title: IgD multiple myeloma a descriptive report of 17 cases: survival and response to therapy.
abstract: BACKGROUND
  Immunoglobulin D multiple myeloma (MM) is rare and has a poorer prognosis than other MM isotypes.
  
  
  METHODS
  Seventeen patients (pts) diagnosed from 1993 to 2009 with IgD MM were selected from six institutions of Multiple Myeloma Latium-Region GIMEMA Working Group.
  
  
  RESULTS
  Median age was 55 years, 14 patients had bone lesions, eight had renal impairment with estimated glomerular filtration rate (eGFR) < 50 ml/min, one serum calcium ≥ 12 mg/dl, 11 had lambda light chains, five stage III of ISS, six with chromosomal abnormalities. Six pts received conventional chemotherapy (CT): five melphalan + steroids based regimens. Eleven underwent high-doses of chemotherapy with autologous stem cell transplantation (HDT/ASCT), five single and six tandem ASCT: six received bortezomib and/or thalidomide as induction therapy and five VAD. Thalidomide maintenance was used in two pts: one in HDT/ASCT and one in CT group; bortezomib was used in one patient after HDT/ASCT. At a median follow up of 38 (range 19-60) and 50 months (range 17-148) for pts treated with CT and HDT/ASCT, respectively, the overall response rate (ORR) was 83% and 90%. In the group of patients treated with CT, median overall survival (OS) was 34 months (95% CI 15- 54 months), median progression free survival (PFS) was 18 months (95% CI 3-33 months) and median duration of response (DOR) was 7 months (95% CI 5-9 months). Median OS, PFS and DOR were not reached at the time of this analysis in the HDT/ASCT group of patients. Death was observed in 27.3% of pts treated with HDT/ASCT and in 66.7% undergone CT.
  
  
  CONCLUSIONS
  Despite the retrospective analysis and the small number of pts our study showed that the use of HDT/ASCT seems to improve also the prognosis of IgD MM patients. Treatment options including new drugs, before and after stem cell transplantation, may further improve the outcomes of these patients.
authors.affiliation: Department of Hematology, Regina Elena National Cancer Institute, Rome, Italy. fr.pisani@tiscali.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Pisani
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Petrucci
authors.forename: Diana
authors.initials: D
authors.lastname: Giannarelli
authors.forename: Velia
authors.initials: V
authors.lastname: Bongarzoni
authors.forename: Marco
authors.initials: M
authors.lastname: Montanaro
authors.forename: Valerio
authors.initials: V
authors.lastname: De Stefano
authors.forename: Giacinto
authors.initials: G
authors.lastname: La Verde
authors.forename: Fabiana
authors.initials: F
authors.lastname: Gentilini
authors.forename: Anna
authors.initials: A
authors.lastname: Levi
authors.forename: Tommaso
authors.initials: T
authors.lastname: Za
authors.forename: Alessandro
authors.initials: A
authors.lastname: Moscetti
authors.forename: Luciana
authors.initials: L
authors.lastname: Annino
authors.forename: Maria Concetta
authors.initials: MC
authors.lastname: Petti
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007072:Immunoglobulin D; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000401:mortality* / Q000628:therapy*; D009367:Neoplasm Staging; D012189:Retrospective Studies; D016019:Survival Analysis; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D007072:Immunoglobulin D