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acquired Haemophilia

Lawless Sarah, Das Prantik, Benson Gary
A cornerstone of the management of Acquired Haemophilia A (AHA) involves inhibitor eradication. First line immunosuppressive agents are usually steroids, either alone or in combination with cyclophosphamide. We present the use of Rituximab, cyclophosphamide, vincristine and prednisolone (RCVP) combination as immunosuppressant in AHA in a small cohort of patients in order to control their symptoms and eradicate inhibitors. This was a retrospective analysis of all AHA patients treated at the Northern Ireland Haemophilia centre over a six year period...
September 2016: Ulster Medical Journal
Julie Omolola Okiro, Amjad Zaman Khan, Fergus Keane, Faiza Murad
A 72-year-old man, on treatment for prostate cancer, attended the emergency department with his 2nd episode of spontaneous extensive bruising and haematomas. His first presentation was 2 months prior but this was thought to be because of his aspirin and he improved when aspirin was discontinued. On this occasion aspirin had been restarted 7 days before he developed his symptoms. His blood investigation was significant for a much raised activated partial thromboplastin time (aPTT). On his 3rd day of admission he deteriorated clinically with a drastic drop in his haemoglobin and worsening tense haematomas...
2016: BMJ Case Reports
Anna See, Siti Radhziah Sudirman, Xin Yong Huang
BACKGROUND: Acquired haemophilia A is caused by the development of an autoantibody to factor VIII in a person with previously normal haemostasis. The most common clinical presentation in hereditary haemophilia is intra-articular bleeding. In contrast, acquired haemophilia more commonly presents with skin, soft tissue and mucosal haemorrhages. METHODS: We present a case of a patient with idiopathic acquired haemophilia A, whose initial presentation was that of spontaneous submental and submandibular haematoma which rapidly progressed to involve multiple sites in the airway, ultimately necessitating an emergent tracheotomy...
August 30, 2016: European Archives of Oto-rhino-laryngology
M D Tarantino, A Cuker, B Hardesty, J C Roberts, M Sholzberg
INTRODUCTION: A recombinant porcine factor VIII B-domain-deleted product (rpFVIII; OBIZUR, Baxalta Incorporated, Deerfield, IL 60015, USA) was recently approved for treatment of bleeding episodes in adults with acquired haemophilia A (AHA) in the United States. To date, no clinical experience outside the registration study has been reported. AIM: To describe early clinical experience using rpFVIII for AHA. METHODS: A retrospective chart review of seven patients with AHA treated with rpFVIII at four institutions from November 2014 to October 2015...
August 10, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
K Amano, I Seita, S Higasa, A Sawada, M Kuwahara, M Shima
INTRODUCTION: Patients with acquired haemophilia A (AHA) have autoantibodies against factor VIII (FVIII), and may develop spontaneous bleeding that requires treatment with FVIII inhibitor bypassing agents such as recombinant activated FVII (rFVIIa, NovoSeven(®) ). However, data regarding the use of rFVIIa are limited. AIM: To investigate the use, efficacy and safety of rFVIIa for the treatment of AHA by analysis of 10-year multicentre Japanese postmarketing surveillance data...
July 25, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Paul R J Ames, Daniel Montero, Jeremy Archer
No abstract text is available yet for this article.
June 2016: Indian Journal of Hematology & Blood Transfusion
M Shima, D Lillicrap, R Kruse-Jarres
The development of inhibitors to factor VIII (FVIII) or factor IX (FIX) remains a major treatment complication encountered in the treatment of haemophilia. Not all patients with even the same severity and genotype develop inhibitors suggesting an underlying mechanism of tolerance against FVIII- or FIX-related immunity. One mechanism may be central tolerance observed in patients in whom the FVIII mutation enables some production of the protein. The other is a peripheral tolerance mechanism which may be evident in patients with null mutation...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Yan Zeng, Ruiqing Zhou, Xin Duan, Dan Long
BACKGROUND: Acquired haemophilia A is a rare bleeding disorder caused by the development of specific autoantibodies against coagulation factor VIII. Rituximab may be an alternative approach to the treatment of acquired haemophilia by eradicating FVIII autoantibodies. OBJECTIVES: To assess and summarise the efficacy and adverse effects of rituximab for treating people with acquired haemophilia A. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's trials registers, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings...
2016: Cochrane Database of Systematic Reviews
Keiji Nogami
Thromboelastography (TEG) was first described by Hartert in 1948, and was designed to monitor viscoelastic clot strength in whole blood in real time. The current TEG method and Rotational Thromboelastometry (ROTEM) were subsequently developed from the original principles. Both of the modern methods provide data by measuring changes in the viscoelastic strength of a small sample of clotting blood in response to a constant rotational force. The important advantage of these techniques is to visually observe and quantify blood coagulation including the propagation, stabilization and dissolution phases of clot formation under low shear conditions...
August 2016: British Journal of Haematology
A V Pathare, S Ghamande, S Fernandes, S Bhatia, F Jijina, S Shetty, K Ghosh, D Mohanty
The present report describes two unusual cases of bleeding due to development of inhibitor against factor VIII. One of the patients was known to have severe haemophilia B (factor IX < 1%) and the other, a healthy male of 46 years, spontaneously started to bleed and was found to have developed inhibitors to factor VIII:C along with other autoantibodies. Development of inhibitors to coagulation factors is a serious clinical problem in de-veloped countries and is more so in developing countries where treatment options are often limited...
July 1997: Haemophilia: the Official Journal of the World Federation of Hemophilia
R Gupta, E M Druy, C M Kessler
Chronic liver disease has affected a majority of haemophiliacs exposed to factor VIII and factor IX concentrates before effective viral attenuation techniques were introduced. Progressive hepatitis and liver failure have increasingly become causes of morbidity, especially in haemophilia patients also seropositive for HIV. Encouraging reports of recombinant alpha interferon to reverse the histological, laboratory and clinical deterioration of chronic active hepatitis justifies obtaining liver biopsies to assess the severity of liver damage; however, the percutaneous method carries a significant risk of bleeding...
July 1997: Haemophilia: the Official Journal of the World Federation of Hemophilia
E Di Bona, M Schiavoni, G Castaman, N Ciavarella, F Rodeghiero
Acquired haemophilia is a rare but often catastrophic haemorrhagic disorder associated with a high mortality rate. No single therapeutic approach has been consistently successful and clinical experience remains mainly anecdotal. This report describes 17 new cases diagnosed at two Italian haemophilia centres between 1979 and 1995. There was no difference in sex distribution. Mean age at diagnosis was 50 years. Fifty-nine per cent of cases had associated disorders and 29% developed an inhibitor post-partum. Eleven (64%) patients required substitutive therapy...
July 1997: Haemophilia: the Official Journal of the World Federation of Hemophilia
K Maliekel, N Rana, D Green
Bleeding in nonhaemophilic patients with high-titre factor VIII autoantibodies is often severe, life-threatening and refractory to treatment with factor VIII concentrates. In this report, we describe an elderly woman who required surgical excision of a large haemophilic pseudotumour adjacent to the left gluteal muscle. The Bethesda titre of 11 U precluded treatment with human factor VIII, and the patient had an anaphylactic reaction to porcine factor VIII. However, haemostasis was successfully achieved with recombinant factor VIIa and the pseudotumour was removed...
January 1997: Haemophilia: the Official Journal of the World Federation of Hemophilia
C R Hay, C A Lee, G Savidge
The identification of infrequent side-effects of clotting factor concentrates, undetected by clinical trials, is facilitated by post-marketing surveillance. We present a post-marketing surveillance study in which 97 patients with haemophilia A, attending three haemophilia centres, were treated over a median follow-up period of 284 days (range 1-1074), and a total follow-up period of 30,080 days, with a pasteurized immunoaffinity purified factor VIII concentrate (Monoclate-P, Armour, Collegeville, USA). 5216 infusions, using 10,527,000 units of Monoclate-P, were carried out, mostly for routine haemarthroses or prophylaxis...
January 1996: Haemophilia: the Official Journal of the World Federation of Hemophilia
(no author information available yet)
No abstract text is available yet for this article.
December 1997: Haemophilia: the Official Journal of the World Federation of Hemophilia
Celeste B Burness, Lesley J Scott
No abstract text is available yet for this article.
May 10, 2016: Drugs
Celeste B Burness, Lesley J Scott
Intravenous susoctocog alfa (Obizur(®)) is a recombinant, B-domain deleted, porcine sequence antihaemophilic factor VIII (FVIII) product that has recently been approved for the treatment of bleeding episodes in adults with acquired haemophilia A (AHA). Intravenous susoctocog alfa was an effective and generally well tolerated treatment for serious bleeding episodes in adult patients with AHA in a multinational, phase II/III trial (n = 28 evaluable). Patients received an initial dose of susoctocog alfa 200 U/kg, with subsequent dosages based on target FVIII trough levels and clinical assessments...
May 2016: Drugs
Liesbeth Schrijvers, Marlène Beijlevelt-Van der Zande, Marjolein Peters, Janske Lock, Marjon Cnossen, Marieke Schuurmans, Kathelijn Fischer
OBJECTIVE: Adolescents with a chronic disorder, such as haemophilia, need to attain responsibility for their disease. The aim was to gain insight into how adolescents achieve self-management of prophylactic treatment. METHODS: In three Dutch Haemophilia Treatment Centres, adolescents (10-25 years) received structured questions on treatment responsibility and self-management (pre-specified definitions) during routine nursing consultation. RESULTS: In total, 155 interviews were performed in 100 patients (median age 14...
July 2016: Patient Education and Counseling
Avraneel Talapatra, Michael J Nash, Charles R M Hay, Jecko Thachil
Acquired Haemophilia (AH) is an autoimmune bleeding disorder, which despite being rare, can be fatal. It occurs in patients with previously normal haemostasis who spontaneously develop IgG autoantibodies against factor VIII. Unlike congenital haemophilia, it manifests as spontaneous bleeding into skin and soft tissues. The presentation can be masked in patients who are receiving warfarin where the bleeding is often attributed to warfarin therapy, as in the case described in this report. Consideration of AH is important in patients taking anticoagulants, when coagulopathy and bleeding fails to correct with usual measures...
2015: Acute Medicine
Rory McDonald, Anant Piyush Patel, Claudine Horrocks
Epistaxis is a potentially life-threatening condition that can compromise a patient's airway, breathing and circulation. Early recognition of underlying bleeding diatheses that are amenable to medical therapy is imperative in order for timely treatment and to reduce morbidity. We present a case in which acquired haemophilia was diagnosed in an 83-year-old man presenting with seemingly uncomplicated epistaxis. The patient suffered multisite haemorrhages and haemodynamic compromise before definitive management with monoclonal antibody therapy...
2015: BMJ Case Reports
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