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acquired hemophilia

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https://www.readbyqxmd.com/read/29780157/targeting-acquired-hemophilia-a-with-rheumatoid-arthritis-by-a-rituximab-shot-a-case-report-and-review-of-the-literature
#1
Imad Ghozlani, Aziza Mounach, Mirieme Ghazi, Anass Kherrab, Radouane Niamane
BACKGROUND Acquired hemophilia A (AH) is a rare hemorrhagic diathesis, characterized by the presence of autoantibodies directed against the pro-coagulant activity of factor VIII. It is associated with rheumatoid arthritis (RA) in 4% to 8% of cases and its prognosis remains severe. CASE REPORT A 66-year-old patient has been followed up for 20 years for deforming and severe RA, which was in low-disease activity. However, the patient presented a polyarticular flare involving the metacarpophalangeal and the proximal interphalangeal joints, the left elbow, and the right knee, which was warm and swollen...
May 21, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29774375/-successful-treatment-of-acquired-hemophilia-a-with-recombinant-porcine-factor-viii
#2
K Trautmann-Grill, O Tiebel, K Hölig, U Platzbecker
Acquired hemophilia A is a rare, potentially life-threatening disease resulting from autoantibodies against coagulation factor VIII. We report the case of a patient with acquired hemophilia A and severe bleeding after incision of a peritonsillar abscess. Treatment with high dose factor VIII and recombinant activated factor VII failed to control bleeding. However, a single infusion of recombinant porcine factor VIII stopped bleeding efficiently and resulted in measurable factor VIII levels.
May 17, 2018: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/29772615/-a-case-of-renal-pelvic-cancer-with-acquired-hemophilia-and-leukemoid-reaction
#3
Daiki Ikarashi, So Omori, Wataru Izumida, Yasuyuki Nakamura, Masakazu Abe, Takashi Ujiie, Wataru Obara
A 71-year-old man was referred to us with a right renal mass that was discovered by computed tomography (CT) examination for acquired hemophilia and leukemoid reaction. He presented with persistent low-grade fever and purpura on the lower legs caused by acquired hemophilia. Contrastenhanced CT scan showed a right renal tumor 6.0×7.4 cm in diameterwith inhomogeneous enhancement. The result of his urine cytology was negative. After improvement of his coagulation by treatment with immunosuppressants and steroids, he underwent open nephrectomy...
April 2018: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/29739073/a-case-of-acquired-hemophilia-a-usefulness-of-various-methods-for-judging-mixing-test-results-for-monitoring-the-effect-of-immunosuppressive-therapy
#4
Ryosuke Moriai, Nozomi Yanagihara, Akemi Endoh, Satoru Yamada, Maki Mochizuki, Takashi Kondo, Teruo Endoh, Koichi Asanuma, Satoshi Takahashi
BACKGROUND: Measurement of FVIII inhibitor (FVIII INH) levels is important for determining the effect of immunosuppressive therapy on acquired hemophilia A (AHA). However, FVIII INH can only be measured at a limited number of laboratories, which means that there are delays in obtaining the results at many sites. METHODS: A series of mixing tests were carried out in a case of AHA, followed by comparison of various methods for judging the obtained results in association with a change of FVIII INH...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29685311/treatment-of-acquired-hemophilia-in-the-elderly-study-of-10-cases
#5
José Antonio Rodríguez-García, Violeta Martínez-Robles, Belén Pérez-Gutiérrez
No abstract text is available yet for this article.
April 20, 2018: Medicina Clínica
https://www.readbyqxmd.com/read/29649469/phosphatidylserine-is-not-just-a-cleanup-crew-but-also-a-well-meaning-teacher
#6
Fiona Y Glassman, Jennifer L Schneider, Radha Ramakrishnan, Robert K Dingman, Murali Ramanathan, Richard B Bankert, Sathy V Balu-Iyer
Phosphatidylserine (PS) exposure during apoptosis leads to silent clearance of cells without adverse immune reactions to self-proteins. Given the biological functions of PS in cellular cleanup and global immunosuppression, we hypothesized that administration of PS-protein complexes would reduce immunogenicity. Here, we report that exposing Pompe disease mice to acid alpha glucosidase (rhGAA) with PS or immunosuppressant dexamethasone (Dex) resulted in lower anti-rhGAA-antibodies than in animals receiving rhGAA alone...
April 9, 2018: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29608457/can-the-plasmaderived-factor-viii-still-play-a-role-in-the-treatment-of-acquired-hemophilia-a-at-the-time-of-new-drugs
#7
Samantha Pasca, Vincenzo De Angelis, Marta Milan, Ezio Zanon
: Bypassing agents are the first-line therapy in the treatment of acquired hemophilia A (AHA), but not the only one. Other options as recombinant porcine factor VIII or plasmaderived concentrates (pdFVIII) are available to clinicians. Aim of this study was to evaluate whether the pdFVIII can still play a role in the treatment of AHA, and which patients could benefit from this therapy. All patients with AHA, presenting severe cardiovascular comorbidities, and treated with pdFVIII with or without von Willebrand factor (vWF), referred to two different hospitals, were initially considered...
March 31, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29582334/acquired-hemophilia-a-associated-with-autoimmune-pancreatitis-with-serum-igg4-elevation
#8
Taisuke Narazaki, Shojiro Haji, Yasuhiro Nakashima, Yasuhiro Tsukamoto, Mariko Tsuda, Akiko Takamatsu, Hirofumi Ohno, Takamitsu Matsushima, Tomoko Matsumoto, Keiji Nogami, Midori Shima, Motoaki Shiratsuchi, Yoshihiro Ogawa
A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL...
March 26, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29526954/the-development-of-acute-systemic-multiple-thrombosis-after-achieving-remission-during-systemic-glucocorticoid-therapy-for-acquired-hemophilia-a
#9
Kazuhiro Toyama, Atsushi Yasumoto, Fumihiko Nakamura, Shunya Arai, Mineo Kurokawa
Acquired hemophilia A (AHA) is a hemorrhagic disorder. Whether or not severe thrombotic events can develop without the use of bypassing agents in AHA patients is unclear. An 80-year-old woman with AHA underwent immunosuppressive therapy with prednisolone at 1 mg/kg daily. After achieving remission, she suddenly developed multiple organ failure due to acute systemic thrombosis and died within a few hours of the diagnosis. Patients with AHA, especially those with risk factors for thrombosis, have a considerable risk of developing thrombosis during the recovery phase of factor VIII activity and should be carefully monitored by coagulation testing...
March 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29381944/hemorrhagic-pericardial-effusion-as-the-debut-of-acquired-hemophilia-in-a-chronic-lymphocytic-leukemia-patient-a-case-report-and-a-review-of-acquired-hemophilia-a-related-hematological-malignancies
#10
José María Bastida, María Teresa Cano-Mozo, Felix Lopez-Cadenas, Victor Eduardo Vallejo, Soraya Merchán, Cecilia Santos-Montón, David González-Calle, Javier Carrillo, Ana Africa Martín, Jose Angel Torres-Hernández, Marcos González, Francisco Martín-Herrero, Pedro Pabón, Jose Ramon González-Porras
BACKGROUND: Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII. Spontaneous bleeding symptoms usually affect the skin and muscle, while pericardial effusion is an extremely rare manifestation. In the elderly, anticoagulant treatment is frequent and bleeding symptoms are usually associated with this. CLINICAL FINDINGS: We report a hemorrhagic pericardial effusion as the AHA debut in a patient with untreated chronic lymphocytic leukemia and anticoagulated with apixaban for atrial fibrillation and chronic arterial ischemia...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29326244/gene-therapy-comes-of-age
#11
REVIEW
Cynthia E Dunbar, Katherine A High, J Keith Joung, Donald B Kohn, Keiya Ozawa, Michel Sadelain
After almost 30 years of promise tempered by setbacks, gene therapies are rapidly becoming a critical component of the therapeutic armamentarium for a variety of inherited and acquired human diseases. Gene therapies for inherited immune disorders, hemophilia, eye and neurodegenerative disorders, and lymphoid cancers recently progressed to approved drug status in the United States and Europe, or are anticipated to receive approval in the near future. In this Review, we discuss milestones in the development of gene therapies, focusing on direct in vivo administration of viral vectors and adoptive transfer of genetically engineered T cells or hematopoietic stem cells...
January 12, 2018: Science
https://www.readbyqxmd.com/read/29296916/thromboembolic-event-rate-in-patients-exposed-to-anti-inhibitor-coagulant-complex-a-meta-analysis-of-40-year-published-data
#12
Matteo Rota, Paolo A Cortesi, Roberto Crea, Alessandro Gringeri, Lorenzo G Mantovani
Anti-inhibitor coagulant complex (AICC), an activated prothrombin complex concentrate, has been available for the treatment of patients with inhibitors since 1977, and thromboembolic events (TEEs) have been reported after infusion of AICC in patients with congenital or acquired hemophilia. With the aim of estimating the TEE incidence rate (IR) related to AICC exposure in these patients, a systematic review of the literature was carried out in Medline, according to PRISMA guidelines, from inception date to March 2017...
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29285161/hemorrhagic-pleural-effusion-related-to-acquired-coagulation-factor-viii-deficiency-a-case-report
#13
Yu-Ping Liu, Xiang-Hua Lin, Xue-Ke Wang, Shang-Zhi Yang, Chang-Qing Fan
A patient with acquired hemophilia A (AHA) with hemorrhagic pericardial effusions was admitted to Xiamen Chang Gung Hospital (Xiamen, China) in August 2015. The patient had been experiencing progressive dyspnea for 1 week. Bloody effusion (~6.3 l) was drained from the membrane surrounding the heart over a period of 20 days. Biochemical, cytological and radiological examinations were unable to elucidate the reason for the effusion. Coincidentally, it was discovered that activated partial thromboplastin time prolongation could not be corrected by plasma mixing...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29144625/acquired-hemophilia-presenting-as-gross-hematuria-following-kidney-stone-a-case-report-and-review-of-the-literature
#14
Max Schmidt-Bowman, Lael Reinstatler, Eric P Raffin, Joseph E Yared, John D Seigne, Einar F Sverrisson
A rare condition in itself, acquired hemophilia A, seldom presents as isolated gross hematuria. It is a serious condition with a high mortality rate and thus clinical suspicion followed by prompt diagnosis is imperative (1). In fact, only 8 cases of such presentation of this condition have been reported thus far in the literature. Of these, none describe the initial presentation of hematuria with the inciting event of a kidney stone. We present a case of a 67-year-old man with signs and symptoms of nephrolithiasis accompanied by profuse hematuria, who was subsequently found to have developed expression of factor VIII inhibitor leading to acquired hemophilia A...
March 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29126301/coagulation-testing-in-the-core-laboratory
#15
REVIEW
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29112188/diagnosis-and-treatment-of-acquired-hemophilia-a-single-center-experience
#16
Bożena Sokołowska, Justyna Kozińska, Magdalena Kozioł, Ewa Wąsik-Szczepanek, Dariusz Szczepanek, Marek Hus
No abstract text is available yet for this article.
November 30, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29099367/dental-management-of-patients-with-inherited-bleeding-disorders-a-multidisciplinary-approach
#17
Hassan Abed, Abdalrahman Ainousa
Bleeding disorders can be inherited or acquired and demonstrate different levels of severity. Dentists may be called on to treat patients who have bleeding disorders such as hemophilia A and von Willebrand disease (vWD). Dental extraction in any patient with clotting factor defects can result in a delayed bleeding episode. Local hemostatic measures provide effective results in a majority of cases but are insufficient in patients with severe hemophilia A and vWD. Therefore, consultation with the patient's hematologist is required to ensure preoperative prophylactic coverage...
November 2017: General Dentistry
https://www.readbyqxmd.com/read/29095762/acquired-hemophilia-with-thrombosis-in-a-cancer-patient-an-unusual-presentation
#18
Senem Maral, Sule Mine Bakanay, Imdat Dilek
: Acquired hemophilia A (AHA) which presents with spontaneous severe intramuscular, mucosal and/or subcutaneous bleeding is a rare bleeding disorder. Even 50% of AHA patients are defined as idiopathic; 10% of cases are related with malignancy. Here, we present a case of AHA in a 43-year-old lady who was diagnosed with malignancy and venous thromboembolism on vena cava 2 years ago. To the best of our knowledge, this is the first report in literature presented with both acquired hemophilia and thrombosis associated with malignancy...
January 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29078851/acquired-hemophilia-and-anti-hu-paraneoplastic-neurologic-syndrome-in-small-cell-lung-cancer
#19
Efi Aggelopoulou, Gerasimos Evangelatos, Konstantina Tzavida, Vasiliki Koulouri, Nikolaos Lazaridis
No abstract text is available yet for this article.
October 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29027258/acquired-factor-xi-deficiency-and-therapeutic-plasma-exchange
#20
Geoffrey D Wool, Angela Treml, Jonathan L Miller
Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case of acquired FXI deficiency in a pregnant woman with lupus. We proved that the inhibitor was an IgG, therefore potentially capable of crossing the placenta. While immune suppression eliminated detectable circulating inhibitor, the woman's FXI remained quite low...
October 13, 2017: Journal of Clinical Apheresis
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