ISSN 2997-1036
International Journal of Hematology | Vol. 9, No. 11, November 2018 | pp. 81–88
DOI: 10.46882/2018/IJH/000107
Case Report
Title: Acquired Factor X inhibitor development presenting with spontaneous massive hemarthrosis in an elderly patient: Successful eradication with oral cyclosporine
Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³
Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Surgery, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
Abstract: Spontaneous development of neutralizing autoantibodies against coagulation Factor X is an exceptionally rare clinical condition that causes catastrophic bleeding complications in elderly individuals. We report a 74-year-old male who presented with sudden, unprovoked, and massive swelling of his right knee joint accompanied by hypovolemic shock. Arthrocentesis yielded 450 ml of gross blood, confirming acute hemarthrosis without prior trauma or anticoagulant therapy. Coagulation profiles demonstrated concurrent prolongation of both prothrombin time (48.5 seconds) and activated partial thromboplastin time (88.4 seconds). A 1:1 mixing study with normal pooled plasma failed to correct either parameter, pointing to a common pathway inhibitor. Functional factor assays confirmed severely depressed Factor X activity (< 1.5%), and a Bethesda assay quantified a Factor X inhibitor titer of 22.0 Bethesda Units. Hemostasis was achieved using prothrombin complex concentrates (50 IU/kg every 12 hours) alongside supportive measures. Immunosuppressive therapy with high-dose corticosteroids was unsuccessful. Subsequently, oral cyclosporine-A (3 mg/kg/day) was initiated, successfully clearing the inhibitor (0 BU) and normalizing Factor X levels (88.4%) by week 6. This case highlights the need to consider common pathway inhibitors in elderly patients with spontaneous hemarthrosis.
Keywords: Acquired factor X inhibitor, hemarthrosis, common pathway, prothrombin complex concentrate, cyclosporine
Manuscript Timeline: Received: August 18, 2018; Revised: September 24, 2018; Accepted: October 10, 2018; Published: November 15, 2018