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Diagnostic approach to and follow-up of difficult cases of AL amyloidosis.
Haematologica. 1995 Sep-Oct; 80(5):409-15.H

Abstract

BACKGROUND

Routine electrophoretic analysis fails to detect a monoclonal component (MC) in a considerable portion of AL amyloidosis patients. We investigated whether the combination of immunofixation (IF) on agarose gel electrophoresis and bone marrow plasma cell (BMPC) light chain kappa/lambda ratio analysis could contribute to diagnosis in these cases. The possible use of the BMPC kappa/lambda ratio in monitoring the clone was also investigated.

METHODS

We performed BMPC kappa/lambda ratio analysis and IF of serum and urine in 16 selected patients with no detectable MC at routine analysis, despite clinical features suggestive of primary amyloidosis. An anti-idiotypic monoclonal antibody specific for the amyloidogenic immunoglobulin and the BMPC kappa/lambda ratio were used to monitor the clone in a patient who underwent autologous peripheral blood stem cell transplantation.

RESULTS

Abnormal kappa/lambda ratios were found in 14 (sensitivity 87.5%), and a MC in 12 (sensitivity 75%). Combination of the two analyses confirmed diagnosis in all cases. In one patient changes in the size of the clone, monitored on serial bone marrow aspirates by an anti-idiotypic antibody, paralleled variations of the kappa/lambda ratio.

CONCLUSIONS

This study demonstrates that the combined use of IF and the BMPC kappa/lambda ratio is extremely powerful in AL amyloidosis. In addition, the BMPC kappa/lambda ratio should be considered for monitoring the amyloidogenic clone when serum or urine MC is not quantifiable.

Authors+Show Affiliations

Research Laboratory of Biotechnology, University of Pavia, Italy.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

8566880

Citation

Perfetti, V, et al. "Diagnostic Approach to and Follow-up of Difficult Cases of AL Amyloidosis." Haematologica, vol. 80, no. 5, 1995, pp. 409-15.
Perfetti V, Garini P, Vignarelli MC, et al. Diagnostic approach to and follow-up of difficult cases of AL amyloidosis. Haematologica. 1995;80(5):409-15.
Perfetti, V., Garini, P., Vignarelli, M. C., Marinone, M. G., Zorzoli, I., & Merlini, G. (1995). Diagnostic approach to and follow-up of difficult cases of AL amyloidosis. Haematologica, 80(5), 409-15.
Perfetti V, et al. Diagnostic Approach to and Follow-up of Difficult Cases of AL Amyloidosis. Haematologica. 1995 Sep-Oct;80(5):409-15. PubMed PMID: 8566880.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Diagnostic approach to and follow-up of difficult cases of AL amyloidosis. AU - Perfetti,V, AU - Garini,P, AU - Vignarelli,M C, AU - Marinone,M G, AU - Zorzoli,I, AU - Merlini,G, PY - 1995/9/1/pubmed PY - 1995/9/1/medline PY - 1995/9/1/entrez SP - 409 EP - 15 JF - Haematologica JO - Haematologica VL - 80 IS - 5 N2 - BACKGROUND: Routine electrophoretic analysis fails to detect a monoclonal component (MC) in a considerable portion of AL amyloidosis patients. We investigated whether the combination of immunofixation (IF) on agarose gel electrophoresis and bone marrow plasma cell (BMPC) light chain kappa/lambda ratio analysis could contribute to diagnosis in these cases. The possible use of the BMPC kappa/lambda ratio in monitoring the clone was also investigated. METHODS: We performed BMPC kappa/lambda ratio analysis and IF of serum and urine in 16 selected patients with no detectable MC at routine analysis, despite clinical features suggestive of primary amyloidosis. An anti-idiotypic monoclonal antibody specific for the amyloidogenic immunoglobulin and the BMPC kappa/lambda ratio were used to monitor the clone in a patient who underwent autologous peripheral blood stem cell transplantation. RESULTS: Abnormal kappa/lambda ratios were found in 14 (sensitivity 87.5%), and a MC in 12 (sensitivity 75%). Combination of the two analyses confirmed diagnosis in all cases. In one patient changes in the size of the clone, monitored on serial bone marrow aspirates by an anti-idiotypic antibody, paralleled variations of the kappa/lambda ratio. CONCLUSIONS: This study demonstrates that the combined use of IF and the BMPC kappa/lambda ratio is extremely powerful in AL amyloidosis. In addition, the BMPC kappa/lambda ratio should be considered for monitoring the amyloidogenic clone when serum or urine MC is not quantifiable. SN - 0390-6078 UR - https://www.unboundmedicine.com/medline/citation/8566880/Diagnostic_approach_to_and_follow_up_of_difficult_cases_of_AL_amyloidosis_ L2 - http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=8566880 DB - PRIME DP - Unbound Medicine ER -