Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2013
[Curative effect observation of patients with primary systemic amyloidosis treated by the combination of bortezomib with dexamethasone and cyclophosphamide].
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Lu Jin, Wang Hui, Huang Xiao-jun
Аннотация
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.
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.