POEMS syndrome natural history, complications and prognosis: Difference between revisions
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== Natural History == | == Natural History == | ||
POEMS syndrome typically presents as a chronic progressive polyneuropathy with both sensory and motor disability, the motor component is usually present to a greater degree than the sensory component of the disease. Many patients may have coexisting multi-organ system disorders such as organomegaly, endocrinopathy (pituitary, thyroid, adrenals), thrombocytosis, anemia, an M-spike (M protein or para-protein) and skin changes. The skin changes include angiomata, hyperpigmentation | * POEMS syndrome typically presents as a chronic progressive polyneuropathy with both sensory and motor disability, the motor component is usually present to a greater degree than the sensory component of the disease. | ||
* Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenals), thrombocytosis, anemia, pulmonary hypertension, an M-spike (M protein or para-protein) and skin changes. The skin changes include angiomata, hyperpigmentation | |||
* The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset. | |||
* POEMS syndrome may also lead to glomerular disease eventually resulting in renal failure. | |||
* If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. | |||
== Complications == | == Complications == | ||
== Prognosis == | == Prognosis == | ||
* The median survival of patients with POEMS syndrome is 165 months. | |||
==References== | ==References== | ||
Revision as of 13:22, 4 February 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief:
Overview
Natural History
- POEMS syndrome typically presents as a chronic progressive polyneuropathy with both sensory and motor disability, the motor component is usually present to a greater degree than the sensory component of the disease.
- Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenals), thrombocytosis, anemia, pulmonary hypertension, an M-spike (M protein or para-protein) and skin changes. The skin changes include angiomata, hyperpigmentation
- The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset.
- POEMS syndrome may also lead to glomerular disease eventually resulting in renal failure.
- If left untreated, patients suffering from POEMS syndrome may become wheel chair bound.
Complications
Prognosis
- The median survival of patients with POEMS syndrome is 165 months.