Circulating peripheral blood plasma cells as a prognostic indicator in patients with primary systemic amyloidosis.
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.
Pardanani
Animesh
A
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Witzig
Thomas E
TE
Schroeder
Georgene
G
McElroy
Edwin A
EA
Fonseca
Rafael
R
Dispenzieri
Angela
A
Lacy
Martha Q
MQ
Lust
John A
JA
Kyle
Robert A
RA
Greipp
Philip R
PR
Gertz
Morie A
MA
Rajkumar
S Vincent
SV
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