WordNet

utter shrill sounds; "The cocks crowed all morning"
black birds having a raucous call
the cry of a cock (or an imitation of it)
express pleasure verbally; "She crowed with joy"
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
a member of the Siouan people formerly living in eastern Montana
a Siouan language spoken by the Crow

PrepTutorEJDIC

『カラス』 / =crowbar
《単位形で》おんどりのかん高い鳴き声 / 〈娩〉《通例単数形で》キャッキャッという赤ん坊の笑い声 / 〈おんどりが〉ときを告げる / 〈赤ん坊が〉キャッキャッと笑う / (一般に)〈人が〉誇らしげに歓声をあげる
(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
〈C〉〈U〉クロー族[の人](米国Montana州東部のアメリカインディアンの一族) / クロー語
chromiumの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/06/02 11:58:09」(JST)

wiki en

POEMS syndrome (also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare medical syndrome. It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy, and effects on many other organ systems. It begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women.^[1] If untreated it is progressive and often fatal, with only 60% of sufferers remaining alive five years after onset.^[1] However, the symptoms can improve if the blood disorder is successfully treated.

The name 'POEMS syndrome', now the most prevalent, was coined in 1980 by Bardwick et al.^[2] The name is an acronym deriving from some of the main features: Polyneuropathy (peripheral nerve damage), Organomegaly (abnormal enlargement of organs), Endocrinopathy (damage to hormone-producing glands) or Edema, M-protein (an abnormal immunoglobulin) and Skin abnormalities (including hyperpigmentation and hypertrichosis).^[3] However, these features are neither an exhaustive list nor seen in all individuals with the condition.

Signs and symptoms

The following are the most common clinical features of POEMS syndrome, either symptoms or signs.^[1]^[4] The first two are present by definition; the remainder are frequent but not universal, and different individuals experience different combinations of them.

Defining features

The polyneuropathy is usually symmetrical and distal. There is a combination of demyelination and axonal damage, and sensory, motor and autonomic neurones are all affected. The typical symptoms are therefore of numbness, tingling and weakness in the feet, later affecting the legs and hands. Pain is unusual, but the weakness may eventually become severe and disabling. The autonomic neuropathy may cause excessive sweating and erectile dysfunction; hormonal changes may also contribute to the latter. It is usually the symptoms of neuropathy which prompt a person with POEMS syndrome to seek medical attention.

Myeloma is the most common plasma-cell proliferative disorder associated with POEMS syndrome, present in more than half of all cases. Most myelomas associated with POEMS syndrome are osteosclerotic, in contrast to the osteolytic bone lesions in most other myelomas. Other blood disorders occur in a significant minority of cases, including Castleman's disease, plasmacytoma and MGUS.

Common features

Papilloedema is a common sign of POEMS syndrome, occurring in a significant proportion of cases. Less frequent ocular features include uveitis and preretinal haemorrhage.

Pulmonary disease: The lungs are often affected at more severe stages of the illness, although since by then physical exertion is usually limited by neuropathy, shortness of breath is unusual. Pulmonary hypertension is the most serious effect on the lungs, and there may also be restriction of chest expansion or impaired gas exchange.

Organomegaly: The liver may be enlarged, and less often the spleen or lymph nodes, though these organs usually function normally.

Oedema: Leakage of fluid into the tissues is a common and often severe problem. This may take several forms, including dependent peripheral oedema, pulmonary oedema, effusions such as pleural effusion or ascites, or generalised capillary leakage (anasarca).

Endocrinopathy: In women, amenorrhoea, and in men, gynaecomastia, erectile dysfunction and testicular atrophy, are common early symptoms due to dysfunction of the gonadal axis. Other hormonal problems occurring in at least a quarter of patients include type 2 diabetes, hypothyroidism and adrenal insufficiency.

Monoclonal paraprotein: In most cases a serum paraprotein can be detected, although this is not universal (75–80%). This may represent IgG or IgA, but the light chain type is almost always lambda. This is in contrast to most paraproteinaemic neuropathies, in which the paraprotein is usually an IgM antibody.

Skin changes: A very wide variety of skin problems have been reported in association with POEMS syndrome. The most common is non-specific hyperpigmentation. The fingernails may be clubbed or white, There may be thickening of the skin, excess hair or hair in unusual places (hypertrichosis), skin angiomas or haemangiomas, or there may be changes reminiscent of scleroderma.

Possible features

Some features have been observed in patients with POEMS syndrome but are not yet certain to form part of the syndrome itself. These include thrombophilia, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.^[5]

Laboratory findings

In addition to tests corresponding to the above findings (such as EMG for neuropathy or CT scan and bone marrow biopsy for myeloma), two other tests give abnormal results. These are: a raised level of VEGF in blood; and a raised CSF protein level, as in some other neuropathies. Even when myeloma is present, anaemia and thrombocytopaenia are rare; on the contrary, polycythaemia and thrombocytosis are often found.

Criteria

The most recent criteria for the diagnosis of POEMS syndrome^[4] require that all of the following be present:

polyneuropathy
a monoclonal plasma-cell proliferative disorder
one or more of the following major criteria:
- sclerotic bone lesions
- Castleman's disease
- elevated VEGF levels
one or more of the following minor criteria:
- organomegaly (enlarged spleen, liver or lymph nodes)
- extravascular volume overload (oedema, pleural effusion or ascites)
- endocrinopathy (adrenal, pituitary, gonadal, parathyroid); diabetes or hypothyroidism alone are insufficient
- skin changes
- papilloedema
- thrombocytosis or polycythaemia

Pathogenesis

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 cytokines produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically interleukin 1β, interleukin 6 and TNFα. There also seems to be a role of vascular endothelial growth factor (VEGF), given the angiogenic changes in many tissues.^[5]

Treatment

There are no randomised controlled trials of treatment in POEMS syndrome, owing to its rarity.^[6] Conventional treatments for demyelinating neuropathy, such as steroids, intravenous immunoglobulin and plasma exchange, are ineffective; treatment must be aimed at the haematological disorder.

Dispenzieri et al.^[5] list numerous treatments, the effect of many of which is almost anecdotal. Prednisolone and alkylating agents are the most commonly used. The Mayo Clinic group attempted hematopoietic stem cell transplantation in sixteen patients; one patient died during the treatment, and in several others respiratory problems were unmasked in the course of the procedure. The outcome of the treatment, though, was good.

Given that VEGF plays a central role in the symptoms of POEMS syndrome, some have tried bevacizumab (Avastin), a monoclonal antibody directed against VEGF. While some reports were positive, others have reported capillary leak syndrome suspected to be the result of overly rapid lowering of VEGF levels. It therefore remains doubtful as to whether this will become part of standard treatment for POEMS syndrome.^[7]

History

R. S. Crow, working in Bristol, first described the combination of osteosclerotic myeloma, polyneuropathy and various unusual features (such as pigmentation and clubbing) in two patients aged 54 and 67.^[8]

References

^ ^a ^b ^c "Neuromuscular Disease Center, Washington University". Retrieved 4 July 2010.
^ Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (1980). "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) 59 (4): 311–22. doi:10.1097/00005792-198007000-00006. PMID 6248720.
^ "POEMS syndrome" at Dorland's Medical Dictionary
^ ^a ^b Dispenzieri, A. (2007). "POEMS syndrome". Blood reviews 21 (6): 285–299. doi:10.1016/j.blre.2007.07.004. PMID 17850941. edit
^ ^a ^b ^c Dispenzieri A, Kyle RA, Lacy MQ, et al. (2003). "POEMS syndrome: definitions and long-term outcome". Blood 101 (7): 2496–506. doi:10.1182/blood-2002-07-2299. PMID 12456500.
^ "The Cochrane Library". Retrieved 4 July 2010.
^ Samaras P, Bauer S, Stenner-Liewen F, et al. (2007). "Treatment of POEMS syndrome with bevacizumab". Haematologica 92 (10): 1438–9. doi:10.3324/haematol.11315. PMID 18024383.
^ Crow RS (1956). "Peripheral neuritis in myelomatosis". Br Med J 2 (4996): 802–4. doi:10.1136/bmj.2.4996.802. PMC 2035359. PMID 13364332.

External links

Mayo Clinic

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. POEMS症候群 poems syndrome
2. キャッスルマン病 castlemans disease
3. 多発性骨髄腫の臨床的特徴、検査所見、および診断 clinical features laboratory manifestations and diagnosis of multiple myeloma
4. エルドハイム・チェスター病 erdheim chester disease
5. 末梢神経と筋肉に影響を与える腫瘍随伴性症候群 paraneoplastic syndromes affecting peripheral nerve and muscle

English Journal

Acquired facial atrophy: A neglected clinical sign of POEMS syndrome.

Bachmeyer C.SourceService de Médecine Interne, CHU Tenon (AP-HP), 4 rue de la Chine, 75020 Paris, France. claude.bachmeyer@tnn.aphp.fr.
American journal of hematology.Am J Hematol.2012 Jan;87(1):131. doi: 10.1002/ajh.22204. Epub 2011 Nov 11.
PMID 22076924

Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study.

Briani C, Fedrigo M, Manara R, Castellani C, Zambello R, Citton V, Campagnolo M, Torre CD, Lucchetta M, Orvieto E, Rotilio A, Marangoni S, Magi S, Pareyson D, Florio I, Pegoraro E, Thiene G, Battistin L, Adami F, Angelini A.SourceDepartment of Neurosciences, University of Padova, Via Giustiniani 5, 35128 Padova, Italy; chiara.briani@unipd.it.
Journal of neurology, neurosurgery, and psychiatry.J Neurol Neurosurg Psychiatry.2012 Jan;83(1):33-7. Epub 2011 Jun 7.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare plasma cell disease. Vascular endothelial growth factor (VEGF) seems to play a pathogenic role. Peripheral neuropathy is the main neurological feature. Cranial pachymeningitis has occasionall
PMID 21653206

Uterine Glomeruloid Hemangioma in a Patient Without POEMS Syndrome.

Giner F, Compañ A, Monteagudo C.SourceUniversity Clinic Hospital, University of Valencia, Valencia, Spain.
International journal of surgical pathology.Int J Surg Pathol.2011 Dec 7. [Epub ahead of print]
Cutaneous glomeruloid hemangioma is a hallmark of POEMS syndrome. These patients have elevated serum levels of vascular endothelial growth factor. The authors report an extracutaneous uterine glomeruloid hemangioma in an 82-year-old woman with a history of breast and endometrial carcinomas. Within t
PMID 22134630

Japanese Journal

基本シリーズ原発性アミロイドーシス,POEMS症候群の治療 (第73回日本血液学会学術集会教育講演)

中世古知昭
臨床血液 52(10), 1496-1506, 2011-10
NAID 40018993955

医学と医療の最前線 Crow-Fukase症候群の病態と新規治療

桑原聡
日本内科学会雑誌 100(8), 2275-2281, 2011-08-10
NAID 40018953983

クロウ・深瀬症候群(POEMS症候群) (特集続・日本人の発見した神経疾患)

岩下宏
Brain and nerve 63(2), 131-139, 2011-02
NAID 40018721583

Related Pictures

★リンクテーブル★

リンク元	「ポエムス症候群」
関連記事	「Cr」「crow」「syndrome」

「ポエムス症候群」

POEMS syndrome
	Classification and external resources
DiseasesDB	29226
MeSH	D016878

　　[★]

英: POEMS syndrome
同: クロウ・深瀬症候群 Crow-Fukase症候群 Crow-Fukase syndrome、高月病 Takatsuki disease

[show details]

ポエムス症候群 : 46 件
ポエムズ症候群 : 15 件
POEMS症候群 : 約 13,700 件
クロウ・深瀬症候群 : 89 件

en

Crow-Fukase syndrome : 約 29,300 件
POEMS syndrome : 約 334,000 件

ja

Crow-Fukase syndrome : 約 1,710 件
POEMS syndrome : 約 3,640 件

参考

1. 単クローン抗体を伴う末梢神経炎（クロウ・フカセ症候群、POEMS症候群） - 難病情報センター

http://www.nanbyou.or.jp/entry/241

2．[PDF]診断基準など

http://www.celltherapytransplantation.com/520-3_520.pdf

3. 疫学研究としての診断基準

http://www.m.chiba-u.ac.jp/class/neurol/kenkyu/ishisyudou/crow-fukase/ishi/sindan.html

腎障害を伴うPOEMS症候群

検索ワード「POEMS syndrome glomerulonephritis」

Serial renal biopsy findings in a case of POEMS syndrome with recurrent acute renal failure.

http://www.ncbi.nlm.nih.gov/pubmed/21979795

Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation.

http://www.ncbi.nlm.nih.gov/pubmed/16564945

Although not included in the acronym, renal lesions also are characteristic of this disease and sometimes require dialysis therapy.

腎障害も良く合併するんだって

POEMS syndrome: definitions and long-term outcome.

http://www.ncbi.nlm.nih.gov/pubmed/12456500

The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes.

Successful stabilisation of nephropathy in a patient with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, skin changes) syndrome on treatment with mycophenolate and steroids: a case report

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2848064/

Nakamoto et al. [7] reviewed 52 cases of Japanese patients with nephropathy. All patients manifested four or all of the five signs of POEMS. Polyneuropathy was present in all but one patient. A majority of cases had monoclonal protein in blood (either IgA- or IgG-) although 10 demonstrated a polyclonal hypergammaglobulinaemia, but in these, four other signs of POEMS supported the diagnosis.

Nakamoto et al. [7] -> http://www.ncbi.nlm.nih.gov/pubmed/10528660