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. | * 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, polycythemia, pulmonary hypertension, an M-spike (M protein or para-protein) and skin changes. The skin changes include angiomata, hyperpigmentation, hypertrichosis | * Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenals), thrombocytosis, polycythemia, pulmonary hypertension, an M-spike (M protein or para-protein, may be IgA lambda component, IgG lambda component or light chain lambda component) and skin changes. The skin changes include angiomata, hyperpigmentation, hypertrichosis | ||
* The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset. Sensory neuropathy precedes motor dysfunction and pin-prick and vibratory sensations are typically affected. | * The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset. Sensory neuropathy precedes motor dysfunction and pin-prick and vibratory sensations are typically affected. | ||
* The endrocrinopathy is present in a majority of cases of POEMS syndrome. Almost all patients exhibiting endocrinopathy have hyperestrogenemia. The increased levels of estrogen may be the cause of hypogonadism and impotence seen in male patients. Hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis are noted in order of descending frequency. | |||
* POEMS syndrome may also lead to glomerular disease (membranoproliferative glomerulonephritis) 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. | ||
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** Thrombocytosis | ** Thrombocytosis | ||
** Polycythemia | ** Polycythemia | ||
** Papilledema | |||
** Myocardial infarction | ** Myocardial infarction | ||
== Prognosis == | == Prognosis == | ||
* The median survival of patients with POEMS syndrome is 165 months. | * The median survival of patients with POEMS syndrome is 165 months. | ||
* Improvement of plasma cell dyscrasia leads to improvement or marked reduction in other abnormalities. | |||
* Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome. | * Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome. | ||
Revision as of 17:49, 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, polycythemia, pulmonary hypertension, an M-spike (M protein or para-protein, may be IgA lambda component, IgG lambda component or light chain lambda component) and skin changes. The skin changes include angiomata, hyperpigmentation, hypertrichosis
- The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset. Sensory neuropathy precedes motor dysfunction and pin-prick and vibratory sensations are typically affected.
- The endrocrinopathy is present in a majority of cases of POEMS syndrome. Almost all patients exhibiting endocrinopathy have hyperestrogenemia. The increased levels of estrogen may be the cause of hypogonadism and impotence seen in male patients. Hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis are noted in order of descending frequency.
- 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
- Patients suffering from POEMS syndrome may develop the following complications:
- Renal failure
- Pulmonary hypertension
- Pathological fractures (due to lytuc/sclerotic bone lesions)
- Ischemic stroke
- Restrictive lung disease
- Thrombocytosis
- Polycythemia
- Papilledema
- Myocardial infarction
Prognosis
- The median survival of patients with POEMS syndrome is 165 months.
- Improvement of plasma cell dyscrasia leads to improvement or marked reduction in other abnormalities.
- Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome.