FACTOR-X DEFICIENCY: A RARE DISORDER TO BE LOOKED FOR IN CASES OF CONGENITAL BLEEDING TENDENCY
Main Article Content
Abstract
ABSTRACT
Factor X deficiency is a rare, autosomal recessive disorder that involves the coagulation cascade. People with this disorder present with a myriad of early life bleeding complications. We report here a 23-year-old male, who presented with bleeding complications (prolonged bleeding from surgical wounds, unresolving hematomas, uncontrolled nasal bleeding, hematuria and hematochezia) at different stages of his life but was diagnosed very late for factor X deficiency after comprehensive evaluation for clotting factors. He was managed by repeated transfusions of fresh frozen plasma and detailed counselling. Factor X deficiency is a rare life-threatening disease and must be differentiated from other disorders of coagulation. The patients are advised to avoid activities with high levels of physical contact, unnecessary invasive interventions and drugs that remarkably affect liver functions and seek immediate medical care in case of bleeding from any site.
KEYWORDS
Hemorrhage (MeSH); Bleeding disorder (Non-MeSH); Factor X Deficiency (MeSH); Inherited (Non-MeSH); Blood Coagulation Disorders, Inherited (MeSH); Blood Coagulation Disorders (MeSH).
Article Details
Work published in KMUJ is licensed under a
Creative Commons Attribution 4.0 License
Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
References
REFERENCES
Uprichard J, Perry DJ. Factor X deficiency. Blood Rev 2002;16 (2):97-110.
Brown DL, Kouides PA. Diagnosis and treatment of inherited factor X deficiency. Haemophilia 2008;14(6):1176-82. DOI: 10.1111/j.1365-2516.2008.01856.x.
Anwar M, Hamdani SN, Ayyub M, Ali W. Factor X deficiency in North Pakistan. J Ayub Med Coll Abbottabad 2004;16(3):1-4.
Borhany M, Shamsi T, Fatima N, Fatima H, Naz A, Patel H. Rare Bleeding Disorders are not so Rare in Pakistan. J Hematol Thromb Dis 2013;2:122. DOI: 10.4172/2329-8790.1000122
Khalid S, Bilwani F, Adil SN, Khurshid M. Frequency and clinical spectrum of rare inherited coagulopathies--a tricenter study. J Pak Med Assoc 2008;58(8):441-4.
Coucke L, Trenson S, Deeren D, Van Haute I, Devreese K. Life-threatening bleeding tendency provoked by an acquired isolated factor X deficiency associated with respiratory infection. Ann Hematol 2013; 92(10):1437-8. DOI: 10.1007/s00277-013-1718-0.