(PA-330) Prognostic Determinants of Overall Survival in POEMS Syndrome: A 7-Year Single-Center Retrospective Analysis

POEMS syndrome is a rare and complex plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. While outcomes have improved with advances in plasma cell–directed therapies, prognosis varies considerably, especially in patients who develop coexisting hematologic malignancies such as multiple myeloma, AL amyloidosis, or lymphoma. The rarity and heterogeneity of the disease have limited efforts to define reliable prognostic markers. In this retrospective study, we aimed to identify clinical and laboratory factors associated with overall survival (OS) in a large real-world cohort of patients with POEMS.

Methods:   

We reviewed 78 patients diagnosed with POEMS syndrome at a tertiary medical center between January 2018 and April 2025. Baseline demographics, laboratory values, treatment approaches, and survival data were collected. Patients were grouped based on whether they had coexisting hematologic malignancies. Most patients received systemic therapy, including regimens based on bortezomib, lenalidomide, and dexamethasone. Multivariate Cox regression was used to identify independent predictors of OS. 

Results:   

The median age at diagnosis was 55.5 years (range: 21–76), and 69.2% were male. Neurological symptoms were the most common presenting complaint (70.5%), followed by peripheral edema (17.9%). The median time from symptom onset to diagnosis was 8 months. Organomegaly (79.5%), fluid overload (71.8%), and endocrinopathy (48.7%) were frequently observed at baseline.

Of the 60 patients who received systemic therapy, 53.3% achieved complete remission and 26.7% achieved partial remission or better. After a median follow-up of 34 months, the estimated 5-year OS rate was 80.0%. In multivariate analysis, age >60 years, serum albumin < 33 g/L, and serum IgA >5.9 g/L emerged as independent predictors of poorer OS (all p < 0.05).

In subgroup analysis, patients with concomitant hematologic malignancies had significantly higher IgA levels, lower platelet counts, and reduced eGFR compared to those without. These findings suggest a more aggressive disease phenotype and correspondingly worse outcomes in this subgroup.

 

Conclusions:   

This study highlights several practical prognostic indicators for POEMS syndrome. Advanced age, low serum albumin, and elevated IgA levels were each independently associated with reduced survival. The presence of coexisting hematologic malignancies further identifies a high-risk group with more aggressive clinical features. These findings emphasize the importance of early risk stratification and may inform individualized treatment strategies. Prospective multicenter studies are warranted to validate these observations and better understand the biological mechanisms driving outcome differences in POEMS.