Contributions
Abstract: PB1818
Type: Publication Only
Background
Rivaroxaban is an anticoagulant and the first orally active direct factor Xa inhibitor. It is indicated for the prevention of venous thromboembolic events, especially stroke and systemic embolism in patients with nonvalvular atrial fibrillation. Routine lab monitoring is not necessary. At advanced age, hemorrhage may become a serious side effect of anticoagulants and it must be investigated to exclude other causes of acquired coagulopathies. One of these is Acquired Hemophilia A (AHA), a rare bleeding disorder caused by an autoantibody to coagulation factor VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute with up to 22% mortality to mild bleeding that requires no treatments. Initial treatment involves the control of acute bleeding with bypassing agents. Immunosuppressive treatment to eradicate the FVIII inhibitor should be started as soon as the diagnosis is confirmed to reduce the time the patient is at risk of bleeding.
Aims
Report a rare case of a patient taking rivaroxaban with bleeding associated with acquired hemophilia A.
Methods
We report a case of a 72-year-old man suffering from atrial fibrillation/coronary, artery disease/ hypertension for several years and taking Rivaroxaban 20mg/24h since 2014. He presented at an emergency with genital urinary and muscle bleedings specially in hands and abdomen.
Results
Hemostatic tests indicated prolonged activated partial thromboplastin time (APTT) to 107 sec (norm 26-36 sec), normal value of the prothrombin index which was 85% (norm 70-130%), fibrinogen concentration to 380 (normal value 200-400 mg/dl), the bleeding time was 5 min (norm < 10 min) and the platelet count was 325 x 109 /l (norm 130-400 x 109 /l). Therapy with Rivaroxaban was discontinued. 48 hours later the patient presented the same alterations in exams. A coagulation disorder was therefore suspected. The autoantibody against factor VIII in a titer 205 Bethesda Units/ml (BU/ml) and decreased factor VIII activity to 0,1% (norm 50 -150%) with normal plasma concentration of factor IX. Activated (FEIBA, Baxter) had been used in the treatment of bleeding episodes for five times. immunosuppressive treatment with oral prednisone 80mg/24h was administered for three weeks without improvements. Then, cyclophosphamide 100mg/24h was added in order to remove the factor VIII inhibitor. One month later, APTT decreased to 57 sec but little bleedings continued. We decided to use Rituximab 375 mg/m2 weekly for four doses. After this, reduction of the factor VIII inhibitor titer to 2,6% and increased of factor VIII activity to 128% led to normalization of hemostatic parameters. We investigated solid tumors and lymphoproliferative diseases by using computerized tomography, PET scan and tumor markers which were negative. Autoantibodies to rheumatologic diseases were negative.
Conclusion
Although the new anticoagulants are safe, hemorrhages may occur and clinicians should conduct these alterations carefully. Delay or undertreatment may be life-threatening. If atypical hemorrhage occurs during anticoagulant therapy, e.g. severe nontraumatic skin hemorrhage, an additional coagulation disorder should be considered. In order to confirm diagnosis, clotting testing can be performed again 48 hours after anticoagulant treatment is interrupted. If APTT, for example, remains abnormally prolonged, clotting disorders such as deficiency of clotting factors VIII, IX, or XI should be ruled out.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired hemophilia, Anticoagulants, Bleeding disorder
Abstract: PB1818
Type: Publication Only
Background
Rivaroxaban is an anticoagulant and the first orally active direct factor Xa inhibitor. It is indicated for the prevention of venous thromboembolic events, especially stroke and systemic embolism in patients with nonvalvular atrial fibrillation. Routine lab monitoring is not necessary. At advanced age, hemorrhage may become a serious side effect of anticoagulants and it must be investigated to exclude other causes of acquired coagulopathies. One of these is Acquired Hemophilia A (AHA), a rare bleeding disorder caused by an autoantibody to coagulation factor VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute with up to 22% mortality to mild bleeding that requires no treatments. Initial treatment involves the control of acute bleeding with bypassing agents. Immunosuppressive treatment to eradicate the FVIII inhibitor should be started as soon as the diagnosis is confirmed to reduce the time the patient is at risk of bleeding.
Aims
Report a rare case of a patient taking rivaroxaban with bleeding associated with acquired hemophilia A.
Methods
We report a case of a 72-year-old man suffering from atrial fibrillation/coronary, artery disease/ hypertension for several years and taking Rivaroxaban 20mg/24h since 2014. He presented at an emergency with genital urinary and muscle bleedings specially in hands and abdomen.
Results
Hemostatic tests indicated prolonged activated partial thromboplastin time (APTT) to 107 sec (norm 26-36 sec), normal value of the prothrombin index which was 85% (norm 70-130%), fibrinogen concentration to 380 (normal value 200-400 mg/dl), the bleeding time was 5 min (norm < 10 min) and the platelet count was 325 x 109 /l (norm 130-400 x 109 /l). Therapy with Rivaroxaban was discontinued. 48 hours later the patient presented the same alterations in exams. A coagulation disorder was therefore suspected. The autoantibody against factor VIII in a titer 205 Bethesda Units/ml (BU/ml) and decreased factor VIII activity to 0,1% (norm 50 -150%) with normal plasma concentration of factor IX. Activated (FEIBA, Baxter) had been used in the treatment of bleeding episodes for five times. immunosuppressive treatment with oral prednisone 80mg/24h was administered for three weeks without improvements. Then, cyclophosphamide 100mg/24h was added in order to remove the factor VIII inhibitor. One month later, APTT decreased to 57 sec but little bleedings continued. We decided to use Rituximab 375 mg/m2 weekly for four doses. After this, reduction of the factor VIII inhibitor titer to 2,6% and increased of factor VIII activity to 128% led to normalization of hemostatic parameters. We investigated solid tumors and lymphoproliferative diseases by using computerized tomography, PET scan and tumor markers which were negative. Autoantibodies to rheumatologic diseases were negative.
Conclusion
Although the new anticoagulants are safe, hemorrhages may occur and clinicians should conduct these alterations carefully. Delay or undertreatment may be life-threatening. If atypical hemorrhage occurs during anticoagulant therapy, e.g. severe nontraumatic skin hemorrhage, an additional coagulation disorder should be considered. In order to confirm diagnosis, clotting testing can be performed again 48 hours after anticoagulant treatment is interrupted. If APTT, for example, remains abnormally prolonged, clotting disorders such as deficiency of clotting factors VIII, IX, or XI should be ruled out.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired hemophilia, Anticoagulants, Bleeding disorder