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The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients.
Haematologica. 1999 Mar; 84(3):218-21.H

Abstract

BACKGROUND AND OBJECTIVE

Primary amyloidosis is a lethal form of plasma cell (PC) dyscrasia characterized by deposits of monoclonal immunoglobulin light chains that cause organ dysfunction. In contrast to multiple myeloma, the amyloid clone is typically indolent and of small size, and marrow PC clonality is not always apparent. This is generally investigated by analyzing the light chain isotype ratio in bone marrow PC. We investigated whether the degree of PC infiltration (PC%) and clonality (PC isotype ratio) affected survival in 56 consecutive patients with primary amyloidosis.

DESIGN AND METHODS

PC% was determined by morphologic examination. Immunofluorescence microscopy was used to determine the PC light chain isotype ratio. Statistical analysis was carried out using Cox regression models.

RESULTS

The degrees of PC clonality and infiltration were inversely correlated with survival (PC isotype ratio, p = 0.001; PC%, p = 0.008). The two variables were weakly correlated (p = 0.02; r = 0.3). Bone marrow PC isotype ratio demonstrated a powerful independent prognostic value at multivariate analysis when analyzed together with congestive heart failure (the major known negative prognostic factor) and PC%. k/l ratio cut-off values of 0.2 (l patients, p = 0.022) and 16 (k patients, p = 0.03) discriminated two groups with a similar number of patients and significantly different survivals.

INTERPRETATION AND CONCLUSIONS

PC clonality and marrow infiltration are important parameters that influence prognosis, presumably because they reflect the amount of pathogenic light chain synthesis.

Authors+Show Affiliations

Biotechnology and Organ Transplantation Research Laboratories, University of Pavia, IRCCS Policlinico S. Matteo, Pavia, Italy.No affiliation info availableNo 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

10189385

Citation

Perfetti, V, et al. "The Degrees of Plasma Cell Clonality and Marrow Infiltration Adversely Influence the Prognosis of AL Amyloidosis Patients." Haematologica, vol. 84, no. 3, 1999, pp. 218-21.
Perfetti V, Colli Vignarelli M, Anesi E, et al. The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. Haematologica. 1999;84(3):218-21.
Perfetti, V., Colli Vignarelli, M., Anesi, E., Garini, P., Quaglini, S., Ascari, E., & Merlini, G. (1999). The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. Haematologica, 84(3), 218-21.
Perfetti V, et al. The Degrees of Plasma Cell Clonality and Marrow Infiltration Adversely Influence the Prognosis of AL Amyloidosis Patients. Haematologica. 1999;84(3):218-21. PubMed PMID: 10189385.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. AU - Perfetti,V, AU - Colli Vignarelli,M, AU - Anesi,E, AU - Garini,P, AU - Quaglini,S, AU - Ascari,E, AU - Merlini,G, PY - 1999/4/6/pubmed PY - 1999/4/6/medline PY - 1999/4/6/entrez SP - 218 EP - 21 JF - Haematologica JO - Haematologica VL - 84 IS - 3 N2 - BACKGROUND AND OBJECTIVE: Primary amyloidosis is a lethal form of plasma cell (PC) dyscrasia characterized by deposits of monoclonal immunoglobulin light chains that cause organ dysfunction. In contrast to multiple myeloma, the amyloid clone is typically indolent and of small size, and marrow PC clonality is not always apparent. This is generally investigated by analyzing the light chain isotype ratio in bone marrow PC. We investigated whether the degree of PC infiltration (PC%) and clonality (PC isotype ratio) affected survival in 56 consecutive patients with primary amyloidosis. DESIGN AND METHODS: PC% was determined by morphologic examination. Immunofluorescence microscopy was used to determine the PC light chain isotype ratio. Statistical analysis was carried out using Cox regression models. RESULTS: The degrees of PC clonality and infiltration were inversely correlated with survival (PC isotype ratio, p = 0.001; PC%, p = 0.008). The two variables were weakly correlated (p = 0.02; r = 0.3). Bone marrow PC isotype ratio demonstrated a powerful independent prognostic value at multivariate analysis when analyzed together with congestive heart failure (the major known negative prognostic factor) and PC%. k/l ratio cut-off values of 0.2 (l patients, p = 0.022) and 16 (k patients, p = 0.03) discriminated two groups with a similar number of patients and significantly different survivals. INTERPRETATION AND CONCLUSIONS: PC clonality and marrow infiltration are important parameters that influence prognosis, presumably because they reflect the amount of pathogenic light chain synthesis. SN - 0390-6078 UR - https://www.unboundmedicine.com/medline/citation/10189385/The_degrees_of_plasma_cell_clonality_and_marrow_infiltration_adversely_influence_the_prognosis_of_AL_amyloidosis_patients_ L2 - http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=10189385 DB - PRIME DP - Unbound Medicine ER -