AI Article Synopsis
- The study analyzed data from 25,423 patients with MGUS, multiple myeloma (MM), and AL amyloidosis to examine the impact of familial plasma cell disorders (PCD) on patient prognosis.
- Among the cohort, only 2.7% reported a family history of PCD, but those with such a history had significantly longer overall survival rates compared to those without.
- However, MGUS patients with familial PCD showed a higher risk of progressing to more severe diseases like MM or AL amyloidosis, indicating a complex relationship between family history and disease outcomes.
Article Abstract
The association between familial plasma cell disorders (PCD) and prognosis in patients with MGUS, multiple myeloma (MM), and systemic light chain (AL) amyloidosis has not been well described. This study retrospectively reviewed outcomes of 25,423 patients (16,744 MGUS, 6194 MM, 2955 AL amyloidosis). Overall, 2.7% of patients reported having a family member with a PCD (defined as MGUS, MM, or AL amyloidosis). Family history was documented in 94% of MGUS, 92% of MM, and 88% of AL amyloidosis patients. The overall survival was consistently longer in patients with versus without familial PCD (crude hazard ratios: 0.52, 95% CI 0.40-0.67, p < 0.001 for MGUS patients; 0.68, 95% CI 0.57-0.79, p < 0.001 for MM patients; 0.60, 95% CI 0.43-0.84, p = 0.003 for AL patients). This association remained consistent when adjusting for baseline patient and disease characteristics. In MGUS patients, the risk of progression to MM, AL amyloidosis, or a lymphoproliferative disorder was higher in patients with familial PCD when accounting for death as a competing risk (cause-specific HR 1.9, 95% 1.3-2.7, p < 0.001). This is the first study to demonstrate that in a cohort of MGUS, MM, and systemic AL amyloidosis, patients with a PCD family history have an improved overall survival.
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| http://dx.doi.org/10.1038/s41375-021-01454-4 | DOI Listing |
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