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. 2009 Aug;94(8):1094-100.
doi: 10.3324/haematol.2008.004119.

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

Ingrid I van Gameren  1 , Martin H van Rijswijk, Johan Bijzet, Edo Vellenga, Bouke P Hazenberg
Affiliations

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

Ingrid I van Gameren et al. Haematologica. 2009 Aug.

Abstract

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.

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Figures

Figure 1.
Figure 1.
Amyloidogenic free light chain (FLC) (mg/L) at start of therapy. Patients were divided into 3 groups: complete responders (CR, n=30), partial responders (PR, n=9), and non-responders (NR, n=11). Horizontal bars denote median values; ***p<0.001.
Figure 2.
Figure 2.
Response of amyloidogenic FLC (mg/L) following chemotherapy. (A) Complete responders (n=30). (B) Partial responders (n=9). (C) Non-responders (n=12). ES denotes end of study, horizontal bars denote median values; *p<0.05, **p<0.005.
Figure 3.
Figure 3.
(A) Change of amyloid grade in fat tissue at last observation in complete responders (CR), partial responders (PR), and non-responders (NR) of FLC; * p<0.05. (B) Linear regression of the change of amyloid grade in fat tissue at last observation for complete responders (r=−0.54, p<0.002).
Figure 4.
Figure 4.
(A) Kaplan-Meier curves of patients with complete response (CR) of serum free light chain. Endpoints are decreases of 1, 2, 3 and 4 grades of amyloid in fat tissue, respectively. The numbers of CR patients at start and later who had the potential to reflect 1, 2, 3, and 4 grades are shown at the bottom. (B) Kaplan-Meier curves of patients with complete response (CR), partial response (PR), and no response (NR) of serum free light chain. Endpoint is a significant decrease of 2 grades of amyloid in fat tissue. The numbers of patients at start and later who had the potential to reflect such a decrease are shown at the bottom; *p<0.05.
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Comment in

  • Current treatment of AL amyloidosis.
    Palladini G, Merlini G. Palladini G, et al. Haematologica. 2009 Aug;94(8):1044-8. doi: 10.3324/haematol.2009.008912. Haematologica. 2009. PMID: 19644136 Free PMC article.

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