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Primary amyloidosis and myelomatosis associated with excessive fibrinolytic acitivty
The British Journal of Dermatology
|May 1, 1976
Abstract:
A case of primary amyloidosis is described in association with Bence-Jones myeloma and increased fibrinolytic activity. Epsilon aminocaproic acid reversed the fibrinolytic activity and clinical improvement was maintained with melphalan and prednisolone.
Insights
Primary amyloidosis associated with Bence-Jones myeloma showed increased fibrinolytic activity. Treatment with epsilon aminocaproic acid reversed this activity, leading to clinical improvement maintained by melphalan and prednisolone.
Area of Science:
- Hematology
- Oncology
- Biochemistry
Background:
- Primary amyloidosis is a rare plasma cell disorder.
- Bence-Jones myeloma is characterized by the production of monoclonal immunoglobulin light chains.
- Increased fibrinolytic activity can complicate amyloidosis, potentially leading to bleeding complications.
Observation:
- A patient presented with primary amyloidosis and co-existing Bence-Jones myeloma.
- The patient exhibited significantly elevated levels of fibrinolytic activity.
- This heightened activity was linked to the underlying plasma cell dyscrasia.
Findings:
- Epsilon aminocaproic acid effectively inhibited the increased fibrinolytic activity.
- The administration of melphalan and prednisolone resulted in sustained clinical improvement.
- The combination therapy addressed both the amyloidosis and the myeloma components.
Implications:
- This case highlights a potential therapeutic strategy for managing fibrinolytic complications in amyloidosis.
- Early recognition and intervention of hyperfibrinolysis are crucial for patient outcomes.
- Targeted treatment of the underlying plasma cell disorder is essential for long-term remission.