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Acquired isolated factor X deficiency associated with systemic amyloidosis. Case report and review of literature
Abstract:
The demonstration of acquired isolated factor X deficiency and of a biclonal homogeneous lambda and kappa light polypeptide chain proteinuria in a patient of 49 years having an obscure hepatomegaly gave clinical evidence for the rate, amyloidosis-associated factor X deficiency, which could be proven by postmortem examination. The mechanisms by which amyloid may affect factor X levels in this case may be preferentially binding to the amyloid fibrils of lambda light chain type.
Insights
This study details a rare case of acquired factor X deficiency linked to amyloidosis in a 49-year-old patient. Postmortem analysis confirmed the deficiency, suggesting amyloid binding to light chains as a potential cause.
Area of Science:
- Hematology
- Nephrology
- Pathology
Background:
- Acquired factor X deficiency is a rare bleeding disorder.
- Amyloidosis can manifest with diverse clinical presentations.
- Biclonal gammopathy indicates an underlying plasma cell dyscrasia.
Observation:
- A 49-year-old patient presented with obscure hepatomegaly and bleeding symptoms.
- Laboratory findings revealed isolated acquired factor X deficiency.
- Urine analysis showed biclonal homogeneous lambda and kappa light chain proteinuria.
Findings:
- Clinical and postmortem examinations confirmed amyloidosis-associated factor X deficiency.
- The deficiency was strongly correlated with the presence of amyloid deposits.
- Amyloid fibrils, specifically of the lambda light chain type, were implicated.
Implications:
- This case highlights a novel mechanism of acquired factor X deficiency.
- Understanding amyloid interactions is crucial for managing bleeding risks in amyloidosis.
- Further research into light chain amyloidosis and coagulation factor interactions is warranted.