vivo Prediction of Comprehensive Coagulation Potential Using Simulated Blood Concentrations of Emicizumab in Patients with Acquired Hemophilia A.

Thromb Haemost 2021 Feb 28. Epub 2021 Feb 28.

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

Introduction:  Emicizumab prophylaxis improves coagulation function in congenital hemophilia A, regardless of inhibitor presence. We recently reported that emicizumab enhanced the coagulant potentials, ex vivo in plasmas from patients with acquired hemophilia A (PwAHAs) at diagnosis. However, coagulant effects of emicizumab in PwAHAs during the clinical course remain unclear. Read More

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February 2021

[Acquired hemophilia A: clinical and biological characteristics and therapeutic management of a series of eight patients hospitalized in Lariboisière and Saint-Louis hospitals].

Ann Biol Clin (Paris) 2021 Feb;79(1):75-82

Service d'hématologie biologique, CRMR des MAT-laboratoire ADAMTS13, CRMR de la Maladie de Willebrand, DMU BioGEM, Hôpital Lariboisière, AP-HP Nord, Université de Paris, Paris, France, EA-3518 Recherche clinique en hématologie, immunologie et transplantation, Groupe MicroAngiopathies Thrombotiques, VWF et ADAMTS13, Institut de recherche Saint-Louis, Université de Paris, Paris, France.

Acquired hemophilia A is a rare autoimmune disease, linked to the appearance of autoantibodies directed against circulating factor VIII, and characterized by a major hemorrhagic syndrome. Acquired hemophilia A is a life-threatening diagnostic and therapeutic medical emergency. We describe here the cohort of patients with acquired hemophilia A treated between 2015 and 2020 at Lariboisière and Saint-Louis University Hospitals (Paris, France). Read More

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February 2021

Acquired hemophilia in a 7-year-old girl successfully treated with recombinant FVIIA and steroids: A case report.

Clin Case Rep 2021 Feb 3;9(2):638-643. Epub 2020 Dec 3.

Division of Hematology AOU "Policlinico - Vittorio Emanuele" Catania Italy.

Acquired hemophilia should be evaluated in pediatric patients with bleeding and isolated prolonged aPTT. Immunosuppressive treatment should be initiated even in minor bleedings. Bypassing agents like rFVIIa can be used in children with success. Read More

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February 2021

Acquired Hemophilia A in an Elderly Patient on Apixaban Therapy.

Isr Med Assoc J 2021 Feb;23(2):130-131

Department of Hematology, Shaare Zedek Medical Center, Jerusalem, Israel.

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February 2021

Idiopathic Acquired Hemophilia A, a Rare Cause of Bleeding: A Case Report and Literature Review.

Am J Case Rep 2021 Feb 17;22:e929401. Epub 2021 Feb 17.

Department of Hematology, University of Antioquia, Medellín, Colombia.

BACKGROUND Acquired hemophilia is a bleeding disorder mediated by an autoimmune process, in which antibodies against clotting factors are developed. This is a rarely suspected complex condition in which the initial manifestations are spontaneous bleeding in the skin, soft tissues, and mucosa in patients with no known history of bleeding disorders. Most of the cases are idiopathic (50%), but it can be associated with autoimmune diseases, malignancy, pregnancy, and medications. Read More

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February 2021