[POEMS syndrome: report of a case and review of the literature].
A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castleman's disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.
Castro Ríos
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MA
Instituto de Investigaciones Médicas Dr. Alfredo Lanari, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Kniznik
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D004487:Edema / Q000175:diagnosis; D004700:Endocrine System Diseases / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010266:Paraproteins / Q000032:analysis; D010859:Pigmentation Disorders / Q000175:diagnosis*; D011115:Polyneuropathies / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D013577:Syndrome
D010266:Paraproteins; C008352:meprednisone; D011241:Prednisone
Síndrome de POEMS: presentación de un caso y revisión de la literatura.