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Primary systemic amyloidosis with delayed progression to multiple myeloma
S V Rajkumar1, M A Gertz, R A Kyle
1Division of Hematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Cancer
|April 29, 1998
Summary
Primary systemic amyloidosis (AL) can progress to multiple myeloma. This case series highlights six patients who developed multiple myeloma months after an initial AL diagnosis, impacting survival.
Area of Science:
- Hematology
- Oncology
- Internal Medicine
Background:
- Primary systemic amyloidosis (AL) and multiple myeloma are related plasma cell disorders.
- While myeloma can coexist with AL, AL progressing to myeloma is uncommon.
- This study investigates cases of AL evolving into multiple myeloma.
Purpose of the Study:
- To describe a series of patients with primary systemic amyloidosis who later developed multiple myeloma.
- To analyze the clinical characteristics and outcomes of AL progressing to multiple myeloma.
Main Methods:
- Computerized search of Mayo Clinic medical records for patients diagnosed with AL between 1960 and 1994.
- Review of 6 patients with biopsy-proven AL who developed multiple myeloma at least 6 months after AL diagnosis.
- Analysis of clinical manifestations, diagnostic criteria for myeloma, treatments, and survival.
Main Results:
- None of the 6 patients had myeloma at the time of AL diagnosis; diagnoses occurred 10-81 months later.
- Presenting AL manifestations included peripheral neuropathy, cutaneous, renal, and amyloid arthropathy.
- Multiple myeloma was diagnosed based on osteolytic lesions or bone marrow plasma cell infiltration; median survival post-myeloma diagnosis was 20 months.
Conclusions:
- Primary systemic amyloidosis can occasionally progress to overt multiple myeloma.
- This progression is more likely in patients without significant cardiac or hepatic involvement who survive long enough.
- The findings underscore the importance of monitoring AL patients for potential myeloma development.