ベンス・ジョーンズタンパク質、ベンス・ジョーンズ蛋白質
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"
PrepTutorEJDIC
- 蛋白(たんばく)質
Wikipedia preview
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)
[Wiki en表示]
"Bence-Jones" redirects here. For Mark Bence-Jones the British author, see Mark Bence-Jones.
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.
Eponymous medical signs for hematology
|
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Inclusion bodies |
- Erythrocyte
- Cabot rings
- Howell-Jolly body
- Pappenheimer bodies
- Heinz body
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Tests |
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Other |
- Arneth count
- Mentzer index
- Virchow's triad
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Index of cells from bone marrow
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Description |
- Immune system
- Cells
- Physiology
- coagulation
- proteins
- granule contents
- colony-stimulating
- heme and porphyrin
|
|
Disease |
- Red blood cell
- Monocyte and granulocyte
- Neoplasms and cancer
- Histiocytosis
- Symptoms and signs
- Blood tests
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Treatment |
- Transfusion
- Drugs
- thrombosis
- bleeding
- other
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|
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Metabolic disease: amyloidosis (E85, 277.3)
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Common amyloid forming proteins |
- AA
- ATTR
- Aβ2M
- AL
- Aβ/APP
- AIAPP
- ACal
- APro
- AANF
- ACys
- ABri
|
|
Systemic amyloidosis |
- AL amyloidosis
- AA amyloidosis
- Aβ2M/Haemodialysis-associated
- AGel/Finnish type
- AA/Familial Mediterranean fever
- ATTR/Transthyretin-related hereditary
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|
Organ-limited amyloidosis |
Heart
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AANF/Isolated atrial
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Brain
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- Familial amyloid neuropathy
- ACys+ABri/Cerebral amyloid angiopathy
- Aβ/Alzheimer's disease
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Kidney
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- AApoA1+AFib+ALys/Familial renal
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Cutaneous
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- Primary cutaneous amyloidosis
- Amyloid purpura
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Endocrine
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- Thyroid
- ACal/Medullary thyroid cancer
- Pituitary
- APro/Prolactinoma
- Pancreas
- AIAPP/Insulinoma
- AIAPP/Diabetes mellitus type 2
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UpToDate Contents
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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
- 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 Links
- 特徴 † ベンスジョーンズ蛋白は異常構造免疫グロブリンで、単クローン性、L鎖の2量体として存在。 糸球体?を通過し、尿中に排出されるので、血中には少ない 56 、15分の加熱で白濁・沈殿し、100 で再溶解する。
- 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
Related Pictures
![Bence jones protein](https://1mg.info/0/7/h/2539.jpg)
![for Bence Jones Protein .](https://1mg.info/0/7/h/2540.jpg)
![crystal of Bence Jones protein created](https://1mg.info/0/7/h/2541.jpg)
![آلية دخول السلاسل](https://1mg.info/0/7/h/2542.jpg)
![Bence Jones protein cast (myeloma cast](https://1mg.info/0/7/h/2543.jpg)
★リンクテーブル★
[★]
- 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
WCH.2669
pathologic feature
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MCL
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B-cell CLL
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FL
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MZL
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WM
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MM
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paraprotein
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None
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Small IgG or IgM
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Usually none
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Small IgM
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IgM (large)
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IgA, IgG
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morphology
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Centrocyte-like; small-to-medium-sized lymphocytes
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Small lymphocyte with clumped chromatin
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Follicle center cells (follicular pattern)
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Monocytoid B cells, heterogeneous
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Plasmacytoid lymphocytes and plasma cells
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Plasma cells
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surface Ig
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+
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+
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+
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+
|
+
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+
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cytoplasmic Ig
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-
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-
|
-
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-
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++
|
+++
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CD19
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+
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+
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+
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+
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+
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+
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CD20
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++
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+
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++
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+
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+
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15% CD20+
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CD23
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-
|
+
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±
|
-
|
-
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-
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CD22
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+
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-
|
-
|
+
|
+
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-
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CD38
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-
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±
|
-
|
-
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+
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++
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CD138
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-
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-
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-
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-
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+
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++
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CD5
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+
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+
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-
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-
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Usually CD5-
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-
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CDlO
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±
|
-
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+
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-
|
-
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-
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cytogenetic aberrations
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t(11;14)(q13;q32), cyclin Dl+
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13q-, 6q-, +12, 11q23-
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t(14;18)(q32; q21), bcl-2+
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t(11;18)(q21; q21), +3
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6q-
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t(4;14)(p16.3;q32),t(11;14)(q13;q32),t(14;16)(q32;q23), other +14q32,13q-, aneuploidy
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somatic hypermutation
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?
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+, 50%; -, 50%
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++
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++
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+++
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+++
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bone marrow involvement (%)
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25
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~100
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85
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50
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>90
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100
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bone lytic lesions
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No
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No
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No
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No
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5%
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70%
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概念
病因
疫学
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
- 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
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多発性骨髄腫
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マクログロブリン血症
|
|
ワルデンシュトレーム・マクログロブリン血症
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MM
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WM
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|
病変のfocus
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主に骨髄
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主にリンパ組織
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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
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やや幼弱なB細胞
|
|
赤血球連戦形成
|
+
|
+++
|
IgMのせい
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出血傾向
|
少
|
多
|
IgMのせい
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肝脾腫、リンパ節腫脹
|
|
骨破壊
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70%
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5%
|
|
過粘稠度症候群
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少
|
多
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IgMのせい
|
M蛋白
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IgG,IgA,IgD,IgE,BJP
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IgM
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|
腎障害
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多
|
少
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IgMは糸球体濾過されにくい
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眼底変化
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少
|
多
|
IgMのせい
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年齢
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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)。
血液生化学
尿検査
治療
予後
USMLE
[★]
- 英
- Bence Jones protein, BJP
- 同
- ベンス・ジョーンズ蛋白、ベンスジョーンズ蛋白質、ベンスジョーンズタンパク、Bence Jonesタンパク、Bence Jones蛋白、Bence-Jones蛋白
概念
- 尿中に排泄される
- モノクローナルな抗体のL鎖
- κ型とλ型がある
- 単量体(24 kDa)か2量体(48 kDa)で存在
- 尿からは試験紙法では検出できず、スルホサリチル酸法により検出される
BJPが出現する疾患
- OLM.32
- IgG, IgA, IgD, IgE, BJP型
- IgM
- γ鎖病、μ鎖病
血清中、尿中に増加
[★]
ベンスジョーンズタンパク Bence Jones protein
[★]
ジョーンズ