title: Successful treatment of renal light chain (AL) amyloidosis with bortezomib and dexamethasone (VD).
abstract: OBJECTIVE
  To assess the efficacy and tolerability of bortezomib with dexamethasone for patients with renal light chain (AL) amyloidosis.
  
  
  METHODS
  Twelve newly diagnosed patients with renal AL amyloidosis were treated with a combination of bortezomib (1.3mg/m(2)/d iv, d1, 4, 8, 11) and dexamethasone (20mg/d iv drip, d1-4).
  
  
  RESULTS
  Median follow-up time was 22.5 months (range, 2.1-53.6). Ten patients were evaluable. Five out of 10 (50%) patients achieved complete hematologic responses (CHR), and totally 8/10 (80%) achieved hematologic responses (HR). Median time to hematologic response was 1 month. All patients who received HR had no hematologic progression during follow-up period. Five patients (50%) had kidney responses and the other 5 patients (50%) were stable. Median time to kidney response was 3 months. No patients presented renal progression during follow-up. One patient achieved PR after 4 cycles of VD and then received autologous peripheral blood stem cell transplantation. Two out of 10 evaluable patients without hematologic response had died with median overall survival of 8.2 months. Eight of them who had HR were alive with median follow-up time of 28.5 months. Infection (6/12) and fatigue (5/12) were the most frequent side effects. Three patients developed herpes zoster and had to discontinue therapy.
  
  
  CONCLUSIONS
  VD produces rapid, deep and durable hematological responses and renal responses in the majority of patients with newly diagnosed renal AL. It is well tolerated. This treatment may be a good option as first-line treatment for renal AL amyloidosis patients.
authors.affiliation: Department of Hematology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address: hbh_st@126.com.
authors.forename: B
authors.initials: B
authors.lastname: Huang
authors.affiliation: Department of Hematology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address: 13719209240@163.com.
authors.forename: J
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Hematology, Zhongshan City People Hospital, Zhongshan 528403, China. Electronic address: doctorxu@163.com.
authors.forename: X
authors.initials: X
authors.lastname: Xu
authors.affiliation: Department of Hematology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address: zcxzd@tom.com.
authors.forename: D
authors.initials: D
authors.lastname: Zheng
authors.affiliation: Department of Hematology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address: zhouzhh2013@163.com.
authors.forename: Z
authors.initials: Z
authors.lastname: Zhou
authors.affiliation: Department of Hematology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address: 13631484622@163.com.
authors.forename: J
authors.initials: J
authors.lastname: Liu
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D000069286:Bortezomib / Q000008:administration & dosage* / Q000009:adverse effects; D003907:Dexamethasone / Q000008:administration & dosage* / Q000009:adverse effects; D018450:Disease Progression; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000009:adverse effects; D000075363:Immunoglobulin Light-chain Amyloidosis; D007674:Kidney Diseases / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D007147:Immunoglobulin Light Chains; D000069286:Bortezomib; D003907:Dexamethasone
keywords: Amylose rénale à chaînes légères; Bortezomib; Bortézomib; Dexamethasone; Dexaméthasone; Renal light chain amyloidosis