[Prognostic factors in light-chain cardiac amyloidosis: a retrospective analysis]

Hiroki Okumura1, Shuku Sato1, Wataru Kamata1

  • 1Division of Hematology, Shonan Kamakura General Hospital.

Insights

Cardiac amyloidosis significantly worsens prognosis in amyloid light-chain (AL) amyloidosis patients. Identifying poor prognostic factors is crucial for treatment decisions and palliative care in AL amyloidosis.

Area of Science:

  • Cardiology
  • Hematology
  • Oncology

Background:

  • Amyloid light-chain (AL) amyloidosis is a plasma cell disorder causing amyloid deposition in organs.
  • Cardiac involvement in AL amyloidosis is a known predictor of poor survival.
  • Limited prognostic indicators exist that comprehensively assess organ involvement and patient condition.

Purpose of the Study:

  • To identify prognostic factors for AL amyloidosis.
  • To evaluate the impact of cardiac involvement on survival in AL amyloidosis patients.
  • To inform treatment strategies and palliative care decisions.

Main Methods:

  • Retrospective review of 27 AL amyloidosis patients diagnosed between 2012 and 2019.
  • Analysis of survival rates based on cardiac involvement.
  • Identification of clinical and laboratory parameters associated with poor prognosis.

Main Results:

  • The 3-year overall survival rate was 20% for patients with cardiac involvement versus 85.7% for those without (p=0.021).
  • Poor prognostic factors included low left ventricular ejection fraction, low hemoglobin, elevated NT-proBNP and BNP, high difference free light chains, advanced NYHA class, Mayo stage IV disease, and cardiac amyloidosis.
  • Four non-survivors within 6 months all had cardiac amyloidosis, Mayo stage IV disease, and insufficient chemotherapy.

Conclusions:

  • Cardiac amyloidosis is a critical factor associated with poor prognosis in AL amyloidosis.
  • Multiple factors including cardiac status, hematologic markers, and disease stage predict survival.
  • Careful treatment planning, including palliative care, is essential for patients with poor prognostic indicators in AL amyloidosis.

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