Tranexamic acid as adjunct therapy to bypassing agents in haemophilia A patients with inhibitors

Haemophilia ◽  
2013 ◽  
Vol 20 (3) ◽  
pp. 369-375 ◽  
Author(s):  
H. T. T. Tran ◽  
B. Sørensen ◽  
C. J. Rea ◽  
S. Bjørnsen ◽  
T. Ueland ◽  
...  
Keyword(s):  
Tranexamic Acid ◽  
Haemophilia A ◽  
Adjunct Therapy ◽  
Bypassing Agents

Efficiency of Tranexamic Acid in Management of Surgical Orthopedic Bleeding in Patients with Haemophilia

2017 ◽  
Vol 68 (3) ◽  
pp. 627-630 ◽  
Author(s):  
Oana Viola Badulescu ◽  
Razvan Tudor ◽  
Wilhelm Friedl ◽  
Mihaela Blaj ◽  
Paul Dan Sirbu
Keyword(s):  
Tranexamic Acid ◽  
Orthopaedic Surgery ◽  
Enzymatic Degradation ◽  
Point Of View ◽  
Factor Ix ◽  
Haemophilia A ◽  
Chromosome X ◽  
Risk Of Bleeding ◽  
Subchondral Bone Tissue ◽  
Knee Endoprosthesis

Haemophilia is an inherited bleeding disorder (gonosomala recessive, related to chromosome X, with transmission from carrying women to male descendents) characterised from the clinic point of view by important bleeding, secondary to some minimum and biologic traumas by deficiency of trombo-plastino-formation, consecutive to either a deficit of factor VIII (haemophilia A), or the factor IX (haemophilia B). The most characteristic manifestation of hemophilia is intra-articular � hemarthrosis. Its repetitive character leads to irreversible lesions of the articular structures, inducing lesions of the synovium with degenerative effects over the articular cartilage and destructive effects for the subchondral bone tissue. In time, these lesions require orthopaedic surgery to improve the locomotor activity. Managing an efficient hemostasis is vital during surgery, due to high risk of bleeding triggered by coagulopathy and surgery. Numerous studies carried out underlined the efficiency of the tranexamic acid (TXA) in reducing bleeding, in different surgery branches, by inhibiting the enzymatic degradation of fibrin. In orthopaedic surgery, the tranexamic acid is frequently used in case of hip and knee arthroplasties, reducing the bleeding and blood transfusion necessary to the treatment of posthaemorrhagic anemia. This paper wants to assess the efficiency of the tranexamic acid in realization of hemostasis to another category of patients, haemophiliac patients with indication of total hip and knee endoprosthesis.

An in-vitro assessment of tranexamic acid as an adjunct to rFVIII or rFVIIa treatment in haemophilia A

2013 ◽  
Vol 93 (4) ◽  
pp. 683-692 ◽  
Author(s):  
Catherine J. Rea ◽  
Jonathan H. Foley ◽  
David H. Bevan ◽  
Benny Sørensen
Keyword(s):  
Tranexamic Acid ◽  
Haemophilia A ◽  
In Vitro Assessment

scholarly journals Prophylaxis therapy with bypassing agents in patients with haemophilia A and inhibitors undergoing surgery: A cost analysis in Spain

2020 ◽  
Vol 105 (1) ◽  
pp. 94-100
Author(s):  
María Mareque ◽  
María Eva Mingot‐Castellano ◽  
María Fernanda López‐Fernández ◽  
María Teresa Álvarez‐Román ◽  
Itziar Oyagüez
Keyword(s):  
Cost Analysis ◽  
Haemophilia A ◽  
Prophylaxis Therapy ◽  
Bypassing Agents

Sequential Infusion of rFVIIa and Plasma-Derived Activated Prothrombin Complex Concentrate In Haemophilia A Paediatric Patient with Inhibitors

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4660-4660
Author(s):  
Giuseppe Lassandro ◽  
Francesco Antonio Scaraggi ◽  
Rosanna Scaraggi ◽  
Teresa Capriati ◽  
Domenico De Mattia ◽  
...  
Keyword(s):  
Prothrombin Complex Concentrate ◽  
Conflicts Of Interest ◽  
Coagulation Factors ◽  
High Titre ◽  
Left Knee ◽  
Haemophilia A ◽  
Activated Prothrombin Complex Concentrate ◽  
End Of Treatment ◽  
Inhibitor Titre ◽  
Bypassing Agents

Abstract Abstract 4660 One of the serious complication in hemophilia therapy is the development of high titre inhibitors to FVIII and less often to others coagulation factors. It makes treatment of bleeds very challenging. We report a case of hemarthrosis in hemophilia A pediatric patient with inhibitors, treated with sequential infuson of rFVIIa (rFVIIa, NOVOSEVEN; Novo Nordisk A/S, Bagsvaerd, Denmark) and plasma activated prothrombin complex concentrate (pd- aPCC, FEIBA; Baxter AG Vienna Austria). rFVIIa and plasma activated prothrombin complex concentrate are, indeed, used as haemostatic bypassing agents to prevent eaemorrages, with the goal of limiting sequelae as arthropathy, or to control quickly heamostasis as intensive on–demand treatment. A 3 years old male patient affected by haemophilia A with inhibitors came to our observation for a traumatic hemarthrosis of the left knee. Clinic examination showed swelling and pain. His inhibitor titre was 29 Bethesda Units. First we infused rFVIIa for seven consecutive days at the dose of 90 ug/kg every 3 hours. This therapy didn't determinate any clinical improvement. Then we infused plasma activated prothrombin complex concentrate for the next consecutive seven days at the dose of 60 UI/kg every 12 hours. At the end of treatment we noticed pain disappearance and reducing swelling. Medical literature recently describes similar paediatric cases treated with sequential infusion of rFVIIa and plasma activated prothrombin complex concentrate. Our positive experience could stimulate to use haemostatic bypassing agents because apparently safe. We encourage to use this therapeutic scheme because it seem to reduce healing times of acute events. Disclosures: No relevant conflicts of interest to declare.

Recombinant activated factor VII and tranexamic acid are haemostatically effective during major surgery in factor XI-deficient patients with inhibitor antibodies

2009 ◽  
Vol 102 (09) ◽  
pp. 487-492 ◽  
Author(s):  
Tami Livnat ◽  
Ilia Tamarin ◽  
Yoram Mor ◽  
Harry Winckler ◽  
Zeev Horowitz ◽  
...  
Keyword(s):  
Tranexamic Acid ◽  
Low Dose ◽  
Null Alleles ◽  
Factor Vii ◽  
Major Surgery ◽  
Haemophilia A ◽  
Factor Xi ◽  
Recombinant Activated Factor Vii ◽  
Excessive Bleeding ◽  
Activated Factor Vii

SummaryOne-third of patients with severe factor XI (FXI) deficiency caused by homozygosity for null alleles develop inhibitor antibodies following exposure to plasma. Haemostasis during surgery is achievable in such patients by recombinant activated factor VII (rFVIIa) at doses used in haemophilia A patients with an inhibitor to FVIII. However, thrombosis has occurred in three of 12 such patients. In this study we discerned whether low-dose rFVIIa would secure haemostasis and cause no thrombosis in patients with severe FXI deficiency and an inhibitor during surgery. In vitro, a very low concentration of rFVIIa (0.24 µg/ml) induced thrombin generation in FXI-deficient plasma quite similarly to 1.9 µg/ml (a concentration that is achieved in patients with haemophilia A and inhibitor after infusion of 80 µg/kg). Based on this finding, a protocol was designed for four patients with severe FXI deficiency and an inhibitor or immunoglobulin A deficiency who underwent five major surgical procedures. This included administration of tranexamic acid from two hours before surgery until seven to 14 days after, and single infusion of low-dose rFVIIa. No excessive bleeding or thrombosis were observed. In conclusion, a single low dose of rFVIIa and tranexamic acid secure normal haemostasis in patients with severe FXI deficiency who can not receive blood products.

scholarly journals Thrombin generation in plasma of patients with haemophilia A and B with inhibitors: Effects of bypassing agents and antithrombin reduction

2020 ◽  
Vol 82 ◽  
pp. 102416 ◽  
Author(s):  
Tami Livnat ◽  
Alfica Sehgal ◽  
Kun Qian ◽  
Huy Van Nguyen ◽  
Kate Madigan ◽  
...  
Keyword(s):  
Thrombin Generation ◽  
Haemophilia A ◽  
Bypassing Agents

scholarly journals Tranexamic‐acid‐induced acute renal cortical necrosis in a patient with haemophilia A

2001 ◽  
Vol 16 (1) ◽  
pp. 189-190 ◽  
Author(s):  
Ali Riza Odabaş ◽  
Ramazan Çetinkaya ◽  
Yilmaz Selçuk ◽  
Hasan Kaya ◽  
Ünsal Coşkun
Keyword(s):  
Tranexamic Acid ◽  
Haemophilia A ◽  
Cortical Necrosis ◽  
Renal Cortical Necrosis

scholarly journals Use of Emicizumab in Patients with Hemophilia a with and without Inhibitors: A Single Center Experience

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4248-4248
Author(s):  
Gaetano Giuffrida ◽  
Daniela Nicolosi ◽  
Annalisa Condorelli ◽  
Uros Markovic ◽  
Francesco Di Raimondo
Keyword(s):  
Quality Of Life ◽  
Replacement Therapy ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Factor X ◽  
Bleeding Events ◽  
Single Center Experience ◽  
Fda Approval ◽  
Bypassing Agents

Abstract Emicizumab is a humanized bispecific recombinant monoclonal antibody that binds the enzyme factor IXa and the substrate factor X and mimics the function of FVIII. It solved some unmet needs of haemophilia A (HA) treatment, such as regimen (once weekly up to once monthly infusion) and route of administration (subcutaneous). Most importantly, emicizumab reduce bleeding episodes and improved the quality of life of these patients. Emicizumab received FDA approval for prophylaxis in HA patients with inhibitors in November 2017 and for patients without inhibitors in October 2018.The aim of this study is to provide information on treatment with emicizumab in patients with severe haemophilia A with and without inhibitors in terms of efficacy, safety and quality of life. Here we report our experience with 9 patients with severe haemophilia A (5 with inhibitors and 4 without inhibitors) that were switched from replacement therapy to emicizumab prophylaxis. Median age at initiation of treatment was 26.8 years for patients with inhibitors and 18.7 years for those without inhibitors. Median duration of therapy was 12 months for patients with inhibitors and 8 months for those without inhibitors. All patients were started on emicizumab with a loading dose of 3 mg/kg once weekly for 4 weeks followed by a maintenance dose of 1.5 mg/kg once weekly or every two weeks. The mean annual bleeding rates (ABR) in patients without inhibitor prior to emicizumab was 1.5 compared to 0 while on emicizumab prophylaxis. In subjects with inhibitors ABR was 1.8 compared to 0.4 while on emicizumab prophylaxis. Furthermore, no adverse events including thrombotic events occurred in course of emicizumab prophylaxis. Bleeding events in course of emicizumab prophylaxis did not request any treatment with replacement therapy or bypassing agents. The questionnaire evaluating quality of life (HAL v 2.0 - 2015 ITA; pedHAL v 2.0 - 2015 ITA) was administered to the patients before switch to emicizumab and after a follow up of six months. The scores showed a significant improvement in terms of quality of life in course of emicizumab prophylaxis. In conclusion, our experience suggests that emicizumab prophylaxis is safe and improved protection against bleeding and quality of life compared to replacement therapy in patients with severe A haemophilia. Disclosures Di Raimondo: Amgen: Honoraria; Jazz Pharmaceutical: Honoraria; Pfizer: Honoraria; Janssen Pharmaceuticals: Honoraria; Bristol Myers Squibb: Honoraria; AbbVie: Honoraria.

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