title: Efficacy of lenalidomide in POEMS syndrome: a retrospective study of 20 patients.
abstract: POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18-FDG-PET/CT at diagnosis (11 with positive findings), and nine patients during follow-up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow-up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses.
authors: Service d'hématologie, Centre Hospitalier Universitaire (CHU) Amiens, France.
authors: Bruno
authors: B
authors: Royer
authors: Lavinia
authors: L
authors: Merlusca
authors: Julie
authors: J
authors: Abraham
authors: Lucile
authors: L
authors: Musset
authors: Julien
authors: J
authors: Haroche
authors: Sylvain
authors: S
authors: Choquet
authors: Xavier
authors: X
authors: Leleu
authors: Catherine
authors: C
authors: Sebban
authors: Olivier
authors: O
authors: Decaux
authors: Lionel
authors: L
authors: Galicier
authors: Muriel
authors: M
authors: Roussel
authors: Christian
authors: C
authors: Recher
authors: Anne
authors: A
authors: Banos
authors: Isabelle
authors: I
authors: Guichard
authors: Jean-Marie
authors: JM
authors: Brisseau
authors: Pascal
authors: P
authors: Godmer
authors: Olivier
authors: O
authors: Hermine
authors: Gaël
authors: G
authors: Deplanque
authors: Thierry
authors: T
authors: Facon
authors: Bouchra
authors: B
authors: Asli
authors: Véronique
authors: V
authors: Leblond
authors: Jean-Paul
authors: JP
authors: Fermand
authors: Jean Pierre
authors: JP
authors: Marolleau
authors: Arnaud
authors: A
authors: Jaccard
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D003907:Dexamethasone / Q000494:pharmacology / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D000077269:Lenalidomide; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D049268:Positron-Emission Tomography; D011859:Radiography; D012008:Recurrence; D012189:Retrospective Studies; D013792:Thalidomide / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
chemical_list: D000970:Antineoplastic Agents; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D013792:Thalidomide; D003907:Dexamethasone; D000077269:Lenalidomide