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Acquired isolated factor VII deficiency in a patient with myxoid pleomorphic liposarcoma, case report

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논문

Acquired isolated factor VII deficiency in a patient with myxoid pleomorphic liposarcoma, case report

학술지

Medicine

저자명

Aljabry, Mansour; Aleid, Manar; Almutairi, Shahad; AlSerhani, Reema; Alsahil, Shahad; Alotaibi, Ghazi

초록

<P><B>Introduction:</B></P><P>Acquired 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.</P><P><B>Patient concerns and diagnosis:</B></P><P>We 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.</P><P><B>Interventions and outcome:</B></P><P>The 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.</P><P><B>Conclusion:</B></P><P>This 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.</P>

발행연도

2023

발행기관

Lippincott WilliamsWilkins

ISSN

0025-7974

ISSN

1536-5964

102

52

페이지

pp.e36621

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논문; 2023-12-29

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