WordNet

United States golfer (1902-1971) (同)Bobby Jones, Robert Tyre Jones
United States railroad engineer who died trying to stop his train from crashing into another train; a friend wrote a famous ballad describing the incident (1864-1900) (同)Casey Jones, John Luther Jones
English phonetician (1881-1967) (同)Daniel Jones
one of the first great English architects and a theater designer (1573-1652) (同)Inigo Jones
American naval commander in the American Revolution (1747-1792) (同)John Paul Jones
United States labor leader (born in Ireland) who helped to found the Industrial Workers of the World (1830-1930) (同)Mother Jones, Mary Harris Jones
any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"

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蛋白(たんばく)質

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/04/11 07:07:40」(JST)

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A crystal of Bence Jones protein.

A Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular weight of 22-24 kDa.^[1] Detection of Bence Jones protein may be suggestive of multiple myeloma or Waldenström's macroglobulinemia.

Bence Jones proteins are particularly diagnostic of multiple myeloma in the context of end-organ manifestations such as renal failure, lytic (or "punched out") bone lesions, anemia, or large numbers of plasma cells in the bone marrow of patients. Bence Jones proteins are present in 2/3 of multiple myeloma cases.^[2]

The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They can be kappa (most of the time) or lambda.^[2] The light chains can be immunoglobulin fragments or single homogeneous immunoglobulins. They are found in urine due to the kidneys' decreased filtration capabilities due to renal failure, sometimes induced by hypercalcemia from the calcium released as the bones are destroyed or from the light chains themselves.^{[citation needed]} The light chains have historically been detected by heating and now by electrophoresis of concentrated urine. More recently serum free light chain assays have been utilised in a number of published studies which have indicated superiority over the urine tests, particularly for patients producing low levels of monoclonal free light chains, as seen in nonsecretory multiple myeloma^[3]^[4]^[5] and AL amyloidosis.^[5]^[6]^[7]^[8] This is primarily because of the re-absorption of free light chains in the kidneys, creating a "threshold" of light chain production which must be exceeded before measurable quantities overflow into the urine. As such, urinalysis is a fickle witness to changing free light chain production.

History

The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in 1848.^[9] The protein was later sequenced by Frank W. Putnam Sr. at the laboratory of Fred Sanger in Cambridge, who was the first to report the entire sequence.

References

^ Bernier, G. M. & Putnam, F. W. (1963). Nature (London), 200, 223±225.
^ ^a ^b Hoffbrand V, Moss P, Pettit J (2006). Essential Haematology (Essential) (5th ed.). Blackwell Publishing Professional. p. 218. ISBN 1-4051-3649-9.
^ Drayson M, Tang LX, Drew R, Mead GP, Carr-Smith H, Bradwell AR (May 2001). "Serum free light-chain measurements for identifying and monitoring patients with nonsecretory multiple myeloma". Blood 97 (9): 2900–2. doi:10.1182/blood.V97.9.2900. PMID 11313287.
^ Shaw GR (August 2006). "Nonsecretory plasma cell myeloma--becoming even more rare with serum free light-chain assay: a brief review". Archives of Pathology & Laboratory Medicine 130 (8): 1212–5. doi:10.1043/1543-2165(2006)130[1212:NPCMEM]2.0.CO;2. PMID 16879026.
^ ^a ^b Katzmann JA, Abraham RS, Dispenzieri A, Lust JA, Kyle RA (May 2005). "Diagnostic performance of quantitative kappa and lambda free light chain assays in clinical practice". Clinical Chemistry 51 (5): 878–81. doi:10.1373/clinchem.2004.046870. PMID 15774572.
^ Lachmann HJ, Gallimore R, Gillmore JD et al. (July 2003). "Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy". British Journal of Haematology 122 (1): 78–84. doi:10.1046/j.1365-2141.2003.04433.x. PMID 12823348.
^ Abraham RS, Katzmann JA, Clark RJ, Bradwell AR, Kyle RA, Gertz MA (February 2003). "Quantitative analysis of serum free light chains. A new marker for the diagnostic evaluation of primary systemic amyloidosis". American Journal of Clinical Pathology 119 (2): 274–8. doi:10.1309/LYWM-47K2-L8XY-FFB3. PMID 12579999.
^ Akar H, Seldin DC, Magnani B et al. (December 2005). "Quantitative serum free light chain assay in the diagnostic evaluation of AL amyloidosis". Amyloid 12 (4): 210–5. doi:10.1080/13506120500352339. PMID 16399645.
^ Jones HB (1848). "On a new substance occurring in the urine of a patient with mollities ossium". Philosophical Transactions of the Royal Society 138: 55–62. doi:10.1098/rstl.1848.0003.

UpToDate Contents

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1. 意義未確定の単クローン性免疫グロブリン血症（MGUS）の診断 diagnosis of monoclonal gammopathy of undetermined significance
2. モノクローナル蛋白の認識 recognition of monoclonal proteins
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. 成人における尿蛋白排泄量の評価および非ネフローゼ域蛋白尿のみの評価 assessment of urinary protein excretion and evaluation of isolated non nephrotic proteinuria in adults
5. 骨髄腫円柱腎症（骨髄腫腎）の病因および診断 pathogenesis and diagnosis of myeloma cast nephropathy myeloma kidney

English Journal

A patient with persistent renal AL amyloid deposition after clinical remission by HDM/SCT therapy.

Okuyama H, Yamaya H, Fukusima T, Yokoyama H.SourceDivision of Nephrology, Division of Hematology and Immunology, Kanazawa Medical University School of Medicine, Ishikawa, Japan.
Clinical nephrology.Clin Nephrol.2013 Mar;79(3):233-6.
A 62-year-old female patient was admitted to our hospital for evaluation of nephrotic syndrome. Monoclonal gammopathy (IgG λ type) and urinary Bence Jones proteins were detected in the serum and urine by the immunofixation method. The initial renal biopsy revealed amyloid deposition in mesangial ar
PMID 23439244

Current therapeutic strategy for multiple myeloma.

Suzuki K.Source*For reprints and all correspondence: Kenshi Suzuki. ken-suzuki@mtb.biglobe.ne.jp.
Japanese journal of clinical oncology.Jpn J Clin Oncol.2013 Feb;43(2):116-24. doi: 10.1093/jjco/hys215. Epub 2013 Jan 4.
This is a review regarding the current therapeutic strategies in the management of multiple myeloma. Due to the introduction of several new effective therapeutic agents, multiple myeloma is one of the most active and changing fields in clinical oncology. Multiple myeloma is caused by the expansion o
PMID 23293370

Japanese Journal

肝機能異常を伴った多発性骨髄腫の1例

斎藤広信,高橋敦史,阿部和道,物江恭子,菅野有紀子,横川順子,大平弘正
肝臓 52(1), 65-69, 2011-01-25
… 検査を施行した．骨髄検査ではCD138陽性の異型形質細胞がびまん性に増生し，造血細胞巣の70％以上を占め，頭部レントゲン検査での打ち抜き像，尿中Bence Jones蛋白陽性，血清蛋白免疫電気泳動と併せ，Bence Jones型の多発性骨髄腫と診断した．肝機能異常については，経過から薬物性肝障害は考え難く，自己抗体も陰性で画像所見においても異常所見が無いこと …
NAID 10029284919

AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein : successful treatment by autologous stem cell transplantation

SAKURAI CHIN Chanhyok,UBARA Yoshifumi,SUWABE Tatsuya,HOSHINO Junichi,YONAHA Tomoki,HASEGAWA Eiko,SUMIDA Keiichi,HIRAMATSU Rikako,YAMANOUCHI Masayuki,HAYAMI Noriko,YAMAUCHI Junji,TOMINAGA Naoyuki,SAWA Naoki,TAKEMOTO Fumi,MASUOKA Kazuhiro,TAKAICHI Kenmei,OOHASHI Kenichi
Clinical and experimental nephrology 14(5), 506-510, 2010-10-01
NAID 10027707419

Related Pictures

★リンクテーブル★

リンク元	「マクログロブリン血症」「ベンスジョーンズ蛋白」「BJP」
関連記事	「Jones」

「マクログロブリン血症」

　　[★]

英: macroglobulinemia
同: ワルデンシュトレーム・マクログロブリン血症 Waldenström macroglobulinemia Waldenstrom macroglobulinemia WM、原発性マクログロブリン血症 primary macroglobulinemia、マクログロブリネミア
関: 単クローン性免疫グロブリン血症。高γグロブリン血症

first aid step1 2006 p.304

Bence Jones protein：multiple myeloma(κ/λ Ig light chains in urine), Waldenström's macroglobulinemia (IgM)
monoclonal antibody spike：multiple myeloma (M protein = IgG/IgA), MGUS (monoclonal gammopathy of undetermined significance), Waldenström's macroglobulinemia (M protein = IgM)

	ALL	ALL(バーキット型)	悪性リンパ腫、CLL	マクログロブリン血症	骨髄腫
分化段階	前駆B細胞	未熟B細胞	成熟B細胞		形質細胞
細胞表面免疫グロブリン	－	＋	＋	±	－
免疫グロブリン分泌	－	－	－	＋	＋

WCH.2669

pathologic feature	MCL	B-cell CLL	FL	MZL	WM	MM
paraprotein	None	Small IgG or IgM	Usually none	Small IgM	IgM (large)	IgA, IgG
morphology	Centrocyte-like; small-to-medium-sized lymphocytes	Small lymphocyte with clumped chromatin	Follicle center cells (follicular pattern)	Monocytoid B cells, heterogeneous	Plasmacytoid lymphocytes and plasma cells	Plasma cells
surface Ig	＋	＋	＋	＋	＋	＋
cytoplasmic Ig	－	－	－	－	＋＋	＋＋＋
CD19	＋	＋	＋	＋	＋	＋
CD20	＋＋	＋	＋＋	＋	＋	15% CD20+
CD23	－	＋	±	－	－	－
CD22	＋	－	－	＋	＋	－
CD38	－	±	－	－	＋	＋＋
CD138	－	－	－	－	＋	＋＋
CD5	＋	＋	－	－	Usually CD5-	－
CDlO	±	－	＋	－	－	－
cytogenetic aberrations	t(11;14)(q13;q32), cyclin Dl+	13q-, 6q-, +12, 11q23-	t(14;18)(q32; q21), bcl-2+	t(11;18)(q21; q21), +3	6q-	t(4;14)(p16.3;q32),t(11;14)(q13;q32),t(14;16)(q32;q23), other +14q32,13q-, aneuploidy
somatic hypermutation	？	＋, 50%; -, 50%	＋＋	＋＋	＋＋＋	＋＋＋
bone marrow involvement (%)	25	～100	85	50	＞90	100
bone lytic lesions	No	No	No	No	5%	70%

概念

病因

疫学

60-70歳

症状

also see WCH.2671

血管粘稠度上昇　＋　病的蛋白質による血管内皮損傷：[眼症状] ソーセージ様の網膜血管のうっ滞・拡張、網膜出血、綿花様白斑、網膜静脈閉塞症、滲出性網膜剥離
リンパ球の浸潤による症状：weakness, 体重減少、骨痛、肝脾腫、リンパ節腫

診断

鑑別診断

慢性リンパ性白血球、骨髄腫(IgM myeloma: bone lesion, CD138+ plamac cell infiltration)、kymphocytic lymphoma

myelomaとの共通点と異差

HIM.706

共通点：

症状：weakness、疲労、反復感染

WMに特徴的：

lymphadenopathy、hepatosplenomegalyがあること。bone lesionや高カルシウム血症は起こさない。(大きさの違いから？)IgMは殆ど人から排泄されないし、-20%の人しかBJPを排泄しないから、腎病変は一般的でない。

WMでより一般的：

鼻出血、視力障害(visual disturbance)、神経症状(めまい、頭痛、一時的な麻痺)

QB.G-269 WCH.2671

	多発性骨髄腫	マクログロブリン血症
	多発性骨髄腫	ワルデンシュトレーム・マクログロブリン血症
	MM	WM
病変のfocus	主に骨髄	主にリンパ組織	集積することで出現する症状が異なる
腫瘍細胞	形質細胞	やや幼弱なB細胞
赤血球連戦形成	＋	＋＋＋	IgMのせい
出血傾向	少	多	IgMのせい
肝脾腫、リンパ節腫脹	少	多
骨破壊	70％	5％
過粘稠度症候群	少	多	IgMのせい
M蛋白	IgG,IgA,IgD,IgE,BJP	IgM
腎障害	多	少	IgMは糸球体濾過されにくい
眼底変化	少	多	IgMのせい
年齢	40-60歳代	40-70歳代

検査

血算

赤沈：高度促進
貧血
白血球：増多(リンパ球様細胞の増多)
血小板：減少　　←　　MMより頻度は低い、はず。一般的でなく、病気の末期まで出現しない(WCH.2671)。患者のほとんどが正常範囲内で、10万/ul以下であるのはほんの9%の患者であり、本疾患における血小板減少症の有病率は22%と報告されている(WCH.2671)。