Read by QxMD icon Read

acquired hemophilia

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
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...
October 5, 2016: Acta Haematologica
C Levy, F Fusil, F Amirache, C Costa, A Girard, D Negre, O Bernadin, G Garaulet, A Rodriguez, N Nair, T Vandendriessche, M Chuah, F-L Cosset, E Verhoeyen
BACKGROUND: B cells are attractive targets for gene therapy of diseases associated with B-cell dysfunction and particularly interesting for immunotherapy. Moreover, B cells are potent protein-secreting cells and can be tolerogenic antigen presenting cells. OBJECTIVE: Evaluation of human B cells for secretion of clotting factors such as factor IX (IX) as possible treatment for hemophilia. METHODS: We tested here for the first time our newly developed baboon envelope pseudotyped lentiviral vectors (BaEV-LVs) for human (h) B-cell transduction followed their adaptive transfer into NSG mouse...
September 29, 2016: Journal of Thrombosis and Haemostasis: JTH
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
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...
September 22, 2016: Thrombosis and Haemostasis
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
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
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
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
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
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
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
John Chapin, Jaqueline Bamme, Fraustina Hsu, Paul Christos, Maria DeSancho
Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed...
July 14, 2016: Clinical and Applied Thrombosis/hemostasis
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
Serious issues in current hemostatic treatment of hemophilia A are the requirement for frequent intravenous administrations of factor (F) VIII, FVIII inhibitor development, and hemostatic treatment for patients with this inhibitor. For the purpose of overcoming these challenges, the FVIIIa-substituting bispecific antibody against FIXa/FX (ACE910, INN emicizumab) was produced. Emicizumab demonstrated marked hemostatic effects on both ongoing and spontaneous joint bleeding in the acquired hemophilia A primate model...
June 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Keiji Nogami
There are some issues in the current factor (F)VIII replacement therapy for severe hemophilia A. One is mental and physical burden for the multiple intravenous infusions, and the other is difficulty in the hemostatic treatment for the patients with FVIII inhibitor. The development of novel drug with fully hemostatic effect, simply procedure, and long-acting reaction has been expected. Recently, FVIIIa-mimicking humanized recombinant bispecific antibody (ACE910) against FIXa and FX was developed. In the non-human clinical study, primate model of acquired hemophilia A demonstrated that the ACE910 was effective on both on-going and spontaneous bleedings...
May 2016: Thrombosis Research
Osamu Kumano, Masahiro Ieko, Sumiyoshi Naito, Mika Yoshida, Nobuhiko Takahashi, Takeshi Suzuki, Yutaka Komiyama
INTRODUCTION: Lupus anticoagulant (LA) is an antibody that interferes with in vitro coagulation reactions. The mixing test is considered useful for LA diagnosis and is also recommended to differentiate between acquired hemophilia A (AHA) and factor deficiency. However, there has been little study to differentiate between LA and AHA. Our aims are to investigate whether we can differentiate LA and AHA by the mixing test and to establish new formulas for the mixing test to differentiate these samples clearly...
July 2016: Thrombosis Research
Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Chiaki Naito, Masahiro Mihara, Hiroshi Handa, Reiko Shizuka, Madoka Inoue, Sumiyoshi Naito, Masahiro Ieko, Akitada Ichinose, Yoshihisa Nojima
Acquired hemophilia A (AHA) is a rare coagulation disorder caused by autoantibodies against coagulation factor VIII (FVIII). We report herein a very rare case of AHA complicated by immune thrombocytopenia (ITP). A 30-year-old woman was hospitalized with severe thrombocytopenia. Her platelet count was 5,000/μl on admission, at which time APTT was normal. ITP was diagnosed and she was treated with γ-globulin, platelet transfusion, and prednisolone at 1 mg/kg/day. She was discharged after platelet count normalization and prednisolone was tapered to 5 mg/day...
April 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Kunio Yanagisawa, Yoshiyuki Ogawa, Takeki Mitsui, Hiroyuki Noguchi, Hiroaki Shimizu, Takuma Ishizaki, Hiroshi Handa, Masahiro Ieko, Akitada Ichinose, Yoshihisa Nojima
Although acquired hemophilia A (AHA) often develops in patients with neoplasms, there are few reports on the efficacy of radiation therapy during the bleeding phase of AHA in the prior literature. We herein present a case of AHA experiencing remission soon after radiation therapy for esophageal cancer. A man in his seventies, who had a history of radical nephrectomy for left renal cell carcinoma, received a diagnosis of esophageal cancer. Three months later, he noticed a right thigh hematoma, and was transferred to our hospital...
April 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Marie Wrobel, Emilie Comio, Valerie Gay, Noureddine Baroudi, Pascal Meyer, Christine Chuniaud-Louche, Maya Hacini, Gian Matteo Pica
BACKGROUND: Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described. CASE PRESENTATION: A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer; these findings led to a diagnosis of acquired hemophilia A for which he was treated with methylprednisolone and recombinant activated factor VII on admission...
2016: Journal of Medical Case Reports
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"