[Treatment of primary systemic amyloidosis with the combination of bortezomib and dexamethasone].
OBJECTIVE
To evaluate the efficacy and feasibility of bortezomib plus dexamethasone (BD) in patients with primary systemic (AL) amyloidosis.


METHODS
Eleven AL amyloidosis patients, including four relapsed or progressed after previous therapies and 7 newly diagnosed were treated with BD. Ten patients had two or more organs involved. Precursor protein analysis showed that 1 was κ light chain, 9 λ light chain; 5 patients with positive immunofixation including 1 IgG κ, 3 IgG λ and 1 IgA λ. BD was administered according to standard two-week schedule.


RESULTS
Eight patients were evaluable, the median number of treatment cycles was 3 (range 1 - 6). Median follow-up duration was 6 months. At least one affected organ response was observed in six patients and median time to organ response was 2 months. Three patients progressed and two of them died. Toxicities were mainly diarrhea, thrombocytopenia, peripheral neuropathy, fatigue and herpes zoster, and 7 evaluable patients who had toxicities were adjusted dosage and 2 of them interrupted therapy. Epilepsia, paralytic ileus, acute cardiac dysfunction, and postural hypotention were occurred in 3 inevaluble patients.


CONCLUSIONS
Bortezomib plus dexamethasone is effective in AL amyloidosis. Adverse events are common, and in some patients are severe.
Department of Hematology, Nanjing General Hospital of Nanjing Military Command, PLA, Nanjing 210002, China.
Yong-Ping
YP
Zhai
Hai-Ning
HN
Liu
Ya-Ping
YP
Yu
Xiao-Gang
XG
Zhou
Ping
P
Song
Feng
F
Li
Xue-Wen
XW
Wang
D000686:Amyloidosis / Q000188:drug therapy; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D003907:Dexamethasone / Q000008:administration & dosage; D006801:Humans; D009101:Multiple Myeloma / Q000188:drug therapy
D001897:Boronic Acids; D000069286:Bortezomib; D003907:Dexamethasone