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acquired hemophilia

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https://www.readbyqxmd.com/read/27902587/influence-of-factor-viii-level-and-its-inhibitor-titer-on-the-therapeutic-response-to-corticosteroids-alone-in-the-management-of-acquired-hemophilia-a-retrospective-single-center-study
#1
Mathieu Vautier, Hubert de Boysson, Christian Creveuil, Yohan Repesse, Annie Borel-Derlon, Xavier Troussard, Gandhi L Damaj, Boris Bienvenu, Philippe Gautier, Achille Aouba
The treatment of acquired hemophilia (AH) involves discussing whether corticosteroids should be administered alone or combined with immunosuppressant drugs, which increase the risk of infection especially in elderly patients and/or those with autoimmunity or neoplastic diseases, who represent the target population of the disease. Prognostic factors highlighting adequate responses to corticosteroids alone must be identified for satisfactory clinical response and lower infectious risk.We aimed to evaluating the efficacy of corticosteroids alone in the management of AH depending on factor VIII (FVIII, ≥ or <1 IU/dL) levels and/or inhibitor (INH, ≤ or >20 Bethesda units per milliliter [BU/mL]) titer...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27885231/two-elderly-patients-with-difficult-to-treat-acquired-hemophilia-a
#2
Makoto Saito, Masanobu Morioka
We herein report the cases of two elderly patients with acquired hemophilia A (AHA) for whom treatment was difficult.An 89-year-old woman (Case 1) was admitted to our department with subcutaneous hemorrhage and melena. Her activated partial thromboplastin time (APTT), factor VIII activity, and factor VIII inhibitor level were 127.7 seconds, 1.0%, and 48 BU/mL, respectively, which was suggestive of AHA. The administration of prednisolone (PSL 0.5 mg/kg) was initiated. After 3 weeks, PSL was combined with cyclophosphamide (CPA 50 mg)...
2016: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/27868097/percutaneous-nephrolithotomy-in-rare-bleeding-disorders-a-case-report-and-review-of-the-literature
#3
Ali Ersin Zumrutbas, Cihan Toktas, Aykut Baser, Omer Levent Tuncay
Surgery in patients with congenital or acquired coagulation defects has always been challenging and requires special care with a multidisciplinary approach. Percutaneous nephrolithotomy (PCNL) is a standard procedure performed in patients with kidney stones. Although prone to bleeding more than most of the widely performed surgical procedures, there are not much data regarding PCNL in patients with bleeding disorders or coagulation defects. There are only case reports or series with a small number of patients for the patients with common coagulation defects, including hemophilias...
2016: Journal of Endourology Case Reports
https://www.readbyqxmd.com/read/27853081/unusual-initial-manifestation-of-acquired-hemophilia-a-a-normal-activated-partial-thromboplastin-time-intramuscular-hematoma-and-cerebral-hemorrhage
#4
Nobuaki Tsuyama, Toshihisa Ichiba, Hiroshi Naito
We herein present a case of acquired hemophilia A with a normal activated partial thromboplastin (aPTT), intramuscular hematoma and cerebral hemorrhage occurring in a 73-year-old man. The patient visited our emergency department with gait disturbance, pain and swelling in his right leg. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed intramuscular hematoma and intracranial hemorrhage. The results of initial coagulation studies were normal, but repeated coagulation studies revealed an isolated prolongation of the aPTT...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27843336/treatment-of-bleeding-in-acquired-hemophilia-a-with-the-proper-administration-of-recombinant-activated-factor-vii-single-center-study-of-7-cases
#5
Makoto Saito, Minoru Kanaya, Koh Izumiyama, Akio Mori, Tatsuro Irie, Masanori Tanaka, Masanobu Morioka, Masahiro Ieko
Recombinant activated factor VII (rFVIIa) is the bypassing agent used in the first-line hemostatic therapy for acquired hemophilia A (AHA); however, the occurrence of thrombotic complications in rFVIIa-treated AHA patients was recently reported to be 2.9-6.5%. Therefore, the investigation of the proper administration of rFVIIa for AHA is needed. In the present study, we retrospectively investigated the clinical features of AHA with regards to the use of rFVIIa (presence or absence of use and total amount) in 7 AHA patients encountered in our department for 7 years between January 2008 and December 2014...
2016: International Journal of General Medicine
https://www.readbyqxmd.com/read/27812813/-coagulation-management-in-geriatric-surgery
#6
H Eichler
BACKGROUND: Elderly patients often suffer from cardiovascular diseases and are treated with anticoagulation medications, which must be taken into consideration when planning elective surgery. OBJECTIVE: The etiology, diagnostic work-up and clinical management of selected inherited and acquired hemophilic and thrombophilic coagulation disorders are described. METHODS: Data from clinical studies, current guidelines and expert opinions are discussed...
November 3, 2016: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/27799968/evaluation-of-aryoseven-safety-recombinant-activated-factor-vii-in-patients-with-bleeding-disorders-an-observational-post-marketing-surveillance-study
#7
Gholamreza Toogeh, Hassan Abolghasemi, Peyman Eshghi, Mohammadreza Managhchi, Mohammadreza Shaverdi-Niasari, Katayoon Karimi, Samin Roostaei, Neda Emran, Alireza Abdollahi
BACKGROUND: Recombinant activated factor VII induces hemostasis in patients with coagulopathy disorders. AryoSeven™ as a safe Iranian Recombinant activated factor VII has been available on our market. This study was performed to establish the safety of AryoSeven on patients with coagulopathy disorder. METHODS: This single-center, descriptive, cross sectional study was carried out in Thrombus and Homeostasis Research Center ValiAsr Hospital during 2013-2014. Fifty one patients with bleeding disorders who received at least one dose of Aryoseven were enrolled...
2016: Iranian Journal of Pathology
https://www.readbyqxmd.com/read/27795652/-antihemophilic-factor-is-not-the-only-answer-for-all-factor-viii-deficiencies-case-report-of-odontogenic-infection-in-a-patient-with-hemophilia-a-complicated-by-factor-viii-inhibitors-and-managed-by-transfusion-of-antihemophilic-factor-and-factor-viii-inhibitor
#8
K M Sudheesh, K S N Siva Bharani, H Y Kiran, Suresh Hanagavadi
Dental extraction in hemophiliacs with acquired inhibitors is always a risky procedure, which often presents a lot of problems associated with bleeding. A known case of hemophilia A complicated with factor VIII inhibitors and having odontogenic infection was successfully managed by transfusion of factor VIII inhibitor bypass activity (FEIBA) and antihemophilic factor. Past medical history was significant for multiple factor VIII transfusions. Bethesda assay done to identify inhibitors revealed low titer factor VIII inhibitors...
September 2016: Indian Journal of Dentistry
https://www.readbyqxmd.com/read/27730530/a-combined-approach-using-global-coagulation-assays-quickly-differentiates-coagulation-disorders-with-prolonged-aptt-and-low-levels-of-fviii-activity
#9
Tomoko Matsumoto, Keiji Nogami, Midori Shima
Patients with mild/moderate hemophilia (H)A, acquired HA (AHA) and lupus anticoagulants (LA), have prolonged aPTTs with low levels of factor (F)VIII activity, but the differentiation of these disorders is complex and time consuming. We established an approach to quickly differentiate these disorders using comprehensive coagulation tests. Patients' plasmas with mild/moderate HA, AHA, LA without anti-phospholipid syndrome [LA-APS(-)], and LA with APS [LA-APS(+)] were examined using clot waveform analysis (CWA) and thrombin generation test (TGT)...
October 11, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27701158/acquired-fviii-and-fix-inhibitors-after-pregnancy-a-case-report
#10
Murat Kose, Oguz Kagan Bakkaloglu, Shirkhan Amikishiyev, Timur Selcuk Akpınar, Basak Saracoglu, Tugce Akcan, Melike Oktem, Mustafa Nuri Yenerel, Kerim Güler, Tufan Tükek
Acquired hemophilia is a relatively rare clinical presentation, and most cases present with acquired FVIII inhibitor. The co-occurrence of inhibitors to multiple coagulation factors is uncommon. These autoantibodies may induce spontaneous life-threatening bleeding in patients who have had no previous bleeding disorder. Herein, we present a patient with postpartum acquired FVIII and FIX inhibitors who developed intramuscular hematoma and hemothorax during follow-up. She was then treated with activated prothrombin complex concentrate and methylprednisolone...
2016: Acta Haematologica
https://www.readbyqxmd.com/read/27685947/baboon-envelope-pseudotyped-lentiviral-vectors-efficiently-transduce-human-b-cells-and-allow-active-factor-ix-b-cell-secretion-in-vivo-in-nod-scid%C3%AE-c-mice
#11
C Levy, F Fusil, F Amirache, C Costa, A Girard-Gagnepain, D Negre, O Bernadin, G Garaulet, A Rodriguez, N Nair, T Vandendriessche, M Chuah, F-L Cosset, E Verhoeyen
: Essentials B cells are attractive targets for gene therapy and particularly interesting for immunotherapy. A baboon envelope pseudotyped lentiviral vector (BaEV-LV) was tested for B-cell transduction. BaEV-LVs transduced mature and plasma human B cells with very high efficacy. BaEV-LVs allowed secretion of functional factor IX from B cells at therapeutic levels in vivo. SUMMARY: Background B cells are attractive targets for gene therapy for diseases associated with B-cell dysfunction and particularly interesting for immunotherapy...
September 29, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27660505/acquired-hemophilia-a-may-be-associated-with-ticagrelor-therapy-in-a-52-year-old-man-after-a-recent-percutaneous-transluminal-coronary-angioplasty
#12
Paola Pasquino, Roberto Canaparo, Tiziana Capello, Barbara Deorsola, Laura Perazzolo, Claudio Marengo, Loredana Serpe
We present a case report of a 52-year-old man who was hospitalized for right leg pain due to a relevant hemorrhagic effusion. He was on dual antiplatelet therapy (DAPT): acetylsalicylic acid and ticagrelor, a reversible P2Y12 receptor antagonist. Signs, symptoms, and laboratory blood tests led to the diagnosis of acquired hemophilia A (AHA). Ticagrelor therapy-associated AHA was hypothesized due to the fact that, before adding this drug, all laboratory and clinical examinations were repeatedly normal. Prednisone and cyclophosphamide treatment was started without DAPT interruption due to the high risk of stent thrombosis...
2016: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/27658429/biophysical-tools-to-assess-the-interaction-of-pf4-with-polyanions
#13
Mihaela Delcea, Andreas Greinacher
The antigen in heparin-induced thrombocytopenia (HIT) is expressed on platelet factor 4 (PF4) when PF4 complexes with polyanions. In recent years, biophysical tools (e. g. circular dichroism spectroscopy, atomic force microscopy, isothermal titration calorimetry, x-ray crystallography, electron microscopy) have gained an important role to complement immunological and functional assays for better understanding the interaction of heparin with PF4. This allowed identification of those features that make PF4 immunogenic (e...
October 28, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27646211/the-tipping-point-the-critical-role-of-therapeutic-apheresis-in-a-case-of-refractory-acquired-hemophilia
#14
Michael Losos, Scott Scrape, Sarita Joshi, Aaron Shmookler, Jian Chen
Acquired hemophilia A (AHA) is a rare autoimmune disorder that leads to factor VIII (FVIII) deficiency via autoantibody formation. Standard treatment options include FVIII bypassing factors and immunosuppression. However, the role of therapeutic plasma exchange (TPE) is not clear in the treatment of AHA. We present a case of idiopathic AHA in a 66 year old female with severe bleeding and a FVIII inhibitor of 17.6 Bethesda units (BU). She failed to respond to standard treatment including maximum dose of recombinant FVIIa (rFVIIa), rituximab, and other immunosuppressive agents...
September 20, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27609734/life-threatening-hemorrhage-from-acquired-hemophilia-a-as-a-presenting-manifestation-of-prostate-cancer
#15
Chirag Sheth, Amandeep Gill, Sumeet Sekhon
Acquired factor VIII deficiency (acquired hemophilia A) is a rare condition characterized by the acquisition of autoantibodies that affect the clotting activity of factor VIII (fVIII). The most common manifestation in affected patients is a hemorrhagic diathesis. This disorder is associated with autoimmune diseases, pregnancy, postpartum period, drugs, and malignancy. Management of this condition begins with attempts to arrest an acute bleed based on the site and severity of bleeding and inhibitor titer. The next priority is eradication of the fVIII antibodies using immunosuppressive therapies...
2016: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/27576087/use-and-future-investigations-of-recombinant-and-plasma-derived-coagulation-and-anticoagulant-products-in-the-neonate
#16
REVIEW
Rowena C Punzalan, Jerome L Gottschall
Although congenital bleeding disorders can manifest in the newborn period, the most common causes of bleeding and thrombosis in neonates are acquired conditions. Factor concentrates are used for specific diagnoses (hemophilia with inhibitors, specific factor deficiency, von Willebrand disease) and approved indications, and increasingly for off-label indications (bleeding in surgery cardiopulmonary bypass, extracorporeal membrane oxygenation). We will review the approved indications for factor products in the neonate and discuss the evidence and rationale for off-label use of factor products in management of bleeding and thrombosis in the neonate...
October 2016: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/27545905/acquired-hemophilia-a-that-developed-during-the-induction-of-hemodialysis-the-use-of-double-filtration-plasmapheresis%C3%A2
#17
Aya Imafuku, Naoki Sawa, Yoshifumi Ubara, Kenmei Takaichi
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). AHA onset during the induction of dialysis is extremely rare, and the management of blood access is difficult. We present a case of AHA that developed during induction of dialysis and treatment with double filtration plasmapheresis (DFPP). An 86-year-old man with chronic kidney disease was admitted to our hospital with multiple subcutaneous hemorrhages. Because of his prolonged activated partial thromboplastin time (aPTT) and high titer of inhibitors to FVIII, he was diagnosed with AHA, and prednisolone treatment was started...
August 22, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27542511/autoimmune-acquired-factor-xiii-deficiency-due-to-anti-factor-xiii-13-antibodies-a-summary-of-93-patients
#18
Akitada Ichinose
Autoimmune acquired factor XIII (F13) deficiency or autoimmune hemophilia-like disease (hemorrhaphilia) resulted from the generation of anti-F13 antibodies (AH13) is a severe bleeding disorder that occurs mainly in the elderly. Although rare, the number of patients diagnosed with AH13 has recently increased. To improve understanding of this disease, the author summarized 93 ever reported/diagnosed AH13 cases. About 50% of cases were idiopathic. In the remaining half of the patients, autoimmune diseases and malignancies were the most common underlying diseases...
August 11, 2016: Blood Reviews
https://www.readbyqxmd.com/read/27511892/coexistence-of-acquired-hemophilia-a-and-epidermolysis-bullosa-acquisita-two-case-reports-and-published-work-review
#19
Tian-Meng Yan, Chun-Xia He, Bao-Lai Hua, Li Li, Hong-Zhong Jin, Yue-Hua Liu, Ya-Gang Zuo
Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients...
August 11, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27467981/assessment-of-acquired-hemophilia-patient-demographics-in-the-united-states-the-hemostasis-and-thrombosis-research-society-registry
#20
Craig M Kessler, Alice D Ma, Hamid A B Al-Mondhiry, Robert Z Gut, David L Cooper
The Hemostasis and Thrombosis Research Society (HTRS) Registry was used to monitor the postapproval use of recombinant factor VIIa. The objective of this manuscript is to provide key insights on the demographics of patients with acquired hemophilia in the HTRS Registry. Acquired hemophilia patient registration in HTRS captured age; sex; comorbidities and predisposing conditions; first bleeding location; laboratory parameters; exposure to blood products, factor, and bypassing agents; and initiation of immune suppression/tolerance therapy...
October 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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