Abstract:
:The POEMS syndrome (polyradiculoneuropathy, organomegaly, multiple endocrinopathies, monoclonal protein, skin changes) is a rare disease associated with a plasma cell dyscrasia. Patients with disseminated POEMS can be treated with high-dose therapy and autologous stem cell transplantation (ASCT). While clinical improvement is nearly universal in these patients, the long-term outcomes after transplantation are unclear. We therefore assessed the long-term clinical outcomes of 59 POEMS patients treated with ASCT at our institution. With a median follow-up of 45 months, 14 patients have progressed with a progression-free survival of 98% and 75% at 1 and 5 years, respectively. Factors associated with progression have included an IgG-λ monoclonal component (hazard ratio [HR] 7.5; 95% confidence interval [CI], 2.3-28.3; P = .0008), fluorodeoxyglucose-avid lesions on baseline positron emission tomography (HR 6.4; 95% CI, 1.2-120; P = .03), lack of complete hematologic response (HR 5.4; 95% CI, 1.8-16.7; P = .003), and patients aged 50 years or younger at transplantation (HR 4.4; 95% CI, 1.3-20; P = .01). The most common progression events have been radiologic followed by rising VEGF. Symptomatic progression has been rare. Most patients could be salvaged with immunomodulatory drugs or radiation. The 5-year survival is 94%. Herein, we describe a system of monitoring response and progression among patients with POEMS after ASCT.
journal_name
Bloodjournal_title
Bloodauthors
D'Souza A,Lacy M,Gertz M,Kumar S,Buadi F,Hayman S,Dingli D,Zeldenrust S,Kyle R,Ansell S,Inwards D,Johnston P,Micallef I,Porrata L,Litzow M,Gastineau D,Hogan W,Dispenzieri Adoi
10.1182/blood-2012-04-423178subject
Has Abstractpub_date
2012-07-05 00:00:00pages
56-62issue
1eissn
0006-4971issn
1528-0020pii
blood-2012-04-423178journal_volume
120pub_type
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