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rituximab and children hemophilia A

W Liu, F Xue, L Zhang, X F Liu, Y F Chen, Y T Huang, R F Fu, R C Yang
OBJECTIVE: To study the risk factors, characteristics and treatment of factor Ⅷ (F Ⅷ) inhibitor in Chinese children with hemophilia A (HA) through a retrospective case-control survey; To investigate the bleeding phenotype before and after inhibitor formation. METHODS: The clinical data of outpatients and inpatients with HA children from January 2010 to December 2014 were collected. A case control study was performed to investigate the risk factors for inhibitor formation...
June 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Chunchen Yang, Ziqiang Yu, Wei Zhang, Lijuan Cao, Jian Su, Pingping Sha, Xia Bai, Zhaoyue Wang, Changgeng Ruan
OBJECTIVE: To investigate the feature of underlying disorders, clinical symptoms, diagnosis and treatment strategies of patients with acquired hemophilia(AH). METHODS: The clinical data and laboratory tests results of 22 patients with AH from March 2010 to June 2014 were retrospectively analyzed. RESULTS: A total of 22 patients with AH were enrolled in our study, including 20 patients diagnosed as acquired hemophilia A (AHA) and 2 as acquired hemophilia B (AHB)...
February 2015: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Anupama Borker, Narendra Choudhary
Rituximab is a chimeric mouse-human monoclonal antibody against the CD 20 antigen on the surface of B lymphocytes. It binds to CD20 and causes B cell death by antibody dependant cell-mediated cytotoxicity, complement mediated cytotoxicity and apoptosis. It leads to rapid and sustained depletion of B cells. It is licensed for use in adults with CD20 positive B-cell lymphoma and rheumatoid arthritis. In children, it has been used in a variety of off-label indications with promising results. It has proved useful as salvage therapy in relapsed refractory non-Hodgkins lymphoma and leukemia, and in hematological conditions including chronic immune thrombocytopenic purpura, hemophilia with inhibitors, and autoimmune hemolytic anemia...
August 2011: Indian Pediatrics
Werner Streif, C Escuriola Ettingshausen, R Linde, G Kropshofer, L-B Zimmerhackl, W Kreuz
UNLABELLED: The development of neutralizing alloantibodies (inhibitors) to factor VIII (FVIII) is one of the most serious complications in the treatment of haemophiliacs. Inhibitors occur in approximately 20 to 30% of previously untreated patients (PUPs), predominantly children, with severe haemophilia A within the first 50 exposure days (ED). Immune tolerance induction (ITI) leads to complete elimination of the inhibitor in up to 80% of the patients and offers the possibility to restore regular FVIII prophylaxis...
May 2009: Hämostaseologie
Jumana H Albaramki, Juliana Teo, Stephen I Alexander
Thrombotic thrombocytopenic purpura (TTP) is a rare disease among pediatric patients, in whom it may be mistaken for hemolytic uremic syndrome (HUS) and idiopathic thrombocytopenic purpura (ITP). Familial forms are caused by mutations in the ADAMTS13 gene, whereas acquired forms may result from an inhibitory antibody directed against ADAMTS13, a metalloprotease that cleaves very large multimers of Von Willebrand factor (VWF), thereby preventing platelet aggregation in blood vessels. We report two cases of TTP...
September 2009: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
M Franchini, C Mengoli, G Lippi, G Targher, M Montagnana, G L Salvagno, M Zaffanello, M Cruciani
Rituximab, a monoclonal antibody against the pan B-cell antigen CD20, has been successfully used in both adults and children for the management of malignant and non-malignant immune-mediated disorders including acquired haemophilia. On the basis of this positive experience, a number of investigators have recently used this agent in patients with congenital haemophilia and inhibitors refractory to first-line treatments. After a careful electronic and hand search, we have collected 29 studies that included 49 cases...
September 2008: Haemophilia: the Official Journal of the World Federation of Hemophilia
Lisa B Giulino, James B Bussel, Ellis J Neufeld
No abstract text is available yet for this article.
April 2007: Journal of Pediatrics
M Moschovi, S Aronis, G Trimis, H Platokouki, K Salavoura, F Tzortzatou-Stathopoulou
The development of antibodies to factor VIII (FVIII) in severely affected haemophilia A patients is a serious complication associated with increased morbidity and mortality. Bypassing agents are used to treat acute bleeding episodes; however, elimination of the inhibitors can only be achieved with immune tolerance therapy (ITT) in 60-80% of cases. High responding (HR) inhibitors are more likely to respond to ITT if the titre is decreased to <5 BU over time or in selected cases after the administration of immunosuppressive drugs, plasmapheresis or immunoabsorption, techniques difficult to apply in children...
January 2006: Haemophilia: the Official Journal of the World Federation of Hemophilia
M Carcao, J St Louis, M-C Poon, E Grunebaum, S Lacroix, A M Stain, V S Blanchette, G E Rivard
When a high titre inhibitor develops in a patient with haemophilia, attempts are made to eradicate it through immune tolerance induction therapy (ITI) involving the frequent and regular administration of factor, usually for months to years. ITI is successful in only two thirds of patients prompting investigators to explore alternate regimens to use in haemophiliacs failing conventional ITI. Rituximab is an anti-CD20 monoclonal antibody, which has shown promise in the treatment of B-cell-mediated disorders. We developed a protocol for the use of rituximab in haemophilia A (HA) patients failing conventional ITI or in those haemophiliacs where the likelihood of success of conventional ITI is poor...
January 2006: Haemophilia: the Official Journal of the World Federation of Hemophilia
Mary Mathias, Kate Khair, Ian Hann, Ri Liesner
We report the use of rituximab (Genentech, San Francisco, CA, USA) in two children with severe haemophilia with inhibitors to factors VIII and IX, which failed to respond to conventional immune tolerance therapy. The treatment was well tolerated by both children. The child with haemophilia B had no clinical improvement or fall in CD19 and he is currently being treated with recombinant activated factor VII (NovoSeven, Novo Nordisk, Denmark) for bleeding episodes. The child with haemophilia A had a good clinical response with a negative inhibitor assay at 11 months follow-up...
May 2004: British Journal of Haematology
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