4th Palermo Conference on INNOVATIVE THERAPIES FOR LYMPHOID MALIGNANCIES
Haematologica, Vol 94, Issue 8, 1094-1100 doi:10.3324/haematol.2008.004119
Copyright © 2009 by Ferrata Storti Foundation
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Amyloidosis

Histological regression of amyloid in AL amyloidosis is exclusively seen after normalization of serum free light chain

Ingrid I. van Gameren1, Martin H. van Rijswijk1, Johan Bijzet1, Edo Vellenga2, Bouke P. Hazenberg1

1 Departments of Rheumatology and Clinical Immunology
2 Haematology, University Medical Center Groningen, University of Groningen, The Netherlands

Correspondence: Ingrid van Gameren, Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands., E-mail:iivgameren{at}xs4all.nl

Background: Histological regression of amyloid has not been studied systematically but is assessed by clinical parameters. We analyzed the change of amyloid deposition in fat tissue in patients with AL amyloidosis following chemotherapy and studied the relation with type of hematologic response.

Design and Methods: Between January 1994 and July 2007 all consecutive patients with AL amyloidosis were evaluated in whom fat tissue aspirate was obtained before and following chemotherapy. Patients were divided into three groups depending on response of serum free light chain: complete, partial or non-responders. Fat tissue was assessed using a validated semi-quantitative test (grading 0–4). A change of 2 grades of amyloid deposition in fat tissue was considered significant and used as event to construct Kaplan-Meier curves of the patients who were able to reflect such a change.

Results: One hundred and twenty consecutive patients were studied. Fifty-one patients fulfilled inclusion criteria. Thirty patients had a complete response of the amyloidogenic free light chain a median 0.5 year (range 0.3–2.9 years) following chemotherapy. Reduction of 2 grades of amyloid deposition in fat tissue was seen in 50% of these patients after 2.4 years and in 80% after 3.2 years. In contrast to complete responders, none of the patients with partial (n=9) and non-response (n=12) showed reduction of 2 grades (p=0.02) with median follow-up of fat tissue analysis of 1.3 and 0.8 years, respectively.

Conclusions: This study in a selected group of patients with AL amyloidosis shows significant histological regression of amyloid deposition in fat tissue exclusively after normalization of serum free light chain.

Key words: AL amyloidosis, free light chain response, regression of amyloid.


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G. Palladini and G. Merlini
Current treatment of AL amyloidosis
Haematologica, August 1, 2009; 94(8): 1044 - 1048.
[Full Text] [PDF]