Dietrich, P.-Y

Duchosal, M. A.

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Annals of Oncology 19: 595–595, 2008letter to the editorBevacizumab therapy beforeautologous stem-celltransplantation for POEMSsyndromePOEMS is an acronym for a rare multisystemic syndromeencompassing polyneuropathy (P), organomegaly (O),endocrinopathy (E), monoclonal gammopathy (M), skinchanges (S) and additional features variably expressed [1].POEMS syndrome is associated with high vascular endothelialgrowth factor (VEGF) serum levels. This angiogenic factorlikely plays a pathogenic role, in particular for neuropathy,possibly via alteration of endoneurial vessels [2]. Treatmentoptions are still debated, but recently autologous stem-celltransplantation (ASCT) appears to be effective. This approachfor POEMS patients, however, was associated with severemorbidity, mainly respiratory failures that appeared to berelated to neuromuscular damage [3].We report a 45-year-old woman presenting with a rapidlyprogressive sensorimotor polyneuropathy involving allextremities, multiple sclerotic bone lesions, skinhyperpigmentation and hemangiomas, hepatomegaly,peripheral edema and papilledema. A monoclonalimmunoglobulin A k protein was detected (5 g/l) with a bonemarrow plasmocytosis (15%). Platelets were 600 · 109/l andcerebrospinal fluid proteins were 30 g/l. Electromyographic andnerve conduction analyses confirmed neuropathy with axonalabnormalities. Within few months appeared walkingimpossibility, swallowing difficulties and respiratory distress.Serum VEGF was highly elevated at 9700 pg/ml (normalrange: 62–707).Steroids treatment was declined by the patient. ASCT wasconsidered at high risk because of her poor condition(Karnofsky score of 50%), neurological defects and respiratoryfailure. We hypothesized that VEGF inhibition may relieveneuropathy. Bevacizumab therapy was initiated at 10 mg/kgevery 2 weeks. Pain relief was observed after two infusions,followed by major improvement in walking and breathingwithin 10 weeks. This was correlated with a drop in VEGF level(61 pg/ml) and a significant improvement inelectromyographic and nerve conduction studies. Treatmentwas continued for 4 months, and further clinical improvementwas observed, confirmed by electroneuromyography andpulmonary function tests. Hepatomegaly and skinabnormalities remained unchanged. Karnofsky scoreimproved at 80%, allowing ASCT conditioned with melphalan(200 mg/m2) to be realized in better conditions. The procedurewas well tolerated, and clinical outcome was excellent witha Karnofsky score at 90% 1 year after the procedure.Rapidly evolving neuropathy may hamper to carry outASCT in POEMS patients. Neurological defects in our POEMSpatient has been partially reversed by bevacizumab therapy,further supporting the role of VEGF in this complication.The large biological and clinical heterogeneities of POEMSsyndrome could explain variable sensitivities to anti-VEGFtherapy [4, 5]. Our report indicates that some patients maybenefit from bevacizumab to improve their clinical conditionbefore applying more standard treatments.P.-Y. Dietrich1* & M. A. Duchosal21Service of Oncology, University Hospital, Geneva, 2Service of Hematology,University Hospital, Lausanne, Switzerland(*E-mail: pierre-yves.dietrich@hcuge.ch)references1. Dispenzieri A, Kyle RA, Lacy MQ et al. POEMS syndrome: definitions andlong-term outcome. Blood 2003; 101: 2496–2506.2. Scarlato M, Previtali SC, Carpo M et al. Polyneuropathy in POEMS syndrome: roleof angiogenic factors in the pathogenesis. Brain 2005; 128: 1911–1920.3. Dispenzieri A, Moreno-Aspitia A, Suarez GA et al. Peripheral blood stemtransplantation in 16 patients with POEMS syndrome, and a review of theliterature. Blood 2004; 104: 3400–3407.4. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood2005; 106: 1135.5. Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome.Blood 2006; 107: 4972–4973.doi:10.1093/annonc/mdm602Published online 21 January 2008lettertotheeditorª 2008 European Society for Medical Oncology.For Permissions, please email: journals.permissions@oxfordjournals.org

Bevacizumab therapy before autologous stem-cell transplantation for POEMS syndrome

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