Changes in serum‐free light chain rather than intact monoclonal immunoglobulin levels predicts outcome following therapy in primary amyloidosis
Open Access
- 2 December 2010
- journal article
- research article
- Published by Wiley in American Journal of Hematology
- Vol. 86 (3), 251-255
- https://doi.org/10.1002/ajh.21948
Abstract
Current response criteria for light‐chain amyloidosis (AL) relegate FLC response to a subsidiary status relative to serum M‐protein response. Given that light chains form the substrate for amyloid fibril formation, we hypothesized that changes in FLC might better predict outcome compared to changes in intact immunoglobulin levels. Two patient cohorts were studied, 347 patients who underwent an autologous stem‐cell transplant (SCT) and 96 patients treated with melphalan/dexamethasone. We identified the lowest value following therapy for intact serum M‐protein and the difference between involved and uninvolved FLC (FLC‐diff). We first examined the relative contribution of M‐protein and FLC‐diff on the overall survival (OS), and found that FLC reduction, rather than M‐protein reduction, significantly impacted OS. The median OS was not reached among those with a 50% decrease in FLC‐diff compared to 20 months for the remainder. On regression analysis, a 90% reduction in FLC‐diff following SCT best predicted being alive at 3 or 5 years. The median OS among those with a 90% decrease was not reached compared to 37.4 months for the rest P < 0.001. The current study supports the notion that FLC response is a more useful measure of hematological response than M‐protein response. It also highlights the importance of achieving at least a 90% reduction in the FLC‐diff to improve the outcome of patients with light‐chain AL. Am. J. Hematol. 2011.Keywords
This publication has 34 references indexed in Scilit:
- Bortezomib With or Without Dexamethasone in Primary Systemic (Light Chain) AmyloidosisJournal of Clinical Oncology, 2010
- Histological regression of amyloid in AL amyloidosis is exclusively seen after normalization of serum free light chainHaematologica, 2009
- Evaluation of the serum-free light chain test in untreated patients with AL amyloidosisHaematologica, 2008
- Long-term outcome of patients with AL amyloidosis treated with high-dose melphalan and stem-cell transplantationBlood, 2007
- Successful treatment of AL amyloidosis with high-dose melphalan and autologous stem cell transplantation in patients over age 65Blood, 2006
- International uniform response criteria for multiple myelomaLeukemia, 2006
- AmyloidosisBest Practice & Research Clinical Haematology, 2005
- Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and AmyloidosisAmerican Journal of Hematology, 2005
- Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantationPublished by American Society of Hematology ,2004
- CRITERIA FOR EVALUATING DISEASE RESPONSE AND PROGRESSION IN PATIENTS WITH MULTIPLE MYELOMA TREATED BY HIGH‐DOSE THERAPY AND HAEMOPOIETIC STEM CELL TRANSPLANTATIONBritish Journal of Haematology, 1998