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Congenital FVII Deficiency and Pulmonary Embolism: A Critical Appraisal of All Reported Cases

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Fourteen patients with congenital factor VII (FVII) deficiency were reported to have had pulmonary embolism. All patients were type 2 defects with variably low activity but normal or near-normal antigen. Concomitant deep vein thrombosis was present in 7 instances. The majority of patients had no or only a mild bleeding tendency. Associated prothrombotic risk factors were present in 11 patients (old age, surgery, substitution therapy with prothrombin complex, plasma-derived or activated FVII concentrates). Pulmonary embolism was usually moderate or severe. In 2 cases, it was fatal. Only 4 patients were studied by means of molecular biology techniques. The Arg304Gln mutation was found in 5 of the 8 alleles. Heparin and Coumadin together with adequate substitution therapy were carried out in 5 patients with satisfactory results. The FVII deficiency does not grant a sure protection from venous thromboembolism.

Keywords FVII deficiency, pulmonary embolism, replacement therapy

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Informasi Detail

Judul Seri

Clinical and Applied Thrombosis/Hemostasis

No. Panggil

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Penerbit

: SAGE Publications Ltd., 2013

Deskripsi Fisik

Clinical and Applied Thrombosis/Hemostasis 19(1) 55-59 , 2013

Bahasa

English

ISBN/ISSN

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Tipe Isi

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Tipe Media

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Tipe Pembawa

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Edisi

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Subjek

FARMASI

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