Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation

Citation
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
Journal title
BONE MARROW TRANSPLANTATION
ISSN journal
02683369 → ACNP
Volume
28
Issue
3
Year of publication
2001
Pages
305 - 309
Database
ISI
SICI code
0268-3369(200108)28:3<305:STOPSW>2.0.ZU;2-5
Abstract
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.