title: Circulating peripheral blood plasma cells as a prognostic indicator in patients with primary systemic amyloidosis.
abstract: This study examined the prognostic value of circulating peripheral blood plasma cells (PBPCs) in patients with primary systemic amyloidosis (AL). A sensitive slide-based immunofluorescence technique was used to assess 147 patients for circulating PBPCs. Circulating monoclonal plasma cells were quantified as a percentage of circulating cytoplasmic immunoglobulin-positive cells (PBPC%). The absolute circulating plasma cell count was also determined. When analyzed retrospectively, 24 (16%) of 147 patients were found to have detectable circulating PBPCs. Overall survival for patients with high PBPC%'s (> 1%) was poorer (median survival, 10 vs 29 months; P =.002). Similarly, overall survival for patients with high PBPC counts (> 0.5 x 10(6)/L) was significantly poorer (median, 13 vs 31 months; P =.003). Increased percentages of bone marrow plasma cells (BMPC%; P =.0004), increased levels of serum beta(2)-microglobulin (P =.04), and dominant cardiac amyloid involvement (P =.03) also predicted poorer survival. The combined consideration of circulating PBPCs and BMPC% identified low-, intermediate-, and high-risk groups with median survivals of 37.5, 15.5, and 10 months, respectively (P =.0003). Multivariate analysis revealed circulating PBPCs and BMPC% to be independent prognostic factors for survival. Patients with PBPC%'s of 2% or higher were significantly more likely to have a coexisting clinical diagnosis of multiple myeloma (50% vs 12%, P =.008). The prognostic value of circulating PBPCs may help select treatment for patients with AL.
authors.affiliation: Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Witzig
authors.forename: Georgene
authors.initials: G
authors.lastname: Schroeder
authors.forename: Edwin A
authors.initials: EA
authors.lastname: McElroy
authors.forename: Rafael
authors.initials: R
authors.lastname: Fonseca
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.forename: John A
authors.initials: JA
authors.lastname: Lust
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: Philip R
authors.initials: PR
authors.lastname: Greipp
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000097:blood* / Q000453:epidemiology / Q000401:mortality*; D001772:Blood Cell Count; D001773:Blood Cells / Q000473:pathology; D002999:Clone Cells / Q000473:pathology; D015897:Comorbidity; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000453:epidemiology; D010950:Plasma Cells / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D012306:Risk; D016019:Survival Analysis