Factor X Deficiency

Blood coagulation disorder usually inherited as an autosomal recessive trait, though it can be acquired. It is characterized by defective activity in both the intrinsic and extrinsic pathways, impaired thromboplastin time, and impaired prothrombin consumption.

Also Known As:

Deficiency, Factor 10; Deficiency, Factor Ten; Deficiency, Factor X; Deficiency, Stuart-Prower; Deficiency, Stuart-Prower Factor; Factor 10 Deficiency; Factor Ten Deficiency; Stuart-Prower Deficiency; Stuart-Prower Factor Deficiency; Deficiencies, Factor 10; Deficiencies, Factor Ten; Deficiencies, Factor X; Deficiency, Stuart Prower; Deficiency, Stuart Prower Factor; Factor 10 Deficiencies; Factor Ten Deficiencies; Factor X Deficiencies; Stuart Prower Deficiency; Stuart Prower Factor Deficiency; Ten Deficiencies, Factor

Networked: 197 relevant articles (7 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Immunoglobulin Light-chain Amyloidosis
2.	Hemorrhage
3.	Paraproteinemias (Monoclonal Gammopathy)
4.	Neoplasm Metastasis (Metastasis)
5.	Adenocarcinoma

Experts

1.	Norton, M: 5 articles (11/2018 - 05/2016)
2.	Girolami, Antonio: 4 articles (01/2019 - 08/2008)
3.	Shapiro, A: 3 articles (01/2018 - 05/2016)
4.	Batsuli, Glaivy: 2 articles (07/2022 - 10/2020)
5.	McGuinn, Catherine E: 2 articles (07/2022 - 10/2020)
6.	Liesner, R: 2 articles (11/2018 - 01/2009)
7.	Ghosh, Kanjaksha: 2 articles (01/2017 - 01/2014)
8.	Auerswald, G: 2 articles (09/2016 - 09/2006)
9.	Austin, S K: 2 articles (05/2016 - 05/2016)
10.	FX Investigators Group: 2 articles (05/2016 - 05/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Factor X Deficiency:

1.	Factor X (Stuart Factor)IBA 07/01/2021 - "Plasma-derived factor X concentrate compassionate use for hereditary factor X deficiency: Long-term safety and efficacy in a retrospective data-collection study." 05/25/2018 - "Use of a High-Purity Factor X Concentrate in Turkish Subjects with Hereditary Factor X Deficiency: Post Hoc Cohort Subanalysis of a Phase 3 Study." 01/01/2005 - "Genetic analysis of hereditary factor X deficiency in a French patient of Sri Lankan ancestry: in vitro expression study identified Gly366Ser substitution as the molecular basis of the dysfunctional factor X." 11/01/1988 - "Congenital factor X (Stuart-Prower factor) deficiency: a family study." 11/01/2023 - "This paper describes the approach to pre-clinical development and characterization of a high-purity plasma-derived factor X concentrate, to achieve orphan drug marketing authorization for the treatment of hereditary factor X deficiency. "
2.	Amyloid (Amyloid Fibrils)IBA 01/01/2023 - "Clinical features and laboratory diagnostic issues of non-immune, non-amyloid related acquired factor X deficiency." 09/01/2012 - "Acquired, non-amyloid related factor X deficiency: review of the literature." 01/01/2004 - "Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency." 03/01/2018 - "Primary amyloid light chain (AL) amyloidosis and acquired factor X deficiency were diagnosed. " 09/01/2017 - "Amyloidosis of the GI tract presenting with massive GI bleed is extremely rare and is thought to be related to small-vessel fragility due to amyloid infiltration and impaired hemostasis caused by factor X deficiency. "
3.	Fibrinogen (Factor I)FDA Link 01/01/1996 - "These included 8 patients with factor X deficiency, 7 with factor XIII deficiency, 4 each with fibrinogen and factor VII deficiency and 1 with factor V deficiency. " 07/01/2012 - "Inherited coagulation defects were found in 27.2%; hemophilia A (70.6%), hemophilia B (13.9%), factor I deficiency (2.3%), factor V deficiency (1.6%), factor X deficiency (4.2%), factor VII deficiency (2.6%), factor XIII deficiency (1.1%), combined factor deficiency (2.1%) and unclassified coagulation disorders in 1.6% of studied patients. " 05/01/1991 - "Induction treatment led to the control of DIC with an almost parallel increase of fibrinogen and factor X up to normal in all patients with factor-X deficiency who achieved complete remission. " 01/01/2014 - "Pubmed search was used (www.pubmed.com) to explore the published literature from India on RBDs using the key words "rare bleeding disorders", "mutations", "India", "fibrinogen", "afibrinogenemia", "factor II deficiency", "prothrombin" "factor VII deficiency", "factor V deficiency", "factor X deficiency", "factor XI deficiency", "combined factor V and VIII deficiency", "factor XIII deficiency", "Bernard Soulier syndrome" and "Glanzmanns thrombasthenia" in different combinations. " 01/01/1997 - "Factor X deficiency is the most common cause of bleeding manifestations, although deficiencies of other clotting factors, a disruption in the conversion of fibrinogen to fibrin, and circulating heparin-like anticoagulants have also been reported. "
4.	Vitamin K FDA Link 01/01/1986 - "The acquired factor X deficiency resolved after fresh frozen plasma and vitamin K administration, although some spontaneous improvement was noted. " 01/01/2021 - "Acquired factor X deficiency has been associated with plasma cell dyscrasias, amyloids, and use of vitamin K antagonists. " 01/01/2022 - "This report presents a unique factor X deficiency case with limited treatment options to improve factor X levels after failed vitamin K administration and an allergic reaction to fresh frozen plasma. " 01/01/2017 - "Eight patients (2.8%) had vitamin K-dependent clotting factor deficiency, Bernard-Soulier syndrome was diagnosed in seven patients (2.5%), factor X deficiency in 2 (0.7%), factor II deficiency in 2 (0.7%), factor XI deficiency and combined factor V and VIII deficiency in 1 (0.4%) patient each. "
5.	AutoantibodiesIBA 03/01/2023 - "Identification of non-neutralizing anti-factor X autoantibodies in three Japanese cases of autoimmune acquired factor X deficiency." 01/01/2019 - "Complete remission in a bleeding patient with idiopathic autoimmune factor X deficiency caused by non-neutralizing anti-factor X autoantibody." 01/01/2023 - "Management of Acquired Factor X Deficiency Caused by In Vitro Non-neutralizing Anti-factor X Autoantibodies with Pre-emptive Corticosteroid Therapy."
6.	SirtuinsIBA 10/01/2021 - "Upregulating sirtuin 6 ameliorates glycolysis, EMT and distant metastasis of pancreatic adenocarcinoma with krüppel-like factor 10 deficiency."
7.	Dacarbazine (DIC)FDA LinkGeneric 05/01/1991 - "We conclude that there is a coincidence of isolated factor-X deficiency and DIC in some patients with ANLL. " 05/01/1991 - "In one patient, recurrence of leukemia was associated with reoccurrence of DIC and marked factor-X deficiency. " 05/01/1991 - "Three of the patients with DIC (two with M3, one with M5 leukemia) had a marked isolated factor-X deficiency (factor X:C 21%, 33%, and 41%, respectively). " 05/01/1991 - "Induction treatment led to the control of DIC with an almost parallel increase of fibrinogen and factor X up to normal in all patients with factor-X deficiency who achieved complete remission. " 05/01/1991 - "In none of the 141 patients without DIC was a marked isolated factor X deficiency observed; two patients had moderately reduced factor X:C levels but normal liver-synthesized proteins. "
8.	Prothrombin (Factor II)IBA 08/01/2016 - "Herein, we report a case of a 56-year-old white man with congenital factor X deficiency who was scheduled for major neurosurgery and who was treated perioperatively with 4-factor prothrombin complex concentrate (4F-PCC). " 08/01/2016 - "Successful Use of Four Factor-Prothrombin Complex Concentrate for Congenital Factor X Deficiency in the Setting of Neurosurgery." 04/01/2010 - "On the contrary no sure venous or arterial thrombotic event has ever been reported in congenital prothrombin or Factor X deficiency. " 01/01/2009 - "Prophylaxis with prothrombin complex concentrate in four children with severe congenital factor X deficiency." 03/01/2001 - "Prophylactic treatment of severe factor X deficiency with prothrombin complex concentrate."
9.	Factor VII (Proconvertin)IBA 01/01/2021 - "Factor VII and factor X deficiency in a child with a chromosome 13q duplication and deletion." 09/01/2014 - "One patient was detected with single factor X deficiency and another with single factor VII (FVII) deficiency, whereas four other patients had complex factor deficiencies. " 12/01/2011 - "[Gene analysis of a combined inherited factor VII and factor X deficiency pedigree]." 08/01/1990 - "Factor VII or Factor X deficiencies also developed in several of these patients. " 01/01/2017 - "Synergistic effect of factor VII gene polymorphisms causing mild factor VII deficiency in a case of severe factor X deficiency."
10.	Factor V (Coagulation Factor V)IBA 10/01/2021 - "Congenital combined deficiency of factor V and factor X deficiency is extremely rare. " 09/01/2013 - "Isolated factor V and factor X deficiency is a very rare condition affecting 1 in 500,000-1,000,000 persons worldwide. " 09/01/2013 - "Rare case of combined factor V and factor X deficiency in pregnancy: presenting as secondary postpartum haemorrhage in first pregnancy and successful outcome in second pregnancy." 10/01/2021 - "Posterior Fossa Hematoma Following Minor Trauma in an Infant with Rare Combined Factor V and Factor X Deficiency." 09/01/2008 - "There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). "

Therapies and Procedures

1.	Splenectomy 03/01/2001 - "Splenectomy ameliorates the factor X deficiency, but achieving satisfactory haemostasis for this operation is problematic. " 12/01/2006 - "Splenic infiltration in primary amyloidosis can be an indication for splenectomy, where removal of a large spleen can also reverse an acquired factor X deficiency. " 01/01/1998 - "Splenectomy in an uraemic patient with acquired factor X deficiency due to AL amyloidosis." 03/01/1983 - "Resolution of factor X deficiency in primary amyloidosis following splenectomy." 11/08/1979 - "Factor X deficiency in primary amyloidosis: resolution after splenectomy."
2.	Drug Therapy (Chemotherapy) 05/01/2001 - "Successful treatment of an acquired haemorrhagic diathesis due to factor X deficiency with chemotherapy." 03/01/1992 - "Acquired factor X deficiency and disseminated intravascular coagulation in a case of metastasizing carcinoma of the stomach and its course under chemotherapy." 12/07/1992 - "The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. " 01/01/2010 - "[A patient with AL amyloidosis and severe factor X deficiency has been in complete haematological remission with normal factor X activity for 7 years following high-dose chemotherapy. " 03/01/2004 - "Successful treatment of systemic amyloidosis with hepatic involvement and factor X deficiency by high dose melphalan chemotherapy and autologous stem cell reinfusion."
3.	Therapeutics 08/01/2016 - "As such, given the wide availability of 4F-PCC, it may be considered as a first-line therapy and an alternative to fresh frozen plasma for factor X deficiencies, particularly in high-risk operative cases. " 12/01/2002 - "The role of primary prophylactic factor replacement therapy in children with severe factor X deficiency." 07/01/1994 - "We reviewed the outcome of four pregnancies in a patient with factor X deficiency, and looked at the effect of factor replacement therapy on pregnancy. " 01/01/1985 - "Replacement therapy for congenital Factor X deficiency." 03/01/2018 - "The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency."
4.	Plasma Exchange 03/01/2008 - "Plasma exchange in a case of severe factor X deficiency in pregnancy: critical review of the literature." 01/01/1997 - "In this manuscript we report the clinical application of plasma exchange in the management of a patient with systemic light chain amyloidosis with acquired factor X deficiency." 01/01/2004 - "Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency."
5.	Cesarean Section (Caesarean Section) 03/18/2020 - "CASE REPORT In this report, we present the case of a successful pregnancy and term delivery by elective cesarean section in a 39-year-old primigravida with congenital factor X deficiency. " 04/01/2012 - "Subgaleal hemorrhage in a neonate with factor X deficiency following a non-traumatic cesarean section." 07/01/2010 - "Subclinical factor X deficiency may increase hemorrhage in women undergoing cesarean section." 03/18/2020 - "The patient's factor X deficiency (0.19 kIU/L) was treated using 4 units of solvent-detergent-treated fresh frozen plasma (SD-FFP) 1 h before the cesarean section, leading to hemostatic levels of factor X and an uneventful intraoperative course. "