Abstract
We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders.
Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
Sommario
Descriviamo 6 pazienti con sindrome POEMS (Polineuropatia, Organomegalia, Endocrinopatia, gammopatia Monoclonale, alterazioni cutanee [Skin changes]), una peculiare malattia ad interessamento multisistemico frequentemente associata a mieloma osteosclerotico o ad altre malattie plasmacellulari. Una polineuropatia sensitivo-motoria si accompagnava ad interessamento pluriviscerale in tutti i pazienti, a mieloma osteosclerotico in due casi, a gammopatia monoclonale di incerto significato in altri due, e a malattia di Castleman negli ultimi due pazienti. In tutti i 6 casi la biopsia del nervo surale dimostrava una neuropatia mista, demielinizzante ed assonale. In due casi sono stati trovati elevati livelli di Interleuchina-6, ma la patogenesi della malattia è ancora ignota.
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References
Beck J.T., Hsu S.M., Wijdenes J., et al.:Brief report: alleviation of systemic manifestations of Castleman's disease by monoclonal anti-interleukin-6 antibody. N. Engl. J. Med., 330:602–605, 1994.
Donaghy M., Hall P., Gawler J., et al.:Peripheral neuropathy associated with Castleman's disease. J. Neurol. Sci., 89:253–267, 1989.
Fukatsu A., Ito Y., Yuzawa Y., et al.:A case of POEMS syndrome showing elevated serum interleukin-6 and abnormal expression of interleukin-6 in the kidney. Nephron 62:47–51, 1992.
Gherardi R., Baudrimont M., Kujas M., et al.:Pathological findings in three non-Japanese patients with the POEMS syndrome. Virchows Arch. (A), 413:357–365, 1988.
Gherardi R., Chouaied S., Malapert D., Fromont G., Bélec L., Degos J.D.:Increased release of monokines in POEMS syndrome: a pathogenic clue for the multi-system dysfunction. Neurology, 43 (S2):A419, 1993.
Isaacson P.G.:Commentary. Castleman's disease. Histopathology, 14:429–432, 1989.
Kyle R.A., Dyck P.J.:Osteosclerotic Myeloma (POEMS syndrome), pp. 1288–1293. In: Peripheral neuropathy, Dick P.J., Thomas P.K., Griffin J.W., Low P.A., Poduslo J.F., eds. 3rd ed. Philadephia: WB Saunders, 1993.
Leger-Ravet M.B., Peuchmaur M., Devergne O., et al.:Interleukin-6 gene expression in Castleman's disease. Blood, 78:2923–2930, 1991.
Mandler R.N., Kerrigan D.P., Smart J., Kuis W., Villiger P., Lotz M.:Castleman's disease in POEMS syndrome with elevated interleukin-6. Cancer, 69:2697–2703, 1992.
Miralles G.D., O'Fallon J.R., Talley N.J.:Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N. Engl. J. Med., 327:1919–1923, 1992.
Nakanishi T., Sobue I., Toyokura Y., et al.:The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology, 34:712–720, 1984.
Reulecke M., Dumas M., Meier C.:Specific antibody activity against neuroendocrine tissue in a case of POEMS syndrome with IgG gammopathy. Neurology, 38:614–616, 1988.
Scully R.E., Mark E.J., McNeely W.F., McNeely B.U.:Case records of the Massachussetts General Hospital (Case 10-1987). N. Engl. J. Med., 316:606–618, 1987.
Scully R.E., Mark E.J., McNeely W.F., McNeely B.U.:Case Records of the Massachussetts General Hospital (Case 39-1992). N. Engl. J. Med., 327:1014–1021, 1992.
Sobue G., Doyu M., Watanabe M., Hayashi F., Mitsuma T.:Extensive demyelinating changes in the peripheral nerves of Crow-Fukase syndrome: a pathological study of one autopsied case. Acta Neuropathol., 84:171–177, 1992.
Yoshizaki K., Matsuda T., Nishimoto N., et al.:Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease. Blood, 74:1360–1367, 1989.
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Pareyson, D., Marazzi, R., Confalonieri, P. et al. The POEMS syndrome: Report of six cases. Ital J Neuro Sci 15, 353–358 (1994). https://doi.org/10.1007/BF02339932
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DOI: https://doi.org/10.1007/BF02339932