Sylvie M N Mulliez1, Katrien M J Devreese1. 1. a Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology , Ghent University Hospital , Belgium.
Abstract
OBJECTIVES: Isolated acquired factor VII (FVII) deficiency is a rare haemorrhagic disorder. We report what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of acquired FVII deficiency. METHODS: We performed a literature search and included all articles published between 1980 and August 2015. RESULTS AND CONCLUSIONS: Acquired FVII deficiency has been reported in 42 patients. There are well-established clinical diseases associated with acquired FVII deficiency, most notably infections, malignancy and haematological stem cell transplantation. The exact pathogenesis of the diseases is still unknown, but different pathophysiological hypotheses have been suggested. The clinical manifestation of acquired FVII deficiency varies greatly in severity; asymptomatic course as well as severe life-threatening bleeding diathesis and fatal bleedings have been described.
OBJECTIVES: Isolated acquired factor VII (FVII) deficiency is a rare haemorrhagic disorder. We report what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of acquired FVII deficiency. METHODS: We performed a literature search and included all articles published between 1980 and August 2015. RESULTS AND CONCLUSIONS:Acquired FVII deficiency has been reported in 42 patients. There are well-established clinical diseases associated with acquired FVII deficiency, most notably infections, malignancy and haematological stem cell transplantation. The exact pathogenesis of the diseases is still unknown, but different pathophysiological hypotheses have been suggested. The clinical manifestation of acquired FVII deficiency varies greatly in severity; asymptomatic course as well as severe life-threatening bleeding diathesis and fatal bleedings have been described.
Entities:
Keywords:
Acquired bleeding disorder; Acquired factor VII deficiency; Acquired factor deficiency; Factor VII inhibitor; Isolated factor VII deficiency