2002
DOI: 10.1097/00001721-200209000-00011
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Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis

Abstract: Acquired factor X deficiency has been described in patients with amyloidosis but acquired factor V deficiency is quite rare. We report here a case of life-threatening bleeding and acquired factor V deficiency associated with primary amyloidosis. A 50-year-old man who had no previous hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis, gingival bleeding and hemospermia. The laboratory examination revealed that both the prothrombin time (PT) and the activated partial thromboplastin … Show more

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Cited by 45 publications
(33 citation statements)
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“…However, neither INR nor aPTT correlated well with the factor X levels [5]. Deficiencies of other coagulation factor deficiencies may coincide; these include factor IX deficiency [14], factor XII deficiency [15], von Willebrand factor deficiency [16], and factor V deficiency [17].…”
Section: Commentarymentioning
confidence: 94%
“…However, neither INR nor aPTT correlated well with the factor X levels [5]. Deficiencies of other coagulation factor deficiencies may coincide; these include factor IX deficiency [14], factor XII deficiency [15], von Willebrand factor deficiency [16], and factor V deficiency [17].…”
Section: Commentarymentioning
confidence: 94%
“…This deficiency has been attributed to adsorption of factor X selectively by amyloid fibrils [4,10,11], and it can be reversed by reduction of the amyloid burden with splenectomy [12,13] or by following successful treatment with HDM/SCT [9]. Deficiency of factors II [14],V [15,16], IX [14], and XIII [17] have also been reported. Over a 4-year-period, four patients with AL amyloidosis presented to Boston Medical Center with hemorrhage and a prolonged activated partial thromboplastin time (aPTT) but no detectable clotting factor deficiency.…”
Section: Introductionmentioning
confidence: 99%
“…Reports in the literature are also scarce [7,21,22,23] (table 2). To our knowledge only 1 report concerns a congenital bleeding disorder, a case of von Willebrand disease [22].…”
Section: Discussionmentioning
confidence: 99%
“…Beside the acquired conditions represented by anticoagulant therapy, a case due to amyloidosis and factor V deficiency has been reported [22]. In this case hemospermia was part of a severe bleeding diathesis, in part due to acquired factor V deficiency without circulating inhibitor, and in part to amyloidosis infiltration of vessel walls [23]. Anatomical reasons may be at the root of the rarity of hemospermia as a bleeding symptom, as the strong pelvic muscles and sphincters may exert a hemostatic pressure.…”
Section: Discussionmentioning
confidence: 99%