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Acquired factor X deficiency: difficulties to establish etiologic diagnosis
Abstract number: PP-TH-204
Kaci1 Z., Ahmidatou1 H., Zidani1 N., Chennoukh1 K., Berkouk1 Y., Belhani1 M.
11Haematology and Blood bank, University Hospital of Beni Messous, Algiers, Algeria
How-to-cite Kaci Z, Ahmidatou H, Zidani N, Chennoukh K, Berkouk Y, Belhani M. Acquired factor X deficiency: difficulties to establish etiologic diagnosis. Journal of Thrombosis and Haemostasis 2009; Volume 7, Supplement 2: Abstract PP-TH-204
Factor X is one of the vitamin K dependent serine proteases wich plays a crucial role in the coagulation. Acquired factor X deficiency is rare, usually complication of amyloidosis. The mechanism is the absorption of FX onto amyloidosis fibrils. It is associated with severe bleeding that is difficult to control. We report two cases of acquired FX deficiency. Objectives are to determine severity of bleeding and difficulties of etiologic diagnostic. Our patients have no previous personal or family history of bleeding. The first patient is 47 years old man; he is admitted with one month lasting history of muscular pain, hematoma and ecchymoses around eyes. PT and APTT were prolonged, platelet count, Fibrinogen and D. Dimer were normal. There was deficiency of factor X (11%) and normal levels of other vitamin K-dependent factors. FX inhibitors were absent. There was neither improvement of bleeding or correction of the PT after transfusion of fresh frozen plasma. Echocardiography showed signs of amyloidosis. Absence of monoclonal antibodies; absence of plamocyts in myelogram. Gum and bone biopsies (performed with substitutive treatment: prothrombin complex concentrate and Novoseven) didnt't show red congo positive straining fibrils. The second patient is 58 year-old man with cardiac pathology. Routine Hemostasis tests showed FX deficiency (7.8%) without inhibitors. He presented few months later, severe gastrointestinal bleeding, his hematocrit has decreased (from 39% to 15% in 24 h).Serum immuno electrophoresis showed IgG monoclonal antibodies without plasmocyts myelography. Rectal and bone biopsies don't demonstrate amyloidosis.
Discussion: In our patients, bleeding are severe: muscular and gastrointestinal and difficult to stop. First case, diagnosis of amyloidosis was clinically suspected: ecchymoses around eyes and echocardiography signs. Second case, amyloidosis was suspected because of IgG monoclonal pic, cardiac pathology and the factor X deficiency. Acquired factor X deficiency is usually a complication of amyloidosis, however, this diagnosis could not be confirmed. It's necessary to practice biopsies in the cardiac tissue, this procedure is very agressive and at high risk of bleeding.
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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