POEMS syndrome natural history, complications and prognosis: Difference between revisions
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* 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 | * 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. | * 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. | * POEMS syndrome may also lead to glomerular disease (membranoproliferative glomerulonephritis) eventually resulting in renal failure. | ||
* If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. | * If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. | ||
| Line 15: | Line 15: | ||
== Prognosis == | == Prognosis == | ||
* The median survival of patients with POEMS syndrome is 165 months. | * The median survival of patients with POEMS syndrome is 165 months. | ||
* Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome. | |||
==References== | ==References== | ||
Revision as of 15:46, 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 (membranoproliferative glomerulonephritis) 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.
- Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome.