POEMS Syndrome

POEMS syndrome (also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare medical syndrome. It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy, and effects on many other organ systems. It begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women. If untreated it is progressive and often fatal, with only 60% of sufferers remaining alive five years after onset. However, the symptoms can improve if the blood disorder is successfully treated.

The name 'POEMS syndrome', now the most prevalent, was coined in 1980 by Bardwick et al. The name is an acronym deriving from some of the main features: Polyneuropathy (peripheral nerve damage), Organomegaly (abnormal enlargement of organs), Endocrinopathy (damage to hormone-producing glands) or Edema, M-protein (an abnormal antibody) and Skin abnormalities (including hyperpigmentation and hypertrichosis). However, these features are neither an exhaustive list nor seen in all individuals with the condition.

Read more about POEMS Syndrome:  Signs and Symptoms, Criteria, Pathogenesis, Treatment, History

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