Dear Editor, A 32-year-old woman was admitted because of progressive numbness in the lower limbs for 15 months, accompanied with pains in the breastbone, spine, and pelvis for 6 months before hospitalization. There was no prior history of exposure to neurologic toxins and no family history of neuropathy. Physical examination revealed splenomegaly, and no hyperpigmentation or other skin changes were observed. Neurological examination demonstrated moderate sensory disturbance of both lower limbs, with disappearance of deep tendon reflexes. Laboratory analysis showed that full blood count and urine routine test are normal. Other normal or negative results include hepatic and renal functions test, folate and VitB12 levels, and auto-antibody screen. Ophthalmoscopy revealed bilateral papilledema. Endocrinologic investigations showed low levels of FT3, FT4, testosterone, and estrogen. Electromyographic analysis demonstrated peripheral motor and sensory nerves demyelinating lesions in upper and lower limbs. Multiple osteosclerotic lesions in ribs, breastbone, spine, and pelvis were disclosed by CT scan (Fig. 1), together with a few lytic lesions in pelvis. Monoclonal IgG (κ) (20.3 g/l)(normal, 7.0–17.0 g/l) was detected in the serum by electrophoresis. There was no Bence-Jones proteinuria. Bone marrow aspiration revealed 2% of mature plasma cell with normal cytogenetics. Normal levels of protein, glucose and cells were detected in the cerebrospinal fluid (CSF). The patient met the criteria for the diagnosis of POEMS syndrome described by Dispenzieri [1]. She was initially treated with two cycles of chemotherapy with MP regimen (melphalan 6 mg/day days 1–7, Dexamethasone 40 mg/day, days 1–4, days 9–12, days 17–20). A minor improvement of numbness in the lower limbs was observed after chemotherapy, with disappearance of splenomegaly and papilledema, but the patient started to present with mild distal weakness of both lower limbs and toes. Bone pains in the breastbone, spine, and pelvis remained, but were stable. As she developed motor deficiency despite systemic chemotherapy, 5 months after diagnosis, she received autologous peripheral blood stem cell transplantation (autoPBSCT). Peripheral blood stem cell collection was performed after mobilization by chemotherapy (intravenous cyclophosphamide 60 mg/kg/day × 2 days), plus subcutaneous granulocyte colony-stimulating factor (rhG-CSF, 5 mg/kg/day) 6 days after chemotherapy and lasted for 3 days. The number of collected mononuclear cells (MNC) was 6.70×10/kg, and the CD34 cells were 7.51×10/kg. High-dose chemotherapy and autoPBSCT were performed 44 days after PBSC collection. She was conditioned using 200 mg/m melphalan, and the PBSC were infused 3 days later. Standard supportive care with prophylactic antibiotics was used before transplantation. Growth factor support was provided 3 days after stem cell transfusion. The transplantation course was uneventful except for a transient neutropenic fever and mucositis, which were settled with broad-spectrum antibacterial therapy. The patient was engrafted and the time to neutrophil count above 0.5×10/ l and platelets above 20×10/l was +10 and +11 days, respectively. Numbness in lower limbs improved gradually, and the area of sensory disturbance of numbness was limited in the bottoms of feet 6 months after transplantation, although Ann Hematol (2008) 87:247–248 DOI 10.1007/s00277-007-0379-2
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