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Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome
Author(s) -
Dispenzieri Angela,
Lacy Martha Q.,
Hayman Suzanne R.,
Kumar Shaji K.,
Buadi Francis,
Dingli David,
Litzow Mark R.,
Gastineau Dennis A.,
Inwards David J.,
Elliott Michelle A.,
Micallef Iva.,
Ansell Stephen M.,
Hogan William J.,
Porrata Luis F.,
Johnston Patrick A.,
Afessa Bekele,
Bryce Alan,
Kyle Robert A.,
Gertz Morie A.
Publication year - 2008
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2008.01037.x
Subject(s) - organomegaly , poems syndrome , medicine , polyneuropathy , thrombocytosis , context (archaeology) , surgery , gastroenterology , peripheral edema , autologous stem cell transplantation , transplantation , adverse effect , paleontology , platelet , biology
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, ∼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.