"A case of successful allogeneic peripheral blood stem cell transplant (APBSCT) for POEMS syndrome"

초록

Seong Hye Choi, Moon Hyun Chung, Jong Weon Choi, Soon Ki Kim (Incheon, KOR) This is the first report in the world of a successful APBSCT for POEMS syndrome, which affected a 46-year-old man. He underwent APBSCT in January 2002 from his HLA identical sister, and his disease is getting better as of this writing, 10 months post APBSCT. Although his disease began in 1993 with weakness and ascites, the diagnosis of POEMS syndrome was made in 2001, based on polyneuropathy, hepatosplenomegaly, endocrinopathy (hypothyroidism), monoclonal protein (IgA lambda), marrow plasmocytosis (10%), and skin discoloration. Osteosclerotic bone lesions were absent. Cytogenetic study of marrow cells disclosed a karyotype of 46, XY, inv(9)(p12q13). He used to be placed on various treatments; prednisone, melphalan, cyclophosphamide, and transjugular intrahepatic portosystemic shunt, under the impression of lymphoma or Castleman?s disease with little benefit from the therapies. On referral to us, he was bedridden with marked emaciation, massive right pleural effusion, and prominent ascites, which had us opt APBSCT rather than autologous transplant for the treatment. He was prepared with fludarabine (50mg on days ?8 to ?4) and melphalan (120mg on days ?3 to ?2). GVHD prophylaxis consisted of cyclosporine. Engraftment of marrow was documented on day 14, under G-CSF support, in mixed chimerism with 1.8% of host DNA. Severe hepatic veno-occlusive disease, renal insufficiency, acute GVHD of grade 1, and CMV antigenemia appeared in immediate post APBSCT period to be treated and resolved. Spontaneous bacterial peritonitis and pseudomembraneous colitis followed at 3-4 months. Aspiration pneumonia developed at 5 months secondary to swallowing difficulty, which forced us to feed him through Levin tube for 3 months. Ascites, hepatosplenomegaly, and monoclonal protein were gone at 6 months. The swallowing difficulty disappeared at 8 months right after he was given high dose immunoglobul