Clotting alterations in primary systemic amyloidosis

Gabriella Gamba, Nadia Montani, Ernesto Anesi, Giovanni Palladini, Michela Capezzera, Elena Soldavini, Giampaolo Merlini

Research output: Contribution to journalArticle

Abstract

Background and Objectives. The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL. Design and Methods. Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year. The following clotting tests were considered in the study: activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVTT), fibrinogen, factor X and α-2 antiplasmin. Results. Hemorrhagic manifestations were mild to moderate in nine patients, but severe and untractable in one. The most frequent clotting anomaly was defective fibrinogen conversion to fibrin, as demonstrated by prolongation of both TT (85% of cases) and RT (90% of cases). Low levels of factor X activity were observed in about 1 out of 4 samples, while fibrinogen and alpha2 antiplasmin levels were distributed over a wide range of values. PT was prolonged in 8 and aPTT in 25 patients. The search for lupus anticoagulant was negative in samples showing a prolongation of aPTT and/or RVVT. Interpretation and Conclusions. The prolongation of TT and RT is not dependent on either the presence of a heparin-like substance in the plasma or on fibrinogen levels; furthermore, the prolongation of RVVT is not related to factor X level. The hypothesized presence in the plasma of an inhibitor of fibrin formation could also affect factor X activation by Russell viper venom. The prolongation of TT and RT represents a peculiar feature of amyloidosis. The variability in the behavior of the other clotting times and hemostatic factors studied is mirrored in the heterogeneity of the clinical features observed in this disease. (C) 2000, Ferrata Storti Foundation.

Original languageEnglish
Pages (from-to)289-292
Number of pages4
JournalHaematologica
Volume85
Issue number3
Publication statusPublished - Mar 2000

Fingerprint

Thrombin Time
Factor X
Amyloidosis
Fibrinogen
Partial Thromboplastin Time
Prothrombin Time
Fibrin
Antifibrinolytic Agents
Russell's Viper
Viper Venoms
Immunoglobulin Light Chains
Lupus Coagulation Inhibitor
Paraproteinemias
Blood Coagulation Factors
Primary amyloidosis
Amyloid Plaques
Hemostatics
Amyloid
Heparin
Hemorrhage

Keywords

  • Amyloidosis
  • Bleeding tendency
  • Clotting tests
  • Hemostasis anomalies

ASJC Scopus subject areas

  • Hematology

Cite this

Gamba, G., Montani, N., Anesi, E., Palladini, G., Capezzera, M., Soldavini, E., & Merlini, G. (2000). Clotting alterations in primary systemic amyloidosis. Haematologica, 85(3), 289-292.

Clotting alterations in primary systemic amyloidosis. / Gamba, Gabriella; Montani, Nadia; Anesi, Ernesto; Palladini, Giovanni; Capezzera, Michela; Soldavini, Elena; Merlini, Giampaolo.

In: Haematologica, Vol. 85, No. 3, 03.2000, p. 289-292.

Research output: Contribution to journalArticle

Gamba, G, Montani, N, Anesi, E, Palladini, G, Capezzera, M, Soldavini, E & Merlini, G 2000, 'Clotting alterations in primary systemic amyloidosis', Haematologica, vol. 85, no. 3, pp. 289-292.
Gamba G, Montani N, Anesi E, Palladini G, Capezzera M, Soldavini E et al. Clotting alterations in primary systemic amyloidosis. Haematologica. 2000 Mar;85(3):289-292.
Gamba, Gabriella ; Montani, Nadia ; Anesi, Ernesto ; Palladini, Giovanni ; Capezzera, Michela ; Soldavini, Elena ; Merlini, Giampaolo. / Clotting alterations in primary systemic amyloidosis. In: Haematologica. 2000 ; Vol. 85, No. 3. pp. 289-292.
@article{e61608008e5c418d96c2cab32b3728c9,
title = "Clotting alterations in primary systemic amyloidosis",
abstract = "Background and Objectives. The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL. Design and Methods. Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year. The following clotting tests were considered in the study: activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVTT), fibrinogen, factor X and α-2 antiplasmin. Results. Hemorrhagic manifestations were mild to moderate in nine patients, but severe and untractable in one. The most frequent clotting anomaly was defective fibrinogen conversion to fibrin, as demonstrated by prolongation of both TT (85{\%} of cases) and RT (90{\%} of cases). Low levels of factor X activity were observed in about 1 out of 4 samples, while fibrinogen and alpha2 antiplasmin levels were distributed over a wide range of values. PT was prolonged in 8 and aPTT in 25 patients. The search for lupus anticoagulant was negative in samples showing a prolongation of aPTT and/or RVVT. Interpretation and Conclusions. The prolongation of TT and RT is not dependent on either the presence of a heparin-like substance in the plasma or on fibrinogen levels; furthermore, the prolongation of RVVT is not related to factor X level. The hypothesized presence in the plasma of an inhibitor of fibrin formation could also affect factor X activation by Russell viper venom. The prolongation of TT and RT represents a peculiar feature of amyloidosis. The variability in the behavior of the other clotting times and hemostatic factors studied is mirrored in the heterogeneity of the clinical features observed in this disease. (C) 2000, Ferrata Storti Foundation.",
keywords = "Amyloidosis, Bleeding tendency, Clotting tests, Hemostasis anomalies",
author = "Gabriella Gamba and Nadia Montani and Ernesto Anesi and Giovanni Palladini and Michela Capezzera and Elena Soldavini and Giampaolo Merlini",
year = "2000",
month = "3",
language = "English",
volume = "85",
pages = "289--292",
journal = "Haematologica",
issn = "0390-6078",
publisher = "NLM (Medline)",
number = "3",

}

TY - JOUR

T1 - Clotting alterations in primary systemic amyloidosis

AU - Gamba, Gabriella

AU - Montani, Nadia

AU - Anesi, Ernesto

AU - Palladini, Giovanni

AU - Capezzera, Michela

AU - Soldavini, Elena

AU - Merlini, Giampaolo

PY - 2000/3

Y1 - 2000/3

N2 - Background and Objectives. The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL. Design and Methods. Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year. The following clotting tests were considered in the study: activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVTT), fibrinogen, factor X and α-2 antiplasmin. Results. Hemorrhagic manifestations were mild to moderate in nine patients, but severe and untractable in one. The most frequent clotting anomaly was defective fibrinogen conversion to fibrin, as demonstrated by prolongation of both TT (85% of cases) and RT (90% of cases). Low levels of factor X activity were observed in about 1 out of 4 samples, while fibrinogen and alpha2 antiplasmin levels were distributed over a wide range of values. PT was prolonged in 8 and aPTT in 25 patients. The search for lupus anticoagulant was negative in samples showing a prolongation of aPTT and/or RVVT. Interpretation and Conclusions. The prolongation of TT and RT is not dependent on either the presence of a heparin-like substance in the plasma or on fibrinogen levels; furthermore, the prolongation of RVVT is not related to factor X level. The hypothesized presence in the plasma of an inhibitor of fibrin formation could also affect factor X activation by Russell viper venom. The prolongation of TT and RT represents a peculiar feature of amyloidosis. The variability in the behavior of the other clotting times and hemostatic factors studied is mirrored in the heterogeneity of the clinical features observed in this disease. (C) 2000, Ferrata Storti Foundation.

AB - Background and Objectives. The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL. Design and Methods. Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year. The following clotting tests were considered in the study: activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVTT), fibrinogen, factor X and α-2 antiplasmin. Results. Hemorrhagic manifestations were mild to moderate in nine patients, but severe and untractable in one. The most frequent clotting anomaly was defective fibrinogen conversion to fibrin, as demonstrated by prolongation of both TT (85% of cases) and RT (90% of cases). Low levels of factor X activity were observed in about 1 out of 4 samples, while fibrinogen and alpha2 antiplasmin levels were distributed over a wide range of values. PT was prolonged in 8 and aPTT in 25 patients. The search for lupus anticoagulant was negative in samples showing a prolongation of aPTT and/or RVVT. Interpretation and Conclusions. The prolongation of TT and RT is not dependent on either the presence of a heparin-like substance in the plasma or on fibrinogen levels; furthermore, the prolongation of RVVT is not related to factor X level. The hypothesized presence in the plasma of an inhibitor of fibrin formation could also affect factor X activation by Russell viper venom. The prolongation of TT and RT represents a peculiar feature of amyloidosis. The variability in the behavior of the other clotting times and hemostatic factors studied is mirrored in the heterogeneity of the clinical features observed in this disease. (C) 2000, Ferrata Storti Foundation.

KW - Amyloidosis

KW - Bleeding tendency

KW - Clotting tests

KW - Hemostasis anomalies

UR - http://www.scopus.com/inward/record.url?scp=0034038320&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034038320&partnerID=8YFLogxK

M3 - Article

C2 - 10702818

AN - SCOPUS:0034038320

VL - 85

SP - 289

EP - 292

JO - Haematologica

JF - Haematologica

SN - 0390-6078

IS - 3

ER -