Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation
2001; Wiley; Volume: 115; Issue: 2 Linguagem: Inglês
10.1046/j.1365-2141.2001.03040.x
ISSN1365-2141
AutoresMontserrat Rovira, Enric Carreras, Joan Bladé, Francesc Graus, J. Valls, Francisco Fernández‐Avilés, Emili Montserrat,
Tópico(s)Amyloidosis: Diagnosis, Treatment, Outcomes
ResumoPOEMS 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.
Referência(s)