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{{CMG}}
__NOTOC__
{{POEMS syndrome}}
{{CMG}} {{AE}} {{Sab}} {{HK}}


{{SI}}
{{SK}} Crow-Fukase syndrome; Takatsuki disease; PEP syndrome


==[[POEMS syndrome overview|Overview]]==


==[[POEMS syndrome historical perspective|Historical Perspective]]==


'''''Synonyms and Key Words:''''' ''Crow-Fukase syndrome, Takatsuki disease'', or ''PEP syndrome''
==[[POEMS syndrome pathophysiology|Pathophysiology]]==


==Overview==
==[[POEMS syndrome causes|Causes]]==
'''POEMS syndrome''' is a [[rare disease|rare]] medical [[syndrome]] named for its main clinically recognizable features: [[polyneuropathy|'''P'''olyneuropathy]] (peripheral nerve damage), [[organomegaly|'''O'''rganomegaly]] (abnormal enlargement of organs), [[Endocrinology|'''E'''ndocrinopathy]] (damage to hormone-producing glands)/[[edema|'''E'''dema]], [[paraprotein|'''M'''-protein]] (an abnormal antibody) and '''S'''kin abnormalities (including [[hyperpigmentation]] and [[hypertrichosis]]).


==Pathophysiology==
==[[POEMS syndrome differential diagnosis|Differentiating POEMS syndrome from other Diseases]]==
While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various [[cytokine]]s produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically [[IL1B|interleukin 1β]], [[interleukin 6]] and [[TNFα]]. There also seems to be a role of [[vascular endothelial growth factor]], given the [[angiogenesis|angiogenetic]] changes in many tissues.<ref name=Dispenzieri />
 
==[[POEMS syndrome epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[POEMS syndrome risk factors|Risk Factors]]==
 
==[[POEMS syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
==Diagnosis==
It was originally described in part by various investigators, and received its present name in 1980 by Bardwick ''et al''<ref name=Bardwick>Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome: report on two cases and a review of the literature. ''Medicine (Baltimore)'' 1980;59:311-22. PMID 6248720.</ref>.
A 2003 study<ref name=Dispenzieri>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. POEMS syndrome: definitions and long-term outcome. ''[[Blood (journal)|Blood]]'' 2003;101:2496-506. PMID 12456500.</ref> followed a series of 99 patients and attempted to delineate criteria for the diagnosis of POEMS syndrome. According to their study, two major criteria and one minor criterion are required for the diagnosis:
* Major criteria:
** [[Polyneuropathy]]
** Monoclonal plasmaproliferative disorder (a [[paraprotein]])
* Minor criteria:
** Sclerotic bone lesions
** Castleman disease
** [[Organomegaly]] ([[splenomegaly]], [[hepatomegaly]] or [[lymphadenopathy]])
** [[Edema]] (edema, [[pleural effusion]] or [[ascites]])
** Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
** Skin changes ([[hyperpigmentation]], [[hypertrichosis]], [[plethora]], [[hemangioma]], white nails)
** [[Papilledema]]


In addition, their study showed a number of associations, the relevance of which to diagnosis was unclear: [[clubbing]], [[weight loss]], [[thrombocytosis]], [[polycythemia]] and [[hyperhidrosis]]. Possible associations included [[pulmonary hypertension]], restrictive lung disease, a [[thrombophilia|thrombotic diathesis]], [[arthralgia]], [[cardiomyopathy]] (systolic dysfunction), [[fever]], low [[vitamin B12]] levels and [[diarrhea]].
[[POEMS syndrome diagnostic criteria|Diagnostic Criteria]] | [[POEMS syndrome history and symptoms|History and Symptoms ]] | [[ POEMS syndrome physical examination|Physical Examination]] | [[POEMS syndrome laboratory findings|Laboratory Findings]] | [[POEMS syndrome chest x ray|Chest X Ray]] | [[POEMS syndrome CT|CT]] | [[POEMS syndrome MRI|MRI]] | [[POEMS syndrome echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[POEMS syndrome other imaging findings|Other Imaging Findings]] | [[POEMS syndrome other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==
Dispenzieri ''et al''<ref name=Dispenzieri /> list numerous treatments, the effect of many of which is almost anecdotal. [[Prednisolone]] and [[alkylating antineoplastic agent|alkylating agent]]s are the most commonly used.
[[POEMS syndrome medical therapy|Medical Therapy]] | [[POEMS syndrome surgery |Surgery]] | [[POEMS syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[POEMS syndrome future or investigational therapies|Future or Investigational Therapies]]


==References==
==Case Studies==
{{reflist|2}}


==External links==
[[POEMS syndrome case study one|Case #1]]
* [http://www.neuro.wustl.edu/neuromuscular/antibody/pnimdem.html#poemsorg WUSTL neuropathy page]
* [http://www.emedicine.com/derm/topic771.htm POEMS syndrome by Wingfield Rehmus, MD, MPH]
* [http://www.blood-disorders.us/poems-syndrome.html POEMS syndrome]


[[Category:Endocrinology]]
[[Category:Endocrinology]]
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[[Category:Rare diseases]]
[[Category:Rare diseases]]
[[Category:Syndromes]]
[[Category:Syndromes]]
[[Category:Disease]]


[[es:Síndrome POEMS]]
[[es:Síndrome POEMS]]

Latest revision as of 15:39, 18 February 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2] Syed Hassan A. Kazmi BSc, MD [3]

Synonyms and keywords: Crow-Fukase syndrome; Takatsuki disease; PEP syndrome

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating POEMS syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

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Case #1

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