Abstract Number: PB0727
Meeting: ISTH 2021 Congress
Background: Congenital factor FVII deficiency is the most wideprad of the rare bleeding disorders . Clinical features are extremely variable ranging from mild cutaneo-mucosal bleeding to lethal cerebral haemorrhges and are often not correleted with FVII coagulant activity (FVII:c). Conversely, some patients can develop arterial or venous thrombosis.
Aims: The purpose of this study is to report thromboembolism in a FVII deficieny’s patient.
Methods: case report description.
Results: A 23 year-old woman was admitted to the department of pneumology due to respiratory distress and tachycardia. Her medical history included diabetes diagnosed since 6 months ago. The patient reported an exertional dyspnea that had progressed for months. A CT angiography of the chest showed a pulmonary hypertension and absence of pulmonary embolism.The pulmonary hypertension was confirmed by transthoracic echocardiogram (PAPS =55mmHg).
An etiological investigation turned out to be negative. In fact, familial pulmonary hypertension history, congenital heart disease, connectivity and portal hypertension were discarded.
The haemostasis tests revealed an isoleted prolonged prothrombin time of 24,3 seconds. Level of FVII activity assayed by a rabbit brain reagent was 4%. Neverless, she had no history of significant bleeding history.
Notably, the patient had not a deficiency of one of the natural inhibitors of coagulation (PS=70%, PC =84%, AT=100%).No resistance to activated protein C (rAPC) was found .Screening for antiphospholipid antibodies was negative.
Thus, arterial pulmonary hypertension was potentially secondary to chronic thromboembolism. In this regard, about 3% of patients with FVII deficiency can develop chronic thromboembolism.
The patient was discharged with diuretic and long-term oxygen.
Conclusions: Thrombotic events are uncommon in congenital bleeding disorders. Among the rare coagulation disorders, FVII deficiency has been reported to be more frequently associated with thrombosis. The clinician must be aware that thrombosis can occur in FVII deficiency under certain provoked circumstances and severe deficiency does not protect against thrombosis.
To cite this abstract in AMA style:krichen I, Moussa N, Elloumi Y, Megdich F, Fekih W, Kammoun Y, Kammoun S, Kallel C. The Paradoxal Association beetween Congenital FVII Deficiency and Thromboembolism [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/the-paradoxal-association-beetween-congenital-fvii-deficiency-and-thromboembolism/. Accessed December 6, 2023.
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