image

28.9. Hevylite in AL amyloidosis

Chapter 28

The recent availability of assays that quantitatively measure immunoglobulin heavy/light chain (Hevylite®, HLC) pairs (Chapter 9) may provide an extra test for the diagnosis, prognosis and monitoring of AL amyloidosis [635][636].

Questions

  1. What is the frequency of an abnormal κ/λ sFLC ratio in AL amyloidosis?
  2. How frequently should sFLCs be assessed in patients undergoing treatment for AL amyloidosis?
  3. What are the major prognostic factors for AL amyloidosis outcome?

Answers

  1. An abnormal κ/λ sFLC ratio is found in 75 - 98% of patients (Section 28.2).
  2. sFLCs are usually assessed prior to every cycle of chemotherapy. British guidelines recommend monitoring response to treatment with sFLCs after each cycle of chemotherapy and every 1 - 3 months thereafter, with the aim of switching to an alternative regimen as soon as the current one is proving ineffectual, which may be assessed after three cycles of therapy or earlier in cardiac patients (Section 28.6.3)
  3. Cardiac involvement and response to therapy (Sections 28.4 and 28.7).
VIEW ANSWERS

References

    • 1. Wechalekar AD, Young P, Wassef N, Gillmore JD, Gibbs SDJ, Pinney JH et al. Normal heavy/light chain (HLC) and free light chain (FLC) ratios are associated with prolonged survival in patients with systemic AL amyloidosis. Presented at XIII International Symposium on Amyloidosis 2012;PB30a
    • 2. Sachchithanantham S, Berlanga O, Harding S, Wassef N, Mahmood S, Sayeed R et al. Heavy/light chain-pair suppression as a novel marker of poor outcomes in systemic AL amyloidosis. Presented at XIV International Symposium on Amyloidosis 2014;PB-34a