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Related Experiment Videos

Clotting alterations in primary systemic amyloidosis.

G Gamba1, N Montani, E Anesi

  • 1Istituto di Medicina Interna e Oncologia Medica, IRCCS Policlinico San Matteo, 27100 Pavia, Italy. gamba@smatteo.pv.it

Haematologica
|March 7, 2000
PubMed
Summary

Patients with immunoglobulin light chain amyloidosis often have bleeding issues due to clotting abnormalities. Prolonged thrombin and reptilase times are common, indicating defective fibrin formation in this condition.

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Area of Science:

  • Hematology
  • Oncology
  • Internal Medicine

Background:

  • Immunoglobulin light chain amyloidosis (AL) is associated with bleeding complications.
  • Pathogenesis involves amyloid deposits, factor interactions, and plasma inhibitors affecting hemostasis.
  • Clotting test alterations are frequently observed in AL patients.

Purpose of the Study:

  • To determine the prevalence of clotting abnormalities in AL patients.
  • To correlate these abnormalities with clinical bleeding manifestations.

Main Methods:

  • Study included 36 consecutive AL patients with biopsy-proven amyloidosis and monoclonal gammopathy.
  • Evaluated activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVVT), fibrinogen, factor X, and alpha-2 antiplasmin.

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Main Results:

  • Hemorrhagic manifestations ranged from mild to severe.
  • Defective fibrinogen conversion was most frequent, shown by prolonged TT (85%) and RT (90%).
  • Low factor X activity occurred in 25% of samples; PT and aPTT were prolonged in some patients.

Conclusions:

  • Prolonged TT and RT are characteristic of AL amyloidosis, linked to impaired fibrin formation.
  • RVVT prolongation may involve an inhibitor affecting fibrin formation, not solely factor X levels.
  • Hemostatic variability in AL mirrors the disease's heterogeneous clinical presentation.