Abstract Number: PB0701
Meeting: ISTH 2021 Congress
Background: FVII deficiency is considered a rare bleeding disorder. Development of FVII inhibitors is very uncommon and represents a serious complication. Management of bleeding episodes in patients with inhibitors is a challenge in the treatment of this disorder.
Aims: Evaluation with global assays of the hemostatic state of a patient with FVII deficiency and inhibitor with hemorrhagic and thrombotic complications.
Methods: Blood samples were collected with CTI and were assessed by thromboelastometry (ROTEM) and thrombin generation test (TGT). ROTEM was performed with low concentration of tissue factor (EXTEM diluted_1:50000). TGT was triggered with PPP-Low. Functional FVII and inhibitor levels were also evaluated.
Results: Two year-old girl diagnosed with severe FVII deficiency (FVII 7%) on prophylaxis with rFVIIa since first month of life due to an intracranial haemorrhage. At 6 months she developed inhibitor against FVII (9.8 BU). As shown in Fig.1, ROTEM and TGT showed that FVII deficiency with inhibitor caused a delayed clotting time (CT) and thrombin generation Lag-time (LT). At the age of 23 months she was admitted at hospital because of anemization and Port-A-Cath infection. Prophylaxis treatment was suspended due to thrombosis of peripheral venous access, but global tests continued showing an hypocoagulable state with delayed CT and LT. After 7 days without prophylaxis and high inhibitor titer (59 BU), she developed a new intracranial haemorrhage, needing treatment with continuous rFVIIa infusion, immunoglobulines and rituximab to erradicate the inhibitor. Hemostatic parameters improved during the administration of these drugs and inhibitor levels decreased. After restarting prophylaxis due to resolution of bleeding episodes, inhibitor and hypocoagulable state were observed again.
Conclusions: This case shows the relevance of monitoring hemostasis in FVII deficiency patients with global coagulation assays especially in presence of bleeding and thrombotic events.
To cite this abstract in AMA style:Rivas Pollmar MI, Garcia Arias-Salgado E, Alvarez Roman MT, Monzon Manzano E, Acuña Butta P, Martin Salces M, Garcia Barcenilla S, Cebanu T, Gonzalez Zorrilla E, Butta Coll N, Jimenez Yuste V. Evaluation of Coagulation Profile in FVII Deficiency with Inhibitor Using Global Coagulation Tests: A Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/evaluation-of-coagulation-profile-in-fvii-deficiency-with-inhibitor-using-global-coagulation-tests-a-case-report/. Accessed November 29, 2021.
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