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Published on: November 30, 2018
Coagulation Abnormalities in Light Chain Amyloidosis
Nadine Abdallah1, Eli Muchtar1, Angela Dispenzieri1
1Division of Hematology, Mayo Clinic, Rochester, MN.
Insights
Coagulation abnormalities are common in systemic light chain (AL) amyloidosis, linked to advanced disease and poorer survival outcomes. Further research is needed to explore treatment response correlations.
Area of Science:
- Hematology
- Oncology
- Internal Medicine
Background:
- Systemic light chain (AL) amyloidosis is a plasma cell disorder characterized by amyloid deposition.
- Coagulation abnormalities are increasingly recognized in AL amyloidosis, but their clinical significance requires further elucidation.
Purpose of the Study:
- To determine the prevalence of coagulation abnormalities in AL amyloidosis patients.
- To investigate the association of these abnormalities with disease characteristics, progression, and survival.
Main Methods:
- Retrospective analysis of 411 AL amyloidosis patients diagnosed between 2006 and 2015.
- Assessment of coagulation parameters at diagnosis and correlation with clinical data and outcomes.
Main Results:
- 19% had prolonged prothrombin time (PT), and 43% had factor X (FX) deficiency.
- FX deficiency correlated with advanced disease markers (higher Mayo stage, multi-organ involvement, liver/cardiac involvement, bone marrow plasma cells >10%).
- Abnormal PT, factor V, factor VII (FVII), FX, and factor XII were associated with increased risk of progression or death; prolonged PT, FVII, and FX deficiency independently predicted mortality.
Conclusions:
- Coagulation abnormalities are prevalent in AL amyloidosis and indicate advanced disease and worse prognosis.
- Abnormalities in PT, FVII, and FX are independent predictors of mortality.
- The relationship between treatment response and coagulation parameter changes requires further investigation in larger cohorts.
Objective:
To assess the prevalence of coagulation abnormalities in patients with systemic light chain (AL) amyloidosis and their association with disease-related characteristics, disease progression, and survival.
Patients And Methods:
This is a retrospective study of patients with AL amyloidosis seen at Mayo Clinic, Rochester, Minnesota, from January 1, 2006, to December 31, 2015. We studied the association between abnormal coagulation parameters and baseline characteristics and their association with survival outcomes.
Results:
The study included 411 patients. Abnormalities at diagnosis included prolonged clotting times and coagulation factor deficiencies; prolonged prothrombin time (PT) and factor X (FX) deficiency were found in 19% (73 of 390) and 43% (177 of 411) of patients, respectively. The FX deficiency was associated with higher Mayo stage, involvement of more than 1 organ, liver and cardiac involvement, and greater than 10% bone marrow plasma cells. On univariate analysis, the risk for disease progression or death was higher in patients with abnormal values for PT and factor V, factor VII (FVII), FX, and factor XII compared with those with normal values. Prolonged PT and FVII and FX deficiencies were independent predictors of death after adjusting for Mayo stage and more than 1 organ involvement. Only 106 patients had repeat testing after treatment; no clear relationship was found between treatment response and changes in coagulation parameters.
Conclusion:
Coagulation abnormalities occur in a significant proportion of patients with AL amyloidosis and are associated with advanced disease and inferior outcomes. Larger studies are needed to establish whether a relationship exists between treatment response and improvement in individual parameters.
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