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Circulating peripheral blood plasma cells as a prognostic indicator in patients with primary systemic amyloidosis
Animesh Pardanani1, Thomas E Witzig, Georgene Schroeder
1Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
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.
Insights
Detecting circulating plasma cells in AL amyloidosis patients indicates a poorer prognosis. High levels of circulating plasma cells (PBPCs) and bone marrow plasma cells (BMPC%) are independent predictors of survival, aiding treatment decisions.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Primary systemic amyloidosis (AL) is a plasma cell disorder.
- Prognostic markers are crucial for managing AL amyloidosis.
- Circulating plasma cells (PBPCs) have been investigated as potential biomarkers.
Purpose of the Study:
- To evaluate the prognostic significance of circulating peripheral blood plasma cells (PBPCs) in patients with AL amyloidosis.
- To determine if PBPCs can predict overall survival.
Main Methods:
- A sensitive slide-based immunofluorescence technique was used.
- 147 patients with AL amyloidosis were assessed for circulating PBPCs.
- Circulating monoclonal plasma cells were quantified as a percentage (PBPC%) and absolute count.
Main Results:
- Detectable circulating PBPCs were found in 16% of patients.
- High PBPC% (>1%) and high PBPC count (>0.5 x 10(6)/L) were associated with significantly poorer overall survival.
- Increased bone marrow plasma cells (BMPC%), elevated serum beta(2)-microglobulin, and cardiac amyloid involvement also predicted poorer survival.
- Combined PBPCs and BMPC% identified distinct risk groups with varying survival rates.
- Circulating PBPCs and BMPC% were independent prognostic factors in multivariate analysis.
- Higher PBPC% (≥2%) correlated with a higher likelihood of coexisting multiple myeloma.
Conclusions:
- Circulating peripheral blood plasma cells are a valuable independent prognostic factor in AL amyloidosis.
- PBPC levels can help stratify patients into risk groups.
- The findings suggest that PBPCs may aid in treatment selection for AL amyloidosis patients.