title: Retrospective case series of three patients with plasma cell leukemia treated with bortezomib-based regimens.
abstract: BACKGROUND
  Data from the literature have suggested that bortezomib is the only effective agent in the treatment of plasma cell leukemia (PCL), a type of plasma cell dyscrasia characterized by poor prognosis despite conventional chemotherapy including autologous and allogeneic transplantation.
  
  
  OBJECTIVE
  This case series examined the antineoplastic activity of the bortezomib-based regimens in a small cohort of patients with PCL.
  
  
  METHODS
  We describe a retrospective review of the 3 cases of PCL diagnosed at Antonio Perrino Hospital, Brindisi, Italy, between July 2004 and October 2006 (2 women and 1 man, all white, ages 71, 64, and 42 years; 2 with primary PCL and 1 with secondary PCL). These patients were treated with bortezomib variously combined with other drugs outside of clinical trials. Patients 1 and 2 received bortezomib-based regimens (bortezomib 1.3 mg/m2 i.v. once daily on days 1, 4, 8, and 11; dexamethasone 20 mg i.v. once daily on days 1-4 and 8-11; oral cyclophosphamide 50 mg once daily on days 1-21, every 28 days) after 2 previous chemotherapeutic treatments. Patient 3 received a bortezomib-based regimen (bortezomib 1.3 mg/m2 i.v. once daily on days 1, 4, 8, and 11; doxorubicin 9 mg/m2 i.v. once daily on days 1-4; and dexamethasone 40 mg i.v. once daily on days 1-4, 8-11, and 15-18 during cycle 1 and days 1-4 during subsequent cycles) after one previous chemotherapeutic regimen. In all 3 patients, circulating plasma cells persisted. Patients 1 and 2 were not considered candidates for autologous peripheral blood stem cell transplantation (PBSCT) because of their nonresponse to the bortezomib-based regimens and severe deterioration of their clinical conditions (kidney and liver failure) due to disease progression. The overall survivals after administration of the bortezomib- based regimens were 4 months in patient 1 and 1 month in patient 2. After further treatment according to the modified protocol for patients with acute lymphatic leukemia (cyclophosphamide 800 mg/m2 i.v. on day 1 and 200 mg/m2 i.v. on days 2-5; vincristine 1.5 mg/m2 i.v. once daily on days 1, 8, and 15; doxorubicin 40 mg/m2 i.v. on day 1; methotrexate 1200 mg/m2/h i.v. + 240 mg/m2/h i.v. for 23 hours [modified CODOX-M protocol]) due to the unavailability of a human leukocyte antigen-identical donor, patient 3 received high-dose (200 mg/m2) melphalan with autologous PBSCT, obtaining partial remission lasting 9 months. The patient died 5 months later because of disease progression.
  
  
  CONCLUSIONS
  These 3 patients with primary or secondary PCL who received a bortezomib-based regimen as rescue medication did not respond to treatment.
authors.affiliation: Clinical Division of Haematology and Bone Marrow Transplantation Unit, Antonio Perrino Hospital, Brindisi, Italy. giuseppemele2007@gmail.com
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Mele
authors.forename: Salvatore
authors.initials: S
authors.lastname: Pinna
authors.forename: Angela
authors.initials: A
authors.lastname: Melpignano
authors.forename: Giovanni
authors.initials: G
authors.lastname: Quarta
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage*; D000069286:Bortezomib; D005260:Female; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011719:Pyrazines / Q000008:administration & dosage*; D012189:Retrospective Studies
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib