Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation.
POEMS syndrome is a rare plasma cell disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, serum monoclonal protein, and skin lesions. Although not included in the acronym, renal lesions also are characteristic of this disease and sometimes require dialysis therapy. We treated a 61-year-old woman with POEMS syndrome with high-dose melphalan therapy (HDT) supported by autologous blood stem cell transplantation (SCT), and clinical remission was achieved. A repeated renal biopsy showed the striking effectiveness of this therapy on renal lesions. Pathological features of the renal lesions, such as membranoproliferative glomerulonephritis-like lesions, microangiopathic glomerulopathy, and mesangiolytic lesions with microcapillaries, almost completely disappeared. This treatment also markedly decreased serum levels of vascular endothelial growth factor (VEGF). These findings indicate that HDT with SCT is effective, even on renal lesions in patients with POEMS syndrome, and suggest that high serum VEGF concentrations are associated closely with the development of renal lesions in patients with this type of plasma cell disorder.
Sanada
Satoru
S
Division of Molecular Medicine, Center for Translational and Advanced Animal Research, Tohoku University School of Medicine, Sendai, Japan. satsanada@mvg.biglobe.ne.jp
Ookawara
Susumu
S
Karube
Hiroki
H
Shindo
Tetsuro
T
Goto
Toshikazu
T
Nakamichi
Takashi
T
Saito
Mikio
M
Matsubara
Mitsunobu
M
Suzuki
Masayuki
M
D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D016878:POEMS Syndrome / Q000150:complications*; D036102:Peripheral Blood Stem Cell Transplantation; D012074:Remission Induction; D012720:Severity of Illness Index
D008558:Melphalan