ISSN 2997-1036
International Journal of Hematology | Vol. 11, No. 7, July 2020 | pp. 49–56
DOI: 10.46882/2020/IJH/000127
Case Report
Title: Spontaneous massive retroperitoneal hemorrhage secondary to acquired Factor VII inhibitor development in an elderly patient: Eradication with 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 directed against coagulation Factor VII is an exceptionally rare clinical condition that causes catastrophic bleeding events in elderly populations. We report a 74-year-old male who presented with sudden, unprovoked left flank pain, lower abdominal distension, and hypovolemic shock. Abdominal computed tomography confirmed a massive retroperitoneal hematoma measuring 12.4 × 8.5 cm without prior trauma or anticoagulant exposure. Coagulation profiles demonstrated isolated, severe prolongation of prothrombin time (52.4 seconds) with a normal activated partial thromboplastin time. A 1:1 mixing study with normal pooled plasma failed to correct the prothrombin time, indicating a specific extrinsic pathway inhibitor. Functional assays confirmed severely depressed Factor VII activity (< 1.0%), and a Bethesda assay quantified a Factor VII inhibitor titer of 24.5 Bethesda Units. Hemostasis was achieved using recombinant activated Factor VII bypassing agents (90 μg/kg every 3 hours) alongside supportive measures. Subsequent immunosuppressive therapy with oral prednisone paired with cyclosporine-A (3 mg/kg/day) cleared the inhibitor (0 BU) by week 8. This case demonstrates that acquired Factor VII autoantibodies can cause life-threatening retroperitoneal bleeding, requiring immediate diagnostic differentiation and multi-modal therapeutic strategies.
Keywords: Acquired factor VII inhibitor, retroperitoneal hemorrhage, extrinsic pathway, bypassing agents, cyclosporine
Manuscript Timeline: Received: April 18, 2020; Revised: May 22, 2020; Accepted: June 10, 2020; Published: July 16, 2020