Wj. Hogan et al., Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation, BONE MAR TR, 28(3), 2001, pp. 305-309
Citations number
18
Categorie Soggetti
Hematology,"Medical Research Diagnosis & Treatment
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal ga
mmopathy, and skin changes) is a plasma cell dyscrasia that differs substan
tially from classic multiple myeloma. It is often associated with disabling
polyneuropathy in younger patients. Current therapeutic approaches are fre
quently inadequate and leave many patients wheelchair-bound with significan
t deterioration in quality and length of life. We present the case of a you
ng man with progressive disease despite conventional therapeutic approaches
. We describe a novel approach to treatment with a bone-seeking radiopharma
ceutical, samarium-153 ethylene diamine tetramethylene phosphonate (Sm-153-
EDTMP), followed by myeloablative chemotherapy with autologous hematopoieti
c progenitor cell reconstitution. This approach resulted in regression of t
he organomegaly and skin changes and in neurologic improvement both clinica
lly and electrophysiologically. The patient progressed from being wheelchai
r-bound to independent ambulation. An aggressive approach should be conside
red in patients with POEMS syndrome in whom standard therapeutic measures f
ail.