POEMS syndrome natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
POEMS syndrome presents as a chronic progressive polyneuropathy. Patients may have coexisting multi-organ system disorders. The neuropathy is usually symmetrical an ascending. Endocrinopathy, present in majority of cases, involves hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis. If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. Comlications of POEMS syndrome include renal failure, pulmonary hypertension, pathologic fractures, ischemic stroke, restrictive lung disease, polycythemia, thrombocytosis, papilledema, and myocardial infarction. 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%. Age >50 years, pulmonary hypertension, pleural effusion, and estimated glomerular filtration rate <30 ml/min/1.73 m<sup>2</sup> were associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model based on randomized sample splitting of 362 patients. Low albumin (defined as<3.2 g/dl) at diagnosis and failure to achieve a complete hematologic response to first-line therapy can be independent risk factors for progression-free survival (PFS). 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. | |||
== 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, may be IgA lambda component, IgG lambda component or light chain lambda component) 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. | * 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. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief:
Overview
POEMS syndrome presents as a chronic progressive polyneuropathy. Patients may have coexisting multi-organ system disorders. The neuropathy is usually symmetrical an ascending. Endocrinopathy, present in majority of cases, involves hypogonadism, hypothyroidism, and abnormalities of the pituitary-adrenal axis. If left untreated, patients suffering from POEMS syndrome may become wheel chair bound. Comlications of POEMS syndrome include renal failure, pulmonary hypertension, pathologic fractures, ischemic stroke, restrictive lung disease, polycythemia, thrombocytosis, papilledema, and myocardial infarction. 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%. Age >50 years, pulmonary hypertension, pleural effusion, and estimated glomerular filtration rate <30 ml/min/1.73 m2 were associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model based on randomized sample splitting of 362 patients. Low albumin (defined as<3.2 g/dl) at diagnosis and failure to achieve a complete hematologic response to first-line therapy can be independent risk factors for progression-free survival (PFS). 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.
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.
- 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%.[1]
- Age >50 years, pulmonary hypertension, pleural effusion, and estimated glomerular filtration rate <30 ml/min/1.73 m2 were associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model based on randomized sample splitting of 362 patients.[1][2][3][4][5]
- Low albumin (defined as<3.2 g/dl) at diagnosis and failure to achieve a complete hematologic response to first-line therapy can be independent risk factors for progression-free survival (PFS).[1][6]
- 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.
References
- ↑ 1.0 1.1 1.2 Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J (January 2017). "Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome". Leukemia. 31 (1): 100–106. doi:10.1038/leu.2016.168. PMID 27338259.
- ↑ Clegg A, Young J, Iliffe S, Rikkert MO, Rockwood K (March 2013). "Frailty in elderly people". Lancet. 381 (9868): 752–62. doi:10.1016/S0140-6736(12)62167-9. PMC 4098658. PMID 23395245.
- ↑ Lesprit P, Godeau B, Authier FJ, Soubrier M, Zuber M, Larroche C, Viard JP, Wechsler B, Gherardi R (March 1998). "Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines". Am. J. Respir. Crit. Care Med. 157 (3 Pt 1): 907–11. doi:10.1164/ajrccm.157.3.9707095. PMID 9517610.
- ↑ Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A (April 2008). "Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients". Chest. 133 (4): 969–74. doi:10.1378/chest.07-1800. PMID 18198255.
- ↑ Li J, Tian Z, Zheng HY, Zhang W, Duan MH, Liu YT, Cao XX, Zhou DB (March 2013). "Pulmonary hypertension in POEMS syndrome". Haematologica. 98 (3): 393–8. doi:10.3324/haematol.2012.073031. PMC 3659947. PMID 22983590.
- ↑ Kourelis TV, Buadi FK, Gertz MA, Lacy MQ, Kumar SK, Kapoor P, Go RS, Lust JA, Hayman SR, Rajkumar V, Zeldenrust SR, Russell SJ, Dingli D, Lin Y, Leung N, Hwa YL, Gonsalves W, Kyle RA, Dispenzieri A (May 2016). "Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment". Leukemia. 30 (5): 1079–85. doi:10.1038/leu.2015.344. PMID 26669974.