POEMS syndrome is a rare systemic multi-organ disease usually reported in Japanese patients. The
clinical course is slow with a 5-year survival of 60%. Death is caused by
polyneuropathy and or anasarka. We observed four cases in our ward (all males, aged 39, 57, 54 and 54 years) who all presented at least four characteristic clinical signs. Gynecomasty together with impotency was seen in all patients. All had
hypogonadism and borderline
hyperprolactinaemia. Hyperoestrogenism was seen in two. In three patients, rare endocrinopathies were part of the
POEMS syndrome. One patient had
diabetes mellitus. The
clinical course was variable and a function of the effectiveness of plasmocyte dyscrasia
therapy. Little work has been done on endocrinopathies in
POEMS syndrome. In most cases, gonadotrope function is impaired with gynecomasty and impotency in men, amenorrhoea in women. Generally the
hypogonadism is hypogonadotropic. Hyperoestrogenism is frequent and
prolactin levels are normal or high with an exaggerated response to thyroid releasing
hormone stimulation. The aetiology of
POEMS syndrome is unknown. Current research is based on an immunologic theory based on the discovery of high levels of
interleukin 6 in POEMS patients with or without
Castleman's disease. The
cytokine would affect the different organs and lead to clinical expression.
Corticosteroids are usually effective in most patients, particularly in reducing the oedema and controlling the
polyneuropathy.