Coagulation Abnormalities in Light Chain Amyloidosis

Nadine Abdallah1, Eli Muchtar1, Angela Dispenzieri1

  • 1Division of Hematology, Mayo Clinic, Rochester, MN.

Mayo Clinic Proceedings
|February 7, 2021
PubMed

Insights

Coagulation abnormalities are common in systemic light chain (AL) amyloidosis, linked to advanced disease and poorer survival outcomes. Further research is needed to explore treatment response correlations.

Area of Science:

  • Hematology
  • Oncology
  • Internal Medicine

Background:

  • Systemic light chain (AL) amyloidosis is a plasma cell disorder characterized by amyloid deposition.
  • Coagulation abnormalities are increasingly recognized in AL amyloidosis, but their clinical significance requires further elucidation.

Purpose of the Study:

  • To determine the prevalence of coagulation abnormalities in AL amyloidosis patients.
  • To investigate the association of these abnormalities with disease characteristics, progression, and survival.

Main Methods:

  • Retrospective analysis of 411 AL amyloidosis patients diagnosed between 2006 and 2015.
  • Assessment of coagulation parameters at diagnosis and correlation with clinical data and outcomes.

Main Results:

  • 19% had prolonged prothrombin time (PT), and 43% had factor X (FX) deficiency.
  • FX deficiency correlated with advanced disease markers (higher Mayo stage, multi-organ involvement, liver/cardiac involvement, bone marrow plasma cells >10%).
  • Abnormal PT, factor V, factor VII (FVII), FX, and factor XII were associated with increased risk of progression or death; prolonged PT, FVII, and FX deficiency independently predicted mortality.

Conclusions:

  • Coagulation abnormalities are prevalent in AL amyloidosis and indicate advanced disease and worse prognosis.
  • Abnormalities in PT, FVII, and FX are independent predictors of mortality.
  • The relationship between treatment response and coagulation parameter changes requires further investigation in larger cohorts.
Abstract

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