Contributions
Type: Publication Only
Background
Haemophilia patients with inhibitors can develop bleeding episodes refractory to monotherapy with either recombinant factor VIIa (rFVIIa) or activated prothrombin complex concentrates (APCC). These bleeds are often very difficult to treat and can result in significant morbidity. For such patients, therapies with sequential administration of standard doses or concomitant administration of lower dose of rFVIIa and APCC have been suggested. However, treatment with a combination of these agents is not widely practiced.
Aims
To present our experience of sequential therapy with rFVIIa and APCC in patients with severe haemophilia A, inhibitors and life threatening bleeding.
Methods
We reviewed the medical records of all the inhibitor patients for whom sequential therapy was prescribed, defined as having received both APCC and rFVIIa within 6 h of each other.
Results
Case 1) A 19 year’s old patient with sever haemophilia A and previously detected inhibitor (11 BU) developed intracranial hemorrhage. He was treated with rFVIIa in dose 90 μg/kg/2 h. Due to very bad venous access central venous catheter was inserted with rVIIa (120 μg/kg every 2 hours, 2 dose). Four hour after surgery massive neck oedema and breathless developed. CT scan showed diffuse large neck muscle and soft tissue hematoma. Therapy with APCC 100 U/kg/12h and rFVIIa 90 μg/kg every 2 to 3 hours resulted in bleeding control. After 12 h of sequential usage, therapy was continued with rFVIIa.
Case 2) A 35 year’s old patient with sever haemophilia A and previously detected inhibitor (220 BU) developed spontaneous mediastinal and pericardial bleeding, without sings of tamponade. He was treated with APCC 100 U/kg/12h. Two days after admission haemoglobin level decreased from 134 to 78 g/L. CT showed massive mediastinal bleeding complicated with pleural effusion. Therapy was continued with rFVIIa 90 μg/kg every 2 hours, APCC 100 U/kg/12 h and blood transfusion. After 24 h of sequential usage therapy was continued with APCC.
Case 3) A 19 year’s old patient with sever haemophilia A and previously detected inhibitor (20 BU) was prepared for tooth extraction with rFVIIa (90 μg/kg). Extraction was complicated with massive subconjuctival, infraorbital and buccal hematoma with difficult swallowing. Dose of rFVIIa was increased to 120 μg/kg every 2 hours, without effect. Sequential therapy with rFVIIa 90 μg/kg/2 hours and APCC 100 U/kg/12 h was continued and bleeding was stopped. After 24 h of sequential therapy treatment was continued with APCC.
Symptoms and sings of thrombosis and disseminate intravascular coagulopathy, as well as d dimer elevation were not seen in our patient’s during sequential therapy.
Summary
Our report confirms efficacy and the safety of short course sequential rFVIIa and APCC for the management of life threatening bleeding refractory to monotherapy with APCC or rFVIIa. However, controlled trials with a larger number of patients are required to further assess the safety, efficacy and cost benefit of this treatment.
Keyword(s): Hemophilia A, Inhibitor
Session topic: Publication Only
Type: Publication Only
Background
Haemophilia patients with inhibitors can develop bleeding episodes refractory to monotherapy with either recombinant factor VIIa (rFVIIa) or activated prothrombin complex concentrates (APCC). These bleeds are often very difficult to treat and can result in significant morbidity. For such patients, therapies with sequential administration of standard doses or concomitant administration of lower dose of rFVIIa and APCC have been suggested. However, treatment with a combination of these agents is not widely practiced.
Aims
To present our experience of sequential therapy with rFVIIa and APCC in patients with severe haemophilia A, inhibitors and life threatening bleeding.
Methods
We reviewed the medical records of all the inhibitor patients for whom sequential therapy was prescribed, defined as having received both APCC and rFVIIa within 6 h of each other.
Results
Case 1) A 19 year’s old patient with sever haemophilia A and previously detected inhibitor (11 BU) developed intracranial hemorrhage. He was treated with rFVIIa in dose 90 μg/kg/2 h. Due to very bad venous access central venous catheter was inserted with rVIIa (120 μg/kg every 2 hours, 2 dose). Four hour after surgery massive neck oedema and breathless developed. CT scan showed diffuse large neck muscle and soft tissue hematoma. Therapy with APCC 100 U/kg/12h and rFVIIa 90 μg/kg every 2 to 3 hours resulted in bleeding control. After 12 h of sequential usage, therapy was continued with rFVIIa.
Case 2) A 35 year’s old patient with sever haemophilia A and previously detected inhibitor (220 BU) developed spontaneous mediastinal and pericardial bleeding, without sings of tamponade. He was treated with APCC 100 U/kg/12h. Two days after admission haemoglobin level decreased from 134 to 78 g/L. CT showed massive mediastinal bleeding complicated with pleural effusion. Therapy was continued with rFVIIa 90 μg/kg every 2 hours, APCC 100 U/kg/12 h and blood transfusion. After 24 h of sequential usage therapy was continued with APCC.
Case 3) A 19 year’s old patient with sever haemophilia A and previously detected inhibitor (20 BU) was prepared for tooth extraction with rFVIIa (90 μg/kg). Extraction was complicated with massive subconjuctival, infraorbital and buccal hematoma with difficult swallowing. Dose of rFVIIa was increased to 120 μg/kg every 2 hours, without effect. Sequential therapy with rFVIIa 90 μg/kg/2 hours and APCC 100 U/kg/12 h was continued and bleeding was stopped. After 24 h of sequential therapy treatment was continued with APCC.
Symptoms and sings of thrombosis and disseminate intravascular coagulopathy, as well as d dimer elevation were not seen in our patient’s during sequential therapy.
Summary
Our report confirms efficacy and the safety of short course sequential rFVIIa and APCC for the management of life threatening bleeding refractory to monotherapy with APCC or rFVIIa. However, controlled trials with a larger number of patients are required to further assess the safety, efficacy and cost benefit of this treatment.
Keyword(s): Hemophilia A, Inhibitor
Session topic: Publication Only