Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome.
Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0-175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, approximately 50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.
Dispenzieri
Angela
A
Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu
Lacy
Martha Q
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Hayman
Suzanne R
SR
Kumar
Shaji K
SK
Buadi
Francis
F
Dingli
David
D
Litzow
Mark R
MR
Gastineau
Dennis A
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Inwards
David J
DJ
Elliott
Michelle A
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Micallef
Ivana N
IN
Ansell
Stephen M
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Hogan
William J
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Porrata
Luis F
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Johnston
Patrick A
PA
Afessa
Bekele
B
Bryce
Alan
A
Kyle
Robert A
RA
Gertz
Morie A
MA
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000150:complications* / Q000601:surgery*; D036102:Peripheral Blood Stem Cell Transplantation / Q000009:adverse effects*; D012307:Risk Factors; D015996:Survival Rate; D013577:Syndrome; D013997:Time Factors