[Curative effect observation of patients with primary systemic amyloidosis treated by the combination of bortezomib with dexamethasone and cyclophosphamide].

OBJECTIVE

To analyze the efficacy and side effects of the combination regimen containing bortezomib, cyclophosphamide and dexamethasone(BCD) in the treatment of primary systemic amyloidosis (PSA).

METHODS

The clinical manifestation, efficacy and side effects of 22 PSA patients after treatment were retrospectively analyzed.

RESULTS

Of the 22 PSA patients, 9 were newly diagnosed cases and 13 relapsed cases, 20(90.9%) had symptomatic cardiac involvement and 20 (90.9%) had 2 or more organs involved. The median free light chain were 140 mg/L. There were no specific abnormality was found by G-banding and FISH. RQ-PCR-MAGE C1/CT7/ABL had high positive incidence in amyloidosis and can be observed ahead of the flow cytometry and morphology examination. Hematological response occurred in 88.9% in newly diagnosed patients, with 55.6% organ response, 2 patients occured sudden death during BCD interval. For relapsed patients, the hematological response was 46.2%, with 30.7% organ response. Ten of the 22 PSA patients developed peripheral neurophathy, 1 liver dysfunction, 1 severe infection after intra-tracheal stent.

CONCLUSIONS

BCD produces rapid and high hematological responses in the PSA patients especially in newly diagnosed patients. It is well tolerated with few side effects. MAGE C1/CT7 might be helpful in the early detection of the clonal plasma cell.