• Mansour Aljabry, Manar Aleid, Shahad Almutairi, Reema AlSerhani, Shahad Alsahil, and Ghazi Alotaibi.
• Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
• Medicine (Baltimore). 2023 Dec 29; 102 (52): e36621e36621.

IntroductionAcquired factor VII (FVII) deficiency is a rare condition with various causes, including acquired inhibitors to FVII, liver disease, and malignancies. Myxoid pleomorphic liposarcoma is a rare and aggressive form of soft tissue sarcoma that can cause a range of clinical manifestations, including bleeding and clotting disorders.Patient Concerns And DiagnosisWe present a case report of a 21-year-old man with severe acquired FVII deficiency due to mediastinal myxoid pleomorphic liposarcoma. The patient presented with elevated International normalized ratio (INR) and a severe reduction in FVII coagulant activity, unresponsive to conventional therapy. While an acquired inhibitor to FVII was initially suspected, negative results from laboratory testing, including protein G sepharose adsorption and a Bethesda assay using Immunoglobulin G purified from patient plasma, made the diagnosis of an acquired inhibitor to FVII uncertain.Interventions And OutcomeThe patient underwent surgical resection of the tumor, supported by recombinant FVII infusion, leading to the normalization of coagulation parameters. However, a relapse of the disease was detected 6 months later when he was noted to have a decline in FVII levels.ConclusionThis case highlights the importance of considering rare causes of bleeding and clotting disorders, particularly in unresponsive or atypical presentations. It also underscores the need for close monitoring and follow-up in patients with acquired FVII deficiency, even after successful treatment.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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