Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation

被引:49
作者
Rovira, M
Carreras, E
Bladé, J
Graus, F
Valls, J
Fernández-Avilés, F
Montserrat, E
机构
[1] Univ Barcelona, Hosp Clin, Inst Invest Biomed August Pi Y Sunyer,Dept Haemat, Postgrad Sch Haematol Farreras Valenti,BMT Unit, E-08036 Barcelona, Spain
[2] Univ Barcelona, Hosp Clin, Inst Invest Biomed August Pi Y Sunyer,Dept Neurol, Postgrad Sch Haematol Farreras Valenti,BMT Unit, E-08036 Barcelona, Spain
关键词
POEMS syndrome; autologous haematopoietic cell transplantation; polyneuropathy; plasma cell dyscrasia; osteoesclerotic myeloma;
D O I
10.1046/j.1365-2141.2001.03040.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:373 / 375
页数:3
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