Abstract
Acquired factor VII (FVII) deficiency in the absence of vitamin K deficiency, oral anticoagulant therapy, synthetic liver dysfunction, or DIC is rare, with only a handful of cases thus far reported. In the period from 1990 to 1996 we identified eight patients with acquired FVII deficiency, all of whom presented with prolongation of the prothrombin time (PT) in the first 2 weeks following stem cell transplantation (SCT). The mean plasma FVII clotting activity (FVII:c) was 22% (range 8-35%) with an approximately equivalent reduction in FVII antigen (FVII:Ag) level. Mean plasma levels of fibrinogen and factors II, V, IX, and X were normal. Protein C activity was significantly depressed in only one of the three patients in whom it was measured. Several patients experienced bleeding complications, and hemorrhage directly accounted for death in two cases. Veno-occlusive disease of the liver developed in three patients. We conclude that FVII deficiency should be considered in the differential diagnosis of prolonged PT in patients who have recently undergone SCT. The mechanism of this acquired deficiency state remains to be defined.
References
Sep 1, 1991·Thrombosis Research·M L ScrobohaciE Gluckman
Aug 1, 1990·Cancer·P A KaufmanW P Peters
Apr 1, 1989·American Journal of Clinical Pathology·O K NdimbieS M Saeed
Mar 1, 1989·British Journal of Haematology·D WeisdorfD S Fair
Apr 1, 1985·Scandinavian Journal of Haematology·M FischerE Deutsch
Jun 1, 1983·American Journal of Clinical Pathology·D A TriplettJ Schaeffer
Oct 1, 1994·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·E de RaucourtA M Fischer
May 1, 1994·The Medical Clinics of North America·E F Mammen
Jan 1, 1996·Haemostasis·J KristensenU Hedner
Feb 1, 1996·European Journal of Clinical Investigation·R M PerenboomJ W van der Meer
May 1, 1997·Bone Marrow Transplantation·Y D ParkK Kawa-Ha
Mar 1, 1997·Haemostasis·C BironJ F Schved
Sep 10, 1998·American Journal of Respiratory and Critical Care Medicine·K J PriceB S Andersson
Jan 5, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·J Ingerslev, H L Kristensen
Mar 30, 1999·Transplantation·S NevoG B Vogelsang
Aug 12, 1999·British Journal of Haematology·B WhiteO P Smith
Citations
Sep 18, 2013·Molecular Therapy : the Journal of the American Society of Gene Therapy·Yingyu ChenQizhen Shi
May 29, 2004·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Charles G MullighanJohn V Lloyd
Jan 13, 2009·Pediatric Blood & Cancer·Jeremy Granger, Vinod K Gidvani
Feb 26, 2013·Haemophilia : the Official Journal of the World Federation of Hemophilia·G LeeAra D Metjian
Sep 25, 2015·Acta Clinica Belgica·Sylvie M N Mulliez, Katrien M J Devreese
Aug 16, 2005·Annales Françaises D'anesthèsie Et De Rèanimation·E MeaudreB Palmier
Jul 21, 2015·Revista brasileira de hematologia e hemoterapia·Vanessa Afonso da SilvaFabrício Frederico Mendes Martins
Jul 6, 2014·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Soumaya AnounAsmaa Quessar
Sep 4, 2015·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·Antonio GirolamiAnna Maria Lombardi
Jan 16, 2016·Therapeutic Advances in Gastroenterology·Muhammad Wasif SaifRobert B Diasio
Jun 15, 2004·Bone Marrow Transplantation·P KaloyannidisA Anagnostopoulos
Dec 12, 2002·Bone Marrow Transplantation·K HicksJ L Gajewski
Dec 27, 2018·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Mahmood ShamsMohammad Reza Rezvani
Nov 23, 2006·Blood·Yvonne LohRichard K Burt
Jun 9, 2020·Journal of Cellular Physiology·Yingyu ChenQizhen Shi
Jun 11, 2020·Journal of Pediatric Hematology/oncology·Abed Abu-QuiderJoseph Kapelushnik
Dec 31, 2014·Pediatrics·Jeong A Park, Byoung-Ju Kim