POEMS syndrome natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
POEMS [[syndrome]] presents as a [[Chronic (medical)|chronic]] progressive [[polyneuropathy]]. [[Patient|Patients]] may have coexisting multi-[[Organ (anatomy)|organ]] system [[Disorder (medicine)|disorders]]. The [[neuropathy]] is usually symmetrical and ascending. [[Endocrinology|Endocrinopathy]], present in majority of cases, involves [[hypogonadism]], [[hypothyroidism]], and abnormalities of the [[Pituitary gland|pituitary]]-[[Adrenal gland|adrenal]] axis. If left untreated, [[patients]] suffering from POEMS [[syndrome]] may become wheel chair bound. [[Complication (medicine)|Complications]] of POEMS [[syndrome]] include [[Renal insufficiency|renal failure]], [[pulmonary hypertension]], [[Fracture|pathologic fractures]], [[ischemic stroke]], [[restrictive lung disease]], [[polycythemia]], [[thrombocytosis]], [[papilledema]], and [[myocardial infarction]]. 3, 5, and 10 year overall survival (OS) for [[Patient|patients]] newly [[Diagnosis|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 [[Hematology|hematologic]] response to [[First-line treatment|first-line therapy]] can be independent [[risk factors]] for progression-free survival (PFS). Improvement of [[Plasma cell disorder|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]]. | POEMS [[syndrome]] presents as a [[Chronic (medical)|chronic]] progressive [[polyneuropathy]]. [[Patient|Patients]] may have coexisting multi-[[Organ (anatomy)|organ]] system [[Disorder (medicine)|disorders]]. The [[neuropathy]] is usually symmetrical and ascending. [[Endocrinology|Endocrinopathy]], present in majority of cases, involves [[hypogonadism]], [[hypothyroidism]], and abnormalities of the [[Pituitary gland|pituitary]]-[[Adrenal gland|adrenal]] axis. If left untreated, [[patients]] suffering from POEMS [[syndrome]] may become wheel chair bound. [[Complication (medicine)|Complications]] of POEMS [[syndrome]] include [[Renal insufficiency|renal failure]], [[pulmonary hypertension]], [[Fracture|pathologic fractures]], [[ischemic stroke]], [[restrictive lung disease]], [[polycythemia]], [[thrombocytosis]], [[papilledema]], and [[myocardial infarction]]. 3, 5, and 10 year overall survival (OS) for [[Patient|patients]] newly [[Diagnosis|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 [[Hematology|hematologic]] response to [[First-line treatment|first-line therapy]] can be independent [[risk factors]] for progression-free survival (PFS). Improvement of [[Plasma cell disorder|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, Complications, and Prognosis == | ||
* POEMS [[syndrome]] typically presents as a [[Chronic (medical)|chronic]] progressive [[polyneuropathy]] with both [[Sensory system|sensory]] and motor disability, the motor component is usually present to a greater degree than the [[Sensory system|sensory]] component of the [[disease]].<ref name="pmid29157616">{{cite journal |vauthors=Mauermann ML |title=The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease |journal=Hematol. Oncol. Clin. North Am. |volume=32 |issue=1 |pages=153–163 |date=February 2018 |pmid=29157616 |doi=10.1016/j.hoc.2017.09.012 |url=}}</ref> | |||
=== Natural History === | |||
*POEMS [[syndrome]] typically presents as a [[Chronic (medical)|chronic]] progressive [[polyneuropathy]] with both [[Sensory system|sensory]] and motor disability, the motor component is usually present to a greater degree than the [[Sensory system|sensory]] component of the [[disease]].<ref name="pmid29157616">{{cite journal |vauthors=Mauermann ML |title=The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease |journal=Hematol. Oncol. Clin. North Am. |volume=32 |issue=1 |pages=153–163 |date=February 2018 |pmid=29157616 |doi=10.1016/j.hoc.2017.09.012 |url=}}</ref> | |||
* Many [[Patient|patients]] may have coexisting multi-[[Organ (anatomy)|organ]] system [[Disorder (medicine)|disorders]] such as [[organomegaly]] ([[liver]], [[spleen]] and [[Lymph node|lymph nodes]]), [[edema]], [[Endocrinology|endocrinopathy]] ([[Pituitary gland|pituitary]], [[thyroid]], [[Adrenal gland|adrenal glands]]), [[thrombocytosis]], [[polycythemia]], [[pulmonary hypertension]], an M-spike ([[M protein]] or para-[[protein]], may be [[Immunoglobulin A|IgA]] lambda component, [[Immunoglobulin G|IgG]] lambda component or [[light chain]] lambda component) and [[skin]] changes. The [[skin]] changes include [[Angiomatosis|angiomata]], [[hyperpigmentation]], [[hypertrichosis]].<ref name="pmid22806697">{{cite journal |vauthors=Dispenzieri A |title=POEMS syndrome: update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=87 |issue=8 |pages=804–14 |date=August 2012 |pmid=22806697 |doi=10.1002/ajh.23288 |url=}}</ref> | * Many [[Patient|patients]] may have coexisting multi-[[Organ (anatomy)|organ]] system [[Disorder (medicine)|disorders]] such as [[organomegaly]] ([[liver]], [[spleen]] and [[Lymph node|lymph nodes]]), [[edema]], [[Endocrinology|endocrinopathy]] ([[Pituitary gland|pituitary]], [[thyroid]], [[Adrenal gland|adrenal glands]]), [[thrombocytosis]], [[polycythemia]], [[pulmonary hypertension]], an M-spike ([[M protein]] or para-[[protein]], may be [[Immunoglobulin A|IgA]] lambda component, [[Immunoglobulin G|IgG]] lambda component or [[light chain]] lambda component) and [[skin]] changes. The [[skin]] changes include [[Angiomatosis|angiomata]], [[hyperpigmentation]], [[hypertrichosis]].<ref name="pmid22806697">{{cite journal |vauthors=Dispenzieri A |title=POEMS syndrome: update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=87 |issue=8 |pages=804–14 |date=August 2012 |pmid=22806697 |doi=10.1002/ajh.23288 |url=}}</ref> | ||
* The [[neuropathy]] is usually symmetrical and ascending, with either insidious or rapidly progressing onset. [[Neuropathy|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. [[Neuropathy|Sensory neuropathy]] precedes motor dysfunction and pin-prick and vibratory sensations are typically affected. | ||
* The [[Endocrinology|endocrinopathy]] is present in a majority of cases of POEMS [[syndrome]]. Almost all [[Patient|patients]] exhibiting [[Endocrinology|endocrinopathy]] have [[Estrogen|hyperestrogenemia]]. The increased levels of [[estrogen]] may be the cause of [[hypogonadism]] and [[Erectile dysfunction|impotence]] seen in [[male]] [[Patient|patients]]. [[Hypogonadism]], [[hypothyroidism]], and abnormalities of the [[Pituitary gland|pituitary]]-[[Adrenal gland|adrenal]] axis are noted in order of descending frequency.<ref name="pmid17605964">{{cite journal |vauthors=Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD |title=Endocrinopathy in POEMS syndrome: the Mayo Clinic experience |journal=Mayo Clin. Proc. |volume=82 |issue=7 |pages=836–42 |date=July 2007 |pmid=17605964 |doi=10.4065/82.7.836 |url=}}</ref><ref name="pmid30239770">{{cite journal |vauthors=Caimari F, Keddie S, Lunn MP, D'Sa S, Baldeweg SE |title=Prevalence and course of endocrinopathy in POEMS syndrome |journal=J. Clin. Endocrinol. Metab. |volume= |issue= |pages= |date=September 2018 |pmid=30239770 |doi=10.1210/jc.2018-01516 |url=}}</ref> | * The [[Endocrinology|endocrinopathy]] is present in a majority of cases of POEMS [[syndrome]]. Almost all [[Patient|patients]] exhibiting [[Endocrinology|endocrinopathy]] have [[Estrogen|hyperestrogenemia]]. The increased levels of [[estrogen]] may be the cause of [[hypogonadism]] and [[Erectile dysfunction|impotence]] seen in [[male]] [[Patient|patients]]. [[Hypogonadism]], [[hypothyroidism]], and abnormalities of the [[Pituitary gland|pituitary]]-[[Adrenal gland|adrenal]] axis are noted in order of descending frequency.<ref name="pmid17605964">{{cite journal |vauthors=Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD |title=Endocrinopathy in POEMS syndrome: the Mayo Clinic experience |journal=Mayo Clin. Proc. |volume=82 |issue=7 |pages=836–42 |date=July 2007 |pmid=17605964 |doi=10.4065/82.7.836 |url=}}</ref><ref name="pmid30239770">{{cite journal |vauthors=Caimari F, Keddie S, Lunn MP, D'Sa S, Baldeweg SE |title=Prevalence and course of endocrinopathy in POEMS syndrome |journal=J. Clin. Endocrinol. Metab. |volume= |issue= |pages= |date=September 2018 |pmid=30239770 |doi=10.1210/jc.2018-01516 |url=}}</ref> | ||
* POEMS [[syndrome]] may also lead to [[glomerular disease]] ([[membranoproliferative glomerulonephritis]]) eventually resulting in [[Renal insufficiency|renal failure]]. | * POEMS [[syndrome]] may also lead to [[glomerular disease]] ([[membranoproliferative glomerulonephritis]]) eventually resulting in [[Renal insufficiency|renal failure]]. | ||
* If left untreated, [[Patient|patients]] suffering from POEMS [[syndrome]] may become wheel chair bound. | * If left untreated, [[Patient|patients]] suffering from POEMS [[syndrome]] may become wheel chair bound. | ||
=== Complications === | |||
*[[Patient|Patients]] suffering from POEMS [[syndrome]] may develop the following [[Complication (medicine)|complications]]: | |||
* [[Patient|Patients]] suffering from POEMS [[syndrome]] may develop the following [[Complication (medicine)|complications]]: | **[[Renal insufficiency|Renal failure]]<ref name="pmid7634552">{{cite journal |vauthors=Modesto-Segonds A, Rey JP, Orfila C, Huchard G, Suc JM |title=Renal involvement in POEMS syndrome |journal=Clin. Nephrol. |volume=43 |issue=5 |pages=342–5 |date=May 1995 |pmid=7634552 |doi= |url=}}</ref> | ||
** [[Renal insufficiency|Renal failure]]<ref name="pmid7634552">{{cite journal |vauthors=Modesto-Segonds A, Rey JP, Orfila C, Huchard G, Suc JM |title=Renal involvement in POEMS syndrome |journal=Clin. Nephrol. |volume=43 |issue=5 |pages=342–5 |date=May 1995 |pmid=7634552 |doi= |url=}}</ref> | **[[Pulmonary hypertension]]<ref name="pmid25610609">{{cite journal |vauthors=Brewis MJ, Church AC, Peacock AJ, Thomson S, Tighe J, Johnson MK |title=Pulmonary hypertension in POEMS syndrome: resolution following radiotherapy |journal=Pulm Circ |volume=4 |issue=4 |pages=732–5 |date=December 2014 |pmid=25610609 |doi=10.1086/678553 |url=}}</ref> | ||
** [[Pulmonary hypertension]]<ref name="pmid25610609">{{cite journal |vauthors=Brewis MJ, Church AC, Peacock AJ, Thomson S, Tighe J, Johnson MK |title=Pulmonary hypertension in POEMS syndrome: resolution following radiotherapy |journal=Pulm Circ |volume=4 |issue=4 |pages=732–5 |date=December 2014 |pmid=25610609 |doi=10.1086/678553 |url=}}</ref> | **[[Fractures|Pathological fractures]] (due to lytic/[[Sclerosis|sclerotic]] [[bone]] [[Lesion|lesions]])<ref name="pmid21720058">{{cite journal |vauthors=Shibuya K, Misawa S, Horikoshi T, Kanai K, Isose S, Nasu S, Sekiguchi Y, Noto Y, Fujimaki Y, Nakaseko C, Kuwabara S |title=Detection of bone lesions by CT in POEMS syndrome |journal=Intern. Med. |volume=50 |issue=13 |pages=1393–6 |date=2011 |pmid=21720058 |doi= |url=}}</ref> | ||
** [[Fractures|Pathological fractures]] (due to lytic/[[Sclerosis|sclerotic]] [[bone]] [[Lesion|lesions]])<ref name="pmid21720058">{{cite journal |vauthors=Shibuya K, Misawa S, Horikoshi T, Kanai K, Isose S, Nasu S, Sekiguchi Y, Noto Y, Fujimaki Y, Nakaseko C, Kuwabara S |title=Detection of bone lesions by CT in POEMS syndrome |journal=Intern. Med. |volume=50 |issue=13 |pages=1393–6 |date=2011 |pmid=21720058 |doi= |url=}}</ref> | **[[Ischemic stroke]]<ref name="pmid29179528">{{cite journal |vauthors=Fu FW, Rao J, Zheng YY, Wang HL, Yang JG, Zheng GQ |title=Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature |journal=Oncotarget |volume=8 |issue=51 |pages=89406–89424 |date=October 2017 |pmid=29179528 |doi=10.18632/oncotarget.20131 |url=}}</ref> | ||
** [[Ischemic stroke]]<ref name="pmid29179528">{{cite journal |vauthors=Fu FW, Rao J, Zheng YY, Wang HL, Yang JG, Zheng GQ |title=Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature |journal=Oncotarget |volume=8 |issue=51 |pages=89406–89424 |date=October 2017 |pmid=29179528 |doi=10.18632/oncotarget.20131 |url=}}</ref> | **[[Restrictive lung disease]]<ref name="pmid18198255">{{cite journal |vauthors=Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A |title=Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients |journal=Chest |volume=133 |issue=4 |pages=969–74 |date=April 2008 |pmid=18198255 |doi=10.1378/chest.07-1800 |url=}}</ref> | ||
** [[Restrictive lung disease]]<ref name="pmid18198255">{{cite journal |vauthors=Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A |title=Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients |journal=Chest |volume=133 |issue=4 |pages=969–74 |date=April 2008 |pmid=18198255 |doi=10.1378/chest.07-1800 |url=}}</ref> | **[[Thrombocytosis]]<ref name="pmid26179010">{{cite journal |vauthors=Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML |title=Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy |journal=Muscle Nerve |volume=52 |issue=4 |pages=658–9 |date=October 2015 |pmid=26179010 |doi=10.1002/mus.24768 |url=}}</ref> | ||
** [[Thrombocytosis]]<ref name="pmid26179010">{{cite journal |vauthors=Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML |title=Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy |journal=Muscle Nerve |volume=52 |issue=4 |pages=658–9 |date=October 2015 |pmid=26179010 |doi=10.1002/mus.24768 |url=}}</ref> | |||
**[[Artery|Arterial]] and [[Vein|venous]] [[Thrombosis|thromboses]]<ref name="pmid29288001">{{cite journal |vauthors=Decker P, Galland J, Risse J, Claudin M, Mohamed S, Perrot A, Wahl D |title=[A POEMS syndrome revealed by a blue toe syndrome] |language=French |journal=Rev Med Interne |volume=39 |issue=2 |pages=127–129 |date=February 2018 |pmid=29288001 |doi=10.1016/j.revmed.2017.09.005 |url=}}</ref> | **[[Artery|Arterial]] and [[Vein|venous]] [[Thrombosis|thromboses]]<ref name="pmid29288001">{{cite journal |vauthors=Decker P, Galland J, Risse J, Claudin M, Mohamed S, Perrot A, Wahl D |title=[A POEMS syndrome revealed by a blue toe syndrome] |language=French |journal=Rev Med Interne |volume=39 |issue=2 |pages=127–129 |date=February 2018 |pmid=29288001 |doi=10.1016/j.revmed.2017.09.005 |url=}}</ref> | ||
** [[Polycythemia]]<ref name="pmid2537136">{{cite journal |vauthors=Schulz W, Domenico D, Nand S |title=POEMS syndrome associated with polycythemia vera |journal=Cancer |volume=63 |issue=6 |pages=1175–8 |date=March 1989 |pmid=2537136 |doi= |url=}}</ref> | **[[Polycythemia]]<ref name="pmid2537136">{{cite journal |vauthors=Schulz W, Domenico D, Nand S |title=POEMS syndrome associated with polycythemia vera |journal=Cancer |volume=63 |issue=6 |pages=1175–8 |date=March 1989 |pmid=2537136 |doi= |url=}}</ref> | ||
** [[Papilledema]]<ref name="pmid26634133">{{cite journal |vauthors=Kumar S, Sharma S |title=Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome |journal=Oxf Med Case Reports |volume=2015 |issue=3 |pages=237–40 |date=March 2015 |pmid=26634133 |doi=10.1093/omcr/omv023 |url=}}</ref> | **[[Papilledema]]<ref name="pmid26634133">{{cite journal |vauthors=Kumar S, Sharma S |title=Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome |journal=Oxf Med Case Reports |volume=2015 |issue=3 |pages=237–40 |date=March 2015 |pmid=26634133 |doi=10.1093/omcr/omv023 |url=}}</ref> | ||
** [[Myocardial infarction]]<ref name="pmid1295088">{{cite journal |vauthors=Manning WJ, Goldberger AL, Drews RE, Goldstein BJ, Matheson JK, Rabinowe SL, Trentham DE, Landsberg L |title=POEMS syndrome with myocardial infarction: observations concerning pathogenesis and review of the literature |journal=Semin. Arthritis Rheum. |volume=22 |issue=3 |pages=151–61 |date=December 1992 |pmid=1295088 |doi= |url=}}</ref> | **[[Myocardial infarction]]<ref name="pmid1295088">{{cite journal |vauthors=Manning WJ, Goldberger AL, Drews RE, Goldstein BJ, Matheson JK, Rabinowe SL, Trentham DE, Landsberg L |title=POEMS syndrome with myocardial infarction: observations concerning pathogenesis and review of the literature |journal=Semin. Arthritis Rheum. |volume=22 |issue=3 |pages=151–61 |date=December 1992 |pmid=1295088 |doi= |url=}}</ref> | ||
=== Prognosis === | |||
* The [[median]] survival of [[Patient|patients]] with POEMS [[syndrome]] is 165 months.<ref name="pmid12456500">{{cite journal |vauthors=Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA |title=POEMS syndrome: definitions and long-term outcome |journal=Blood |volume=101 |issue=7 |pages=2496–506 |date=April 2003 |pmid=12456500 |doi=10.1182/blood-2002-07-2299 |url=}}</ref> | * The [[median]] survival of [[Patient|patients]] with POEMS [[syndrome]] is 165 months.<ref name="pmid12456500">{{cite journal |vauthors=Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA |title=POEMS syndrome: definitions and long-term outcome |journal=Blood |volume=101 |issue=7 |pages=2496–506 |date=April 2003 |pmid=12456500 |doi=10.1182/blood-2002-07-2299 |url=}}</ref> | ||
*3, 5, and 10 year overall survival (OS) for [[Patient|patients]] newly [[Diagnosis|diagnosed]] with POEMS [[syndrome]] is 87.7%, 84.1%, and 77%.<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref> | *3, 5, and 10 year overall survival (OS) for [[Patient|patients]] newly [[Diagnosis|diagnosed]] with POEMS [[syndrome]] is 87.7%, 84.1%, and 77%.<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref> | ||
*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 [[Patient|patients]].<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref><ref name="pmid23395245">{{cite journal |vauthors=Clegg A, Young J, Iliffe S, Rikkert MO, Rockwood K |title=Frailty in elderly people |journal=Lancet |volume=381 |issue=9868 |pages=752–62 |date=March 2013 |pmid=23395245 |pmc=4098658 |doi=10.1016/S0140-6736(12)62167-9 |url=}}</ref><ref name="pmid9517610">{{cite journal |vauthors=Lesprit P, Godeau B, Authier FJ, Soubrier M, Zuber M, Larroche C, Viard JP, Wechsler B, Gherardi R |title=Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines |journal=Am. J. Respir. Crit. Care Med. |volume=157 |issue=3 Pt 1 |pages=907–11 |date=March 1998 |pmid=9517610 |doi=10.1164/ajrccm.157.3.9707095 |url=}}</ref><ref name="pmid18198255">{{cite journal |vauthors=Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A |title=Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients |journal=Chest |volume=133 |issue=4 |pages=969–74 |date=April 2008 |pmid=18198255 |doi=10.1378/chest.07-1800 |url=}}</ref><ref name="pmid22983590">{{cite journal |vauthors=Li J, Tian Z, Zheng HY, Zhang W, Duan MH, Liu YT, Cao XX, Zhou DB |title=Pulmonary hypertension in POEMS syndrome |journal=Haematologica |volume=98 |issue=3 |pages=393–8 |date=March 2013 |pmid=22983590 |pmc=3659947 |doi=10.3324/haematol.2012.073031 |url=}}</ref> | *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 [[Patient|patients]].<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref><ref name="pmid23395245">{{cite journal |vauthors=Clegg A, Young J, Iliffe S, Rikkert MO, Rockwood K |title=Frailty in elderly people |journal=Lancet |volume=381 |issue=9868 |pages=752–62 |date=March 2013 |pmid=23395245 |pmc=4098658 |doi=10.1016/S0140-6736(12)62167-9 |url=}}</ref><ref name="pmid9517610">{{cite journal |vauthors=Lesprit P, Godeau B, Authier FJ, Soubrier M, Zuber M, Larroche C, Viard JP, Wechsler B, Gherardi R |title=Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines |journal=Am. J. Respir. Crit. Care Med. |volume=157 |issue=3 Pt 1 |pages=907–11 |date=March 1998 |pmid=9517610 |doi=10.1164/ajrccm.157.3.9707095 |url=}}</ref><ref name="pmid18198255">{{cite journal |vauthors=Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A |title=Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients |journal=Chest |volume=133 |issue=4 |pages=969–74 |date=April 2008 |pmid=18198255 |doi=10.1378/chest.07-1800 |url=}}</ref><ref name="pmid22983590">{{cite journal |vauthors=Li J, Tian Z, Zheng HY, Zhang W, Duan MH, Liu YT, Cao XX, Zhou DB |title=Pulmonary hypertension in POEMS syndrome |journal=Haematologica |volume=98 |issue=3 |pages=393–8 |date=March 2013 |pmid=22983590 |pmc=3659947 |doi=10.3324/haematol.2012.073031 |url=}}</ref> | ||
*Low [[albumin]] (defined as<3.2 g/dl) at [[diagnosis]] and failure to achieve a complete [[Hematology|hematologic]] response to [[First-line treatment|first-line therapy]] can be independent [[Risk factor|risk factors]] for progression-free survival (PFS).<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref><ref name="pmid26669974">{{cite journal |vauthors=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 |title=Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment |journal=Leukemia |volume=30 |issue=5 |pages=1079–85 |date=May 2016 |pmid=26669974 |doi=10.1038/leu.2015.344 |url=}}</ref> | *Low [[albumin]] (defined as<3.2 g/dl) at [[diagnosis]] and failure to achieve a complete [[Hematology|hematologic]] response to [[First-line treatment|first-line therapy]] can be independent [[Risk factor|risk factors]] for progression-free survival (PFS).<ref name="pmid27338259">{{cite journal |vauthors=Wang C, Huang XF, Cai QQ, Cao XX, Duan MH, Cai H, Zhou DB, Li J |title=Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome |journal=Leukemia |volume=31 |issue=1 |pages=100–106 |date=January 2017 |pmid=27338259 |doi=10.1038/leu.2016.168 |url=}}</ref><ref name="pmid26669974">{{cite journal |vauthors=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 |title=Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment |journal=Leukemia |volume=30 |issue=5 |pages=1079–85 |date=May 2016 |pmid=26669974 |doi=10.1038/leu.2015.344 |url=}}</ref> | ||
*[[Papilledema]], [[DLCO|decreased diffusing capacity of the lung for carbon monoxide (DLCO)]] and treatment with [[Corticosteroid|corticosteroids]] alone are also related to poor [[prognosis]].<ref name="pmid24141729">{{cite journal |vauthors=Cui R, Yu S, Huang X, Zhang J, Tian C, Pu C |title=Papilloedema is an independent prognostic factor for POEMS syndrome |journal=J. Neurol. |volume=261 |issue=1 |pages=60–5 |date=January 2014 |pmid=24141729 |doi=10.1007/s00415-013-7143-4 |url=}}</ref> | *[[Papilledema]], [[DLCO|decreased diffusing capacity of the lung for carbon monoxide (DLCO)]] and treatment with [[Corticosteroid|corticosteroids]] alone are also related to poor [[prognosis]].<ref name="pmid24141729">{{cite journal |vauthors=Cui R, Yu S, Huang X, Zhang J, Tian C, Pu C |title=Papilloedema is an independent prognostic factor for POEMS syndrome |journal=J. Neurol. |volume=261 |issue=1 |pages=60–5 |date=January 2014 |pmid=24141729 |doi=10.1007/s00415-013-7143-4 |url=}}</ref> | ||
* Improvement of [[Plasma cell disorder|plasma cell dyscrasia]] leads to improvement or marked reduction in other abnormalities. | * Improvement of [[Plasma cell disorder|plasma cell dyscrasia]] leads to improvement or marked reduction in other abnormalities. | ||
* [[Neuropathy]], [[stroke]] and [[myocardial infarction]] are the most common [[Causality|causes]] of death in POEMS [[syndrome]]. | *[[Neuropathy]], [[stroke]] and [[myocardial infarction]] are the most common [[Causality|causes]] of death in POEMS [[syndrome]]. | ||
==References== | ==References== | ||
Latest revision as of 13:03, 9 August 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Sabawoon Mirwais, M.B.B.S, M.D.[3]
Overview
POEMS syndrome presents as a chronic progressive polyneuropathy. Patients may have coexisting multi-organ system disorders. The neuropathy is usually symmetrical and 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. Complications 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, Complications, and Prognosis
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.[1]
- Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenal glands), 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.[2]
- 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 endocrinopathy 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.[3][4]
- 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:
Prognosis
- The median survival of patients with POEMS syndrome is 165 months.[15]
- 3, 5, and 10 year overall survival (OS) for patients newly diagnosed with POEMS syndrome is 87.7%, 84.1%, and 77%.[16]
- 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.[16][17][18][9][19]
- 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).[16][20]
- Papilledema, decreased diffusing capacity of the lung for carbon monoxide (DLCO) and treatment with corticosteroids alone are also related to poor prognosis.[21]
- 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
- ↑ Mauermann ML (February 2018). "The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease". Hematol. Oncol. Clin. North Am. 32 (1): 153–163. doi:10.1016/j.hoc.2017.09.012. PMID 29157616.
- ↑ Dispenzieri A (August 2012). "POEMS syndrome: update on diagnosis, risk-stratification, and management". Am. J. Hematol. 87 (8): 804–14. doi:10.1002/ajh.23288. PMID 22806697.
- ↑ Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD (July 2007). "Endocrinopathy in POEMS syndrome: the Mayo Clinic experience". Mayo Clin. Proc. 82 (7): 836–42. doi:10.4065/82.7.836. PMID 17605964.
- ↑ Caimari F, Keddie S, Lunn MP, D'Sa S, Baldeweg SE (September 2018). "Prevalence and course of endocrinopathy in POEMS syndrome". J. Clin. Endocrinol. Metab. doi:10.1210/jc.2018-01516. PMID 30239770.
- ↑ Modesto-Segonds A, Rey JP, Orfila C, Huchard G, Suc JM (May 1995). "Renal involvement in POEMS syndrome". Clin. Nephrol. 43 (5): 342–5. PMID 7634552.
- ↑ Brewis MJ, Church AC, Peacock AJ, Thomson S, Tighe J, Johnson MK (December 2014). "Pulmonary hypertension in POEMS syndrome: resolution following radiotherapy". Pulm Circ. 4 (4): 732–5. doi:10.1086/678553. PMID 25610609.
- ↑ Shibuya K, Misawa S, Horikoshi T, Kanai K, Isose S, Nasu S, Sekiguchi Y, Noto Y, Fujimaki Y, Nakaseko C, Kuwabara S (2011). "Detection of bone lesions by CT in POEMS syndrome". Intern. Med. 50 (13): 1393–6. PMID 21720058.
- ↑ Fu FW, Rao J, Zheng YY, Wang HL, Yang JG, Zheng GQ (October 2017). "Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature". Oncotarget. 8 (51): 89406–89424. doi:10.18632/oncotarget.20131. PMID 29179528.
- ↑ 9.0 9.1 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.
- ↑ Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML (October 2015). "Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy". Muscle Nerve. 52 (4): 658–9. doi:10.1002/mus.24768. PMID 26179010.
- ↑ Decker P, Galland J, Risse J, Claudin M, Mohamed S, Perrot A, Wahl D (February 2018). "[A POEMS syndrome revealed by a blue toe syndrome]". Rev Med Interne (in French). 39 (2): 127–129. doi:10.1016/j.revmed.2017.09.005. PMID 29288001.
- ↑ Schulz W, Domenico D, Nand S (March 1989). "POEMS syndrome associated with polycythemia vera". Cancer. 63 (6): 1175–8. PMID 2537136.
- ↑ Kumar S, Sharma S (March 2015). "Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome". Oxf Med Case Reports. 2015 (3): 237–40. doi:10.1093/omcr/omv023. PMID 26634133.
- ↑ Manning WJ, Goldberger AL, Drews RE, Goldstein BJ, Matheson JK, Rabinowe SL, Trentham DE, Landsberg L (December 1992). "POEMS syndrome with myocardial infarction: observations concerning pathogenesis and review of the literature". Semin. Arthritis Rheum. 22 (3): 151–61. PMID 1295088.
- ↑ Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA (April 2003). "POEMS syndrome: definitions and long-term outcome". Blood. 101 (7): 2496–506. doi:10.1182/blood-2002-07-2299. PMID 12456500.
- ↑ 16.0 16.1 16.2 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.
- ↑ 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.
- ↑ Cui R, Yu S, Huang X, Zhang J, Tian C, Pu C (January 2014). "Papilloedema is an independent prognostic factor for POEMS syndrome". J. Neurol. 261 (1): 60–5. doi:10.1007/s00415-013-7143-4. PMID 24141729.