Factor VIII inhibitor bypassing fraction/octocog-α/von-Willebrand factor
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Reactions 1280 - 28 Nov 2009 S Factor VIII inhibitor bypassing fraction/octocog-α/von-Willebrand factor Anaphylaxis in an elderly patient: case report A 70-year-old man, who had severe haemophilia A, developed anaphylaxis initially during treatment with von- Willebrand factor and later during treatment with octocog- α and then with factor VIII inhibitor bypassing fraction. The man, who had a history of haemophilic rheumatic disorders, chronic hepatitis C infection , hypertension and diabetes mellitus, had been treated with cryoprecipitate and plasma-derived factor VIII concentrates. Between January 1996 and April 2005, he received octocog-α for a total of 18 days. In April 2005, he a left knee arthroplasty was performed and he received octocog-α for 2 months, In November 2005, a high-titre inhibitor against factor VIII was diagnosed for the first time. A recovery test with octocog-α showed no response, but was followed by an anamnestic response of the inhibitor. Over the next 2 months, he developed two haemorrhagic episodes that were successfully treated with eptacog-α. In January 2007, he was admitted to an ICU due to an acute coronary episode. An angiography, performed with concomitant infusion of eptacog-α, revealed a severe three-vessel coronary artery disease. He received treatment without antiplatelets. In addition, a decision was made to start an immune tolerance protocol for performing the cardiac intervention. After a central venous catheter was inserted under haemostatic coverage with eptacog-α, he started immune tolerance in March 2007. However, the infusion of von-Willebrand factor [Haemate-P] was stopped after 10mL because he developed allergy with pruritus, headache, urticaria, hypotension and bronchospasm. The man received emergency treatment, after which his symptoms disappeared. Laboratory investigations showed the following: IgG level 2030 mg/dL, total IgE 220 mg/dL, C3 level 83%, total complement activity 20% and inhibitor titre 7.2 BU. The next month, immune tolerance was restarted with octocog-α [Helixate; dosage not stated], but the infusion was stopped after administration of a few millilitres due to a urticarial reaction. In December 2007, he developed a similar reaction after administration of factor VIII inhibitor bypassing fraction [FEIBA; dosage and time to reaction onset not stated]; FEIBA was administered after a scarce response to eptacog-α for post-traumatic haemarthrosis at the left knee. He received no factor VIII- containing concentrates after December 2007 and, in November 2008, he had a inhibitor titre of 6.2 BU, an IgG level of 1860 mg/dL, an IgE level of 48 mg/dL and a total complement activity of 90%. Rivolta GF, et al. Management of coronary artery disease in a severe haemophilia patient with high titre inhibitor and anaphylaxis. Haemophilia 15: 1161-1163, No. 5, 2009 - Italy 801154644 1 Reactions 28 Nov 2009 No. 1280 0114-9954/10/1280-0001/$14.95 © 2010 Adis Data Information BV. All rights reserved
Transcript of Factor VIII inhibitor bypassing fraction/octocog-α/von-Willebrand factor
Reactions 1280 - 28 Nov 2009
SFactor VIII inhibitor bypassingfraction/octocog-α/von-Willebrandfactor
Anaphylaxis in an elderly patient: case reportA 70-year-old man, who had severe haemophilia A,
developed anaphylaxis initially during treatment with von-Willebrand factor and later during treatment with octocog-α and then with factor VIII inhibitor bypassing fraction.
The man, who had a history of haemophilic rheumaticdisorders, chronic hepatitis C infection , hypertension anddiabetes mellitus, had been treated with cryoprecipitateand plasma-derived factor VIII concentrates. BetweenJanuary 1996 and April 2005, he received octocog-α for atotal of 18 days. In April 2005, he a left knee arthroplastywas performed and he received octocog-α for 2 months, InNovember 2005, a high-titre inhibitor against factor VIIIwas diagnosed for the first time. A recovery test withoctocog-α showed no response, but was followed by ananamnestic response of the inhibitor. Over the next2 months, he developed two haemorrhagic episodes thatwere successfully treated with eptacog-α. In January 2007,he was admitted to an ICU due to an acute coronaryepisode. An angiography, performed with concomitantinfusion of eptacog-α, revealed a severe three-vesselcoronary artery disease. He received treatment withoutantiplatelets. In addition, a decision was made to start animmune tolerance protocol for performing the cardiacintervention. After a central venous catheter was insertedunder haemostatic coverage with eptacog-α, he startedimmune tolerance in March 2007. However, the infusion ofvon-Willebrand factor [Haemate-P] was stopped after10mL because he developed allergy with pruritus,headache, urticaria, hypotension and bronchospasm.
The man received emergency treatment, after which hissymptoms disappeared. Laboratory investigations showedthe following: IgG level 2030 mg/dL, total IgE 220 mg/dL,C3 level 83%, total complement activity 20% and inhibitortitre 7.2 BU. The next month, immune tolerance wasrestarted with octocog-α [Helixate; dosage not stated], butthe infusion was stopped after administration of a fewmillilitres due to a urticarial reaction. In December 2007, hedeveloped a similar reaction after administration of factorVIII inhibitor bypassing fraction [FEIBA; dosage and time toreaction onset not stated]; FEIBA was administered after ascarce response to eptacog-α for post-traumatichaemarthrosis at the left knee. He received no factor VIII-containing concentrates after December 2007 and, inNovember 2008, he had a inhibitor titre of 6.2 BU, an IgGlevel of 1860 mg/dL, an IgE level of 48 mg/dL and a totalcomplement activity of 90%.Rivolta GF, et al. Management of coronary artery disease in a severe haemophiliapatient with high titre inhibitor and anaphylaxis. Haemophilia 15: 1161-1163, No.5, 2009 - Italy 801154644
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Reactions 28 Nov 2009 No. 12800114-9954/10/1280-0001/$14.95 © 2010 Adis Data Information BV. All rights reserved
