Beyond the acronym, POEMS syndrome, a multisystemic enigma: a case report and comprehensive evidence-based perspective
DOI:
https://doi.org/10.18203/2320-6012.ijrms20252039Keywords:
POEMS syndrome, Polyradiculoneuropathy, Polyneuropathies, Chronic inflammatory demyelinatingAbstract
The POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) is a rare and complex disease involving a variety of body systems. Emphasis is placed on peripheral neuropathy and the presence of a monoclonal protein. Additionally, major and minor criteria, including bone lesions, elevated VEGF and Castleman's disease, are significant factors. On the therapeutic front, options range from radiotherapy to systemic therapy, underscoring the importance of an early treatment response. A 64-yearsold diabetic male, complain with weakness in lower limbs, along with edema and hyperpigmentation. He is diagnosed with neuropathy and CIDP is confirmed in EMG. An abdominal CT scan reveals hepatomegaly. Elevated proteins are identified as a finding in the liver function tests. Subsequent serum electrophoresis confirms an increase in M protein. Treatment with bortezomib and dexamethasone is initiated, resulting in clinical improvement. Despite this progress, the patient discontinues follow-up appointments. The POEMS syndrome is a diagnostic challenge, especially when primary symptom is polyneuropathy. The main key to diagnosis is the identification of the M protein through electrophoresis and immunofixation. A multidisciplinary diagnostic remains essential in addressing its complexity.
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References
Kim YR. Update on the POEMS syndrome. Blood Res. 2022;57(1):27-31. DOI: https://doi.org/10.5045/br.2022.2022001
Ali T, Qazilbash MH. POEMS syndrome: A multisystem clonal disorder. Eur J Haematol. 2021;106(1):14-8. DOI: https://doi.org/10.1111/ejh.13514
Jurczyszyn A, Olszewska-Szopa M, Vesole D. POEMS Syndrome-Clinical Picture and Management. Current Knowledge. Clin Lymphoma Myeloma Leuk. 2023;23(8):575-82. DOI: https://doi.org/10.1016/j.clml.2023.04.008
Keddie S, Foldes D, Caimari F. Clinical characteristics, risk factors and outcomes of POEMS syndrome: A longitudinal cohort study. Neurology. 2020; 95:268. DOI: https://doi.org/10.1212/WNL.0000000000009940
Sung JY, Kuwabara S, Ogawara K. Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve. 2002;26:189. DOI: https://doi.org/10.1002/mus.10182
Abe D, Nakaseko C, Takeuchi M. Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. Blood 2008; 112:836. DOI: https://doi.org/10.1182/blood-2007-12-126979
D'Sa S, Khwaja J, Keddie S. Comprehensive Diagnosis and Management of POEMS Syndrome. Hemasphere. 2022;6(11):796. DOI: https://doi.org/10.1097/HS9.0000000000000796
Khouri J, Nakashima M, Wong S. Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy and Skin Changes) Syndrome: A Review. JAMA Oncol. 2021;7(9):1383-91. DOI: https://doi.org/10.1001/jamaoncol.2021.0586
Masaki Y, Arita K, Sakai T. Castleman disease and TAFRO syndrome. Ann Hematol. 2022;101(3):485-90. DOI: https://doi.org/10.1007/s00277-022-04762-6
Dong X, Jing R, Li J. POEMS syndrome. Am J Med Sci. 2023;365(1):13. DOI: https://doi.org/10.1016/j.amjms.2022.08.020
Miest RY, Comfere NI, Dispenzieri A. Cutaneous manifestations in patients with POEMS syndrome. Int J Dermatol. 2013;52:1349. DOI: https://doi.org/10.1111/j.1365-4632.2012.05648.x
Khwaja J, D'Sa S, Lunn MP. Evidence-based medical treatment of POEMS syndrome. Br J Haematol. 2023;200(2):128-36. DOI: https://doi.org/10.1111/bjh.18400
Koike H, Katsuno M. Paraproteinemia and neuropathy. Neurol Sci. 2021;42(11):4489-501. DOI: https://doi.org/10.1007/s10072-021-05583-7
Dispenzieri, A. POEMS Syndrome: 2020 Update on Diagnosis, Risk-stratification and Management. American J Hematol. 2020;95(6):648-59. DOI: https://doi.org/10.1002/ajh.25856
Li J, Zhang W, Jiao L. Clinical characteristics, treatments and prognostic factors of POEMS syndrome: A retrospective study of 68 patients. Medicine. 2021;100(30):26717.
Ohwada C, Sakaida E, Kawajiri-Manako C. Long-term evaluation of physical improvement and survival of autologous stem cell transplantation in POEMS syndrome. Blood. 2018;131:2173. DOI: https://doi.org/10.1182/blood-2017-07-795385
Tomkins O, Keddie S, Lunn MP. High-dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise. Br J Haematol. 2019;186:78. DOI: https://doi.org/10.1111/bjh.16057
Li J, Tian Z, Zheng HY. POEMS syndrome: a retrospective study of 37 Chinese patients. Clinical Lymphoma Myeloma and Leukemia. 2020;20(8):512-9.
Kourelis TV, Kumar SK, Gertz MA. Advances in the diagnosis, classification and treatment of POEMS syndrome. Blood Cancer J. 2020;10(1):1-13.
Watanabe O, Maruyama I, Arimura K. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle & Nerve: Official Journal of the American Association of Electrodiagnostic Med. 2020;23(12):1817-20.
Brown R, Ginsberg L. POEMS syndrome: clinical update. J Neurol. 2019;266(1):268-77. DOI: https://doi.org/10.1007/s00415-018-9110-6
Guo X, Qin X, Zhang Y, Huang C, Yu G. Electrophysiological features of POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Clin Neurosci. 2014;21(4):587-90. DOI: https://doi.org/10.1016/j.jocn.2013.05.023