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ARTICLES: S. Kuwabara, S. Misawa, K. Kanai, Y. Suzuki, Y. Kikkawa, S. Sawai, T. Hattori, M. Nishimura, and C. Nakaseko Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome Neurology 2008; 71: 1691-1695 [Abstract] [Full text] [PDF]

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Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome

Mario Sabatelli, Marco Luigetti, Luca Laurenti, Amelia Conte, Francesca Madia, Silvia De Matteis, Patrizia Chiusolo, Michela Tarnani, and Simona Sica   (6 February 2009)

Reply from the authors

Satoshi Kuwabara, Sonoko Misawa, Kazuaki Kanai, and Chiaki Nakaseko   (6 February 2009)

Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome 6 February 2009
Mario Sabatelli, Catholic University of Sacred Heart Lgo A. Gemelli 8, 00168 Rome, Italy, Marco Luigetti, Luca Laurenti, Amelia Conte, Francesca Madia, Silvia De Matteis, Patrizia Chiusolo, Michela Tarnani, and Simona Sica Send Correspondence to journal: Re: Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome msabatelli{at}rm.unicatt.it Mario Sabatelli, et al.	We read with interest the recent article by Kuwabara et al. describing the clinical and electrophysiological findings in nine patients affected by polyneuropathy, organomegaly, endocrinopathy, M- protein and skin changes (POEMS) syndrome and treated with autologous peripheral blood stem cell transplantation (auto-PBSCT). [1] From May 2003 until September 2006, we treated four patients affected by POEMS syndrome with chemotherapy and auto-PBSCT. [2] There were three men and one woman with a median age of 53 years (range 44–62). Three patients had severe and rapidly progressive sensory–motor peripheral neuropathy with predominant involvement of the lower limbs leading to inability to walk, reduced muscle strength of the upper limbs, absent deep tendon reflexes, and distal sensory impairment. One patient presented with a mild clinical picture of distal paresthesias and hypoesthesia in the lower limbs and normal muscle strength. The median time from first symptoms to diagnosis was 7 months (range 4–48 months). Before auto-PBSCT, three out of four patients received standard treatment for inflammatory neuropathy with no improvement. Time from the diagnosis to auto-PBSCT was 3 months. After a median follow-up of 52.5 months (range 24–64 months), all patients are still living and have marked improvement of neurological symptoms. At last follow-up, the three patients with tetraparesis were able to walk but had severe weakness of tibio-peroneal muscles. Their upper limb strength was normal and sensory disturbances had improved. The patient who had only sensory disturbances had also improved and this was confirmed by electrophysiological studies. Our experience with a longer follow-up confirms Kuwabara et al.’s findings. It is crucial to establish a diagnosis in a short time from symptom onset to avoid the typical progression of POEMS syndrome and increase the probability of recovery. Our only patient who had a delayed diagnosis (48 months) still markedly improved, although mechanical ventilation for respiratory failure was required at the time of auto- PBSCT. In conclusion, auto-PBSCT could be considered the first-line treatment in POEMS syndrome at any clinical stage. Regular follow-up, including vascular endothelial growth factor VEGF dosage, is necessary to prevent the possibility of relapse. [3-4] It has been shown that in the event of relapse, bevacizumab therapy may be a viable option. [5] References 1. Kuwabara S, Misawa S, Kanai K, et al. Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology 2008;71:1691-1695. 2. Laurenti L, De Matteis S, Sabatelli M et al. Early diagnosis followed by front-line autologous peripheral blood stem cell transplantation for patients affected by POEMS syndrome. Leuk Res 2008;32:1309-1312. 3. Scarlato M, Previtali SC, Carpo M et al. Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 2005;128:1911-1920. 4. Samaras P, Bauer S, Stenner-Liewen F et al. Treatment of POEMS syndrome with bevacizumab. Haematologica 2007;92:1438-1439. 5. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood 2005;106:1135. Disclosure: The authors report no disclosures.
Reply from the authors 6 February 2009
Satoshi Kuwabara, Department of Neurology, Chiba University School of Medicine 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670, Japan, Sonoko Misawa, Kazuaki Kanai, and Chiaki Nakaseko Send Correspondence to journal: Re: Reply from the authors kuwabara-s{at}faculty.chiba-u.jp Satoshi Kuwabara, et al.	We thank Dr. Sabatelli et al. for their comments. They confirmed beneficial effects of auto-PBSCT for POEMS syndrome in their four patients with longer follow-up period (median, 52.5 months) than our nine patients (median, 20 months), all of them showing dramatic improvement in neuropathy and other symptoms. We began treating patients with auto-PBSCT for POEMS syndrome in December 2003, and the first four patients have now been followed for 54 to 60 months. We agree that it is vital to establish a diagnosis in a short time from symptom onset to avoid the progression typical of POEMS syndrome and increase the probability of recovery. Dr. Sabatelli et al. also mention that three of their four patients received standard treatment for inflammatory neuropathy with no improvement. POEMS syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP) cause peripheral nerve demyelination, and the electrodiagnostic findings may be similar. Most POEMS patients show peripheral neuropathy as an initial manifestation, and could be misdiagnosed as having CIDP. Two of our nine patients were initially diagnosed as having CIDP, and one of them had been treated with repeated intravenous immunoglobulin for 2 years without benefit. For early diagnosis, we suggest that electrodiagnostic features should be investigated in addition to careful examination for edema and skin changes. We have reviewed nerve conduction studies of POEMS patients and compared their results with those in CIDP patients. [6] The patients with POEMS syndrome showed: slowing of nerve conduction that was more predominant in the intermediate than distal nerve segments; rare conduction block; and more severe attenuation of compound muscle action potentials in the lower than upper limbs. Findings in the CIDP patients were characterized by multifocal conduction slowing that was occasionally dominant distally, frequent conduction block, and less discrepancy between upper and lower limb nerves. These findings were confirmed by a later study. [7] The pattern of nerve conduction abnormalities differs between these disorders. Recognition of these typical patterns may be helpful for early diagnosis of POEMS syndrome. References 6.Sung JY, Kuwabara S, Ogawara K, Kanai K, Hattori T. Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve 2002;26:189-193. 7.Min JH, Hong YH, Lee KW. Electrophysiological features of patients with POEMS syndrome. Clin Neurophysiol 2005;116:965-968. Disclosure: The authors report no disclosures.