Journal
INTERNAL MEDICINE
Volume 47, Issue 20, Pages 1783-1790Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.47.0966
Keywords
flow cytometry; free light chain; plasma cell; AL amyloidosis; chemotherapy
Categories
Funding
- Intractable Disease Division
- Ministry of Health and Welfare
- Research Committee for Epochal Diagnosis
- Treatment of Amyloidosis in Japan
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Objective Primary systemic AL amyloidosis arises from immunoglobulin light chains produced by plasma cell dyscrasia. To prospectively investigate the production of M-protein and plasma cells in bone marrow before and after chemotherapy, we performed flow cytometry and analysis of serum free light chains (FLCs). Patients and Methods Fifty-nine patients with primary systemic AL amyloidosis (mean age, 59.9 +/- 8.8 years) were enrolled in this study, and of these 31 were serially studied before and after chemotherapy. Complete hematological remission was defined as normalization of the FLC kappa/lambda ratio. Results MPC-1-CD45 (p<0.05) and MPC-1(+)CD45 CD49e (p<0.005) were significantly higher, and MPC-1-CD45(+)(p<0.05), MPC-1(+)CD45(+)CD49e (p<0.0001) and MPC-1(+)CD45(+)CD49e(+) (p<0.0005) were significantly lower in the patients with AL amyloidosis than in controls. There was a significantly positive correlation between the serum predominant FLC/serum creatinine ratio and MPC-1(+)CD45CD49e (p<0.05). After chemotherapies, such as high-dose melphalan with autologous stem cell support, 20 of 31 patients with AL amyloidosis achieved complete hematological remission. There were no significant differences in any subtype of plasma cells before treatment between the remission and non-remission groups, but in the former group MPC-1(+)CD45CD49e and MPC-1-CD45(+) were significantly decreased and increased after chemotherapy compared with before, respectively. Conclusion Abnormal plasma cells in the bone marrow, particularly the MPC-1+CD45CD49e subset, may be important as a follow-up marker before and after chemotherapy in primary systemic AL amyloidosis. These cells maintain low levels as long as no relapse occurs.
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