Acquired Factor X Deficiency in Patients With Primary Light Chain Amyloidosis

Siroj Dejhansathit, Attaya Suvannasankha

Research output: Contribution to journalArticle

Abstract

Acquired factor X (FX) deficiency is a rare but serious complication of primary amyloidosis, presumably caused by the binding of amyloid proteins to the clotting factors. The prolonged prothrombin time, partial thromboplastin time, and low FX level, which are correctable by mixing study, are the disease hallmarks. An immediate goal of care is to stop bleeding. Clotting factor replacement requires close monitoring of coagulogram and FX levels due to varying FX clearance among patients. High-purity FX is currently approved for hereditary FX deficiency and has been successfully used in some acquired FX deficiency cases. Ongoing bleeding risk complicates the treatment decision. Novel therapies yielding rapid and deep response reduce amyloid protein production and improve long-term outcome.

Original languageEnglish (US)
JournalJournal of Investigative Medicine High Impact Case Reports
Volume7
DOIs
StatePublished - Mar 1 2019

Fingerprint

Factor X Deficiency
Factor X
Amyloidosis
Amyloidogenic Proteins
Light
Blood Coagulation Factors
Patient Care Planning
monitoring
Hemorrhage
Disease
Partial Thromboplastin Time
Monitoring
Prothrombin Time
Carrier Proteins
time
Therapeutics

Keywords

  • AL amyloidosis
  • blood coagulation disorders
  • factor X deficiency

ASJC Scopus subject areas

  • Epidemiology
  • Safety, Risk, Reliability and Quality
  • Safety Research

Cite this

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abstract = "Acquired factor X (FX) deficiency is a rare but serious complication of primary amyloidosis, presumably caused by the binding of amyloid proteins to the clotting factors. The prolonged prothrombin time, partial thromboplastin time, and low FX level, which are correctable by mixing study, are the disease hallmarks. An immediate goal of care is to stop bleeding. Clotting factor replacement requires close monitoring of coagulogram and FX levels due to varying FX clearance among patients. High-purity FX is currently approved for hereditary FX deficiency and has been successfully used in some acquired FX deficiency cases. Ongoing bleeding risk complicates the treatment decision. Novel therapies yielding rapid and deep response reduce amyloid protein production and improve long-term outcome.",
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AB - Acquired factor X (FX) deficiency is a rare but serious complication of primary amyloidosis, presumably caused by the binding of amyloid proteins to the clotting factors. The prolonged prothrombin time, partial thromboplastin time, and low FX level, which are correctable by mixing study, are the disease hallmarks. An immediate goal of care is to stop bleeding. Clotting factor replacement requires close monitoring of coagulogram and FX levels due to varying FX clearance among patients. High-purity FX is currently approved for hereditary FX deficiency and has been successfully used in some acquired FX deficiency cases. Ongoing bleeding risk complicates the treatment decision. Novel therapies yielding rapid and deep response reduce amyloid protein production and improve long-term outcome.

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