Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation.
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, serum monoclonal protein and skin changes) is a rare plasma cell disorder of unknown pathogenesis and is diagnosed by the demonstration of a plasma cell proliferation at the biopsy of an osteoesclerotic lesion. When the lesions are in a limited area, radiation therapy is usually highly effective. Patients with disseminated disease require systemic chemotherapy, which is not effective in most cases. A patient with severe widespread POEMS syndrome resistant to melphalan who experienced a dramatic improvement after high-dose melphalan followed by autologous haematopoietic cell transplantation (AHCT) is reported. We believe that this is the first reported case of POEMS syndrome treated with AHCT, a procedure that could be considered in similarly affected patients.
Rovira
M
M
Department of Haematology, BMT Unit, Postgraduate School of Haematology Farreras-Valentí, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, University of Barcelona, Barcelona, Spain. mrovira@clinic.ub.es
Carreras
E
E
Bladé
J
J
Graus
F
F
Valls
J
J
Fernández-Avilés
F
F
Montserrat
E
E
D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008558:Melphalan / Q000627:therapeutic use; D016878:POEMS Syndrome / Q000628:therapy*; D014182:Transplantation, Autologous; D016896:Treatment Outcome
D018906:Antineoplastic Agents, Alkylating; D008558:Melphalan