The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients

Vittorio Perfetti, Maurizio Colli Vignarelli, Ernesto Anesi, Pietro Garini, Silvana Quaglini, Edoardo Ascari, Giampaolo Merlini

Research output: Contribution to journalArticle

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%. κ/λ ratio cut-off values of 0.2 (λ patients, p = 0.022) and 16 (λ 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.

Original languageEnglish
Pages (from-to)218-221
Number of pages4
JournalHaematologica
Volume84
Issue number3
Publication statusPublished - 1999

Fingerprint

Amyloidosis
Plasma Cells
Bone Marrow
Light
Bone Marrow Cells
Survival
Immunoglobulin Light Chains
Paraproteinemias
Multiple Myeloma
Fluorescence Microscopy
Proportional Hazards Models
Amyloid
Multivariate Analysis
Heart Failure
Clone Cells

Keywords

  • AL amyloidosis
  • Plasma cell
  • Prognosis

ASJC Scopus subject areas

  • Hematology

Cite this

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), 218-221.

The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. / Perfetti, Vittorio; Colli Vignarelli, Maurizio; Anesi, Ernesto; Garini, Pietro; Quaglini, Silvana; Ascari, Edoardo; Merlini, Giampaolo.

In: Haematologica, Vol. 84, No. 3, 1999, p. 218-221.

Research output: Contribution to journalArticle

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, vol. 84, no. 3, pp. 218-221.
Perfetti V, Colli Vignarelli M, Anesi E, Garini P, Quaglini S, Ascari E et al. The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. Haematologica. 1999;84(3):218-221.
Perfetti, Vittorio ; Colli Vignarelli, Maurizio ; Anesi, Ernesto ; Garini, Pietro ; Quaglini, Silvana ; Ascari, Edoardo ; Merlini, Giampaolo. / The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients. In: Haematologica. 1999 ; Vol. 84, No. 3. pp. 218-221.
@article{6dc9b4783607447cabfb1e73279dc215,
title = "The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients",
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{\%}. κ/λ ratio cut-off values of 0.2 (λ patients, p = 0.022) and 16 (λ 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.",
keywords = "AL amyloidosis, Plasma cell, Prognosis",
author = "Vittorio Perfetti and {Colli Vignarelli}, Maurizio and Ernesto Anesi and Pietro Garini and Silvana Quaglini and Edoardo Ascari and Giampaolo Merlini",
year = "1999",
language = "English",
volume = "84",
pages = "218--221",
journal = "Haematologica",
issn = "0390-6078",
publisher = "NLM (Medline)",
number = "3",

}

TY - JOUR

T1 - The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients

AU - Perfetti, Vittorio

AU - Colli Vignarelli, Maurizio

AU - Anesi, Ernesto

AU - Garini, Pietro

AU - Quaglini, Silvana

AU - Ascari, Edoardo

AU - Merlini, Giampaolo

PY - 1999

Y1 - 1999

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%. κ/λ ratio cut-off values of 0.2 (λ patients, p = 0.022) and 16 (λ 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.

AB - 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%. κ/λ ratio cut-off values of 0.2 (λ patients, p = 0.022) and 16 (λ 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.

KW - AL amyloidosis

KW - Plasma cell

KW - Prognosis

UR - http://www.scopus.com/inward/record.url?scp=0344172816&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0344172816&partnerID=8YFLogxK

M3 - Article

C2 - 10189385

AN - SCOPUS:0344172816

VL - 84

SP - 218

EP - 221

JO - Haematologica

JF - Haematologica

SN - 0390-6078

IS - 3

ER -