POEMS Syndrome: a clinical challenge / Síndrome de POEMS: um desafio clínico
DOI:
https://doi.org/10.34119/bjhrv5n1-232Keywords:
POEMS syndrome, chronic inflammatory demyelinating polyneuropathy, hypergammaglobulinemiaAbstract
A 42-year-old male patient admitted to our service with a history of progressive and symmetrical weakness, paresthesia of the lower limbs, weight loss and urinary retention for about 8 months. Physical examination demonstrated poor general condition, lower limb edema, hepatomegaly and digital clubbing. Neurological examination showed a polyneuropathic pattern, with hypoesthesia and absence of deep reflexes of the lower limbs. Initial laboratory investigation demonstrated erythrocytosis and thyroid and adrenal dysfunction in addition to hypergammaglobulinemia. CSF analysis, due to the clinical suspicion of chronic inflammatory demyelinating polyneuropathy, resulted in protein-cytological dissociation. PIDC was hypothesized to be due to light-chain disease, but for this association we supplemented with bone marrow analysis and whole-body CT scan, which showed 4% atypia in plasma cells and osteolytic and osteoblastic lesions in the vertebro-costal region between the 8th and 9th vertebrae. Subsequent biopsy confirmed plasmacytoma. He performed autologous bone marrow transplantation, but evolved with infectious complications and died within 3 months.
References
Bardwick PA, Zvaifler NJ, Gill GN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine (Baltimore) 1980; 59:311
Chang Y, Cesarman E, Pessin MS, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi's sarcoma. Science 1994; 266:1865.
Gherardi RK, Bélec L, Soubrier M, et al. Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. Blood 1996; 87:1458.
Hashiguchi T, Arimura K, Matsumuro K, et al. Highly concentrated vascular endothelial growth factor in platelets in Crow-Fukase syndrome. Muscle Nerve 2000; 23:1051
Endo I, Mitsui T, Nishino M, et al. Diurnal fluctuation of edema synchronized with plasma VEGF concentration in a patient with POEMS syndrome. Intern Med 2002; 41:1196.
Shinde A, Matsumae H, Maruyama A, et al. [A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma]. Rinsho Shinkeigaku 2001; 41:121.
Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998; 21:1390.
Valenzuela F, Gajardo MF, Gutiérrez DM, Herrera PA. Síndrome de POEMS. Piel. Formación continuada en dermatología. 2012; 27:453-5.
