Plasma Cell precursor and Other Disorders
Category: Plasma Cell precursor and Other Disorders
CLINICAL CHARACTERISTICS AND LONG TERM OUTCOMES OF POEMS SYNDROME FROM A TERTIARY CARE CANCER CENTER
Amrit Dhar, MBBS,MD (he/him/his)
Fellow
Tata Memorial Hospital, Parel
This was a retrospective analysis at the Tata Memorial Centre in Mumbai. All patients diagnosed with POEMS syndrome (n=34) between January 2013 and November 2024 were reviewed for baseline features, treatment, and outcomes. All data were obtained from the electronic medical record system.
Results:
The median age was 51 years (range, 25-78), and 76% were male. The median time from symptom onset to diagnosis was 9 months (range 2–48). The most common presenting symptoms were parasthesisas and difficulty walking. Demyelinating pattern (78%) of neuropathy was most observed on NCV. Extravascular overload was observed in 76%, with peripheral edema and ascites being the most prevalent. Other characteristics were endocrinopathy (58%), organomegaly (56%), and skin abnormalities (40%). Serum VEGF levels could be measured in 58% (n=20), with a median of 514 pg/mL (range, 27-1930). Lenalidomide-based regimens were most employed in 88% of patients (n=28), with maintenance lenalidomide used in 32%. Autologous stem cell transplant (ASCT) was performed for only one patient. The best hematological response of VGPR was reported in 66%,with CR in 19% and PR in 10%. Amongst evaluable patients (n=15), 60% showed VEGF response of CR. The median duration of follow-up was 50 months. Three deaths were reported. 5-year PFS was 76.6%, with a median PFS of 105 months [95% CI, 96-113], and the 5-year OS was 90.8%, with a median OS of 125 months [95% CI, 109-142]. Univariate analysis revealed no significant factors for overall survival.
Conclusions:
Within the limitations of retrospective analysis, we describe the clinical features and treatment used for patients with POEMS in our context. With timely, precise diagnosis and appropriate treatment, patients have a good overall long-term survival rate despite not receiving ASCT.