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Sequential therapy with rFVIIa and activated prothrombin complex concentrates (aPCCs) in haemophilia B patient with inhibitor and life threatening bleeding

Abstract number: PP-TH-609

Katsarou¹ O., Kouraba¹ A., Anastasopoulou¹ I., Kotsi¹ P., Gavalaki¹ M., Markakis¹ K., Karafoulidou¹ A.

¹2nd Blood Transfusion and Haemophilia Center, Laikon General Hospital, Athens, Greece

How-to-cite Katsarou O, Kouraba A, Anastasopoulou I, Kotsi P, Gavalaki M, Markakis K, Karafoulidou A. Sequential therapy with rFVIIa and activated prothrombin complex concentrates (aPCCs) in haemophilia B patient with inhibitor and life threatening bleeding. Journal of Thrombosis and Haemostasis 2009; Volume 7, Supplement 2: Abstract PP-TH-609

Background: rFVIIa has proved efficacious to treat most bleeding episodes in haemophilia patients with inhibitors, however in refractory bleeding, successful use of sequential treatment with two different by passing agents has been reported. Haemophilia B patients with inhibitors, present a big challenge concerning the treatment of the bleeding episodes because the use of aPCCs may present a risk of anaphylactic reactions.

Aim: We present the complexity of the treatment in a case of a young haemophilia B patient with inhibitor and history of allergic reactions who presented with life threatening postoperative haemorrhage following laparotomy for kidney rupture and huge postoperative haematoma.

Case presentation: Severe haemophilia B patient, 27 years old, with history of high inhibitor and anaphylactic reactions, was admitted in our hospital diagnosed 15 days before with retroperitoneal haematoma, treated until his admission with FIX concentrates and corticosteroids. Inhibitor titre in his admission was 89 BU. Because of haemodynamic instability he was operated under rFVIIa monotherapy (dose 120 μg /kg every 2 and then 3 h) and a ruptured kidney was removed. Postoperatively, although rFVIIa dose was increased, haemostasis was not achieved, and sequential treatment was started with two by passing agents (rFVIIa 95 μg/kg every 4 h and Feiba 50 mg/kg every 12 h). The patient was stabilized for 20 days during which he was able to be reoperated successfully twice, for repairing interventions. In addition inhibitor titre was reduced to 9 BU. He was moved out of the ICU but after 4 days he presented with a severe anaphylactic reaction, treated with corticosteroids and antihistamines. aPCCs were interrupted and he continued with rFVIIa. Unfortunately he developed a resistant Gram negative infection that required new surgical intervention and 15 days later he died with septic shock and disseminated intravascular coagulation.

Conclusions: Sequential treatment with rFVIIa and aPCC has proved effective in achieving haemostasis in haemophilia B inhibitor patient with refractory to rFVIIa monotherapy bleeding. Despite the heavy and long term treatment, no thrombotic event was observed, but the possibility of an anaphylactic reaction seems to be present.

Disclosure of interest: none declared.

To cite this abstract use the following format:

Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number

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Date:	Unpresented
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Session name:	ISTH2009
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