mucosa-associated lymphoid tissue lymphoma

MALTリンパ腫

WordNet

1. having partial rights and privileges or subordinate status; "an associate member"; "an associate professor"
2. a person who joins with others in some activity or endeavor; "he had to consult his associate before continuing"
3. a person with subordinate membership in a society, institution, or commercial enterprise; "associates in the law firm bill at a lower rate than do partners"
4. any event that usually accompanies or is closely connected with another; "first was the lightning and then its thunderous associate"
5. make a logical or causal connection; "I cannot connect these two pieces of evidence in my mind"; "colligate these facts"; "I cannot relate these events at all" (同)tie_in, relate, link, colligate, link up, connect
6. a soft thin (usually translucent) paper (同)tissue_paper
7. part of an organism consisting of an aggregate of cells having a similar structure and function
8. resembling lymph or lymphatic tissues
9. a neoplasm of lymph tissue that is usually malignant; one of the four major types of cancer

PrepTutorEJDIC

1. 〈人〉'を'(…の)『仲間に加える』,(…に)連合させる《+『名』〈人〉+『with』+『名』》 / (…と)…'を'結びつけて考える(起こす)《+『名』+『with』+『名』》 / (…と)『仲間になる』,交際する《+『with』+『名』》 / (…と)合体する,提携する《+『with』+『名』》 / 『仲間』,『同僚』;組合員 / 準会員 / 付属物;連想されるもの / 連合した;仲間の,同僚の / 補助の,準…
2. 〈U〉〈C〉(生物体の)『組織』 / 〈U〉〈C〉『薄織物』 / 〈U〉〈C〉水を吸収する柔らかな薄紙 / 〈C〉カーボンコピー用薄紙 / 〈C〉《a ~》(…を)織り交ぜて作ったもの《+of+名》 / =tissue paper
3. リンパ状(液)の

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/11/20 23:32:59」(JST)

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MALT lymphoma
Endoscopic image of gastric MALT lymphoma taken in body of stomach in patient who presented with upper GI hemorrhage. Appearance is similar to gastric ulcer with adherent clot.
Classification and external resources
Specialty	oncology
ICD-10	C88.4
ICD-9-CM	200.3
ICD-O	M9699/3
OMIM	604860
DiseasesDB	31339
MeSH	D018442

MALT lymphoma (MALToma) is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT, and is also called extranodal marginal zone B cell lymphoma.

Diagnosis and staging

MALT lymphoma is an often multifocal disease in the organ of origin and is frequently macroscopically indistinguishable from other disease processes in the GI tract. Endoscopy is key to diagnosing MALT lymphoma, with multiple biopsies of the visible lesions required, as well as samples of macroscopically normal tissue, termed gastric mapping. Histologically, there is expansion of the marginal zone compartment with development of sheets of neoplastic small lymphoid cells. The morphology of the neoplastic cells is variable with small mature lymphocytes, cells resembling centrocytes (centrocyte like cells), or marginal zone/monocytoid B cells. Plasmacytoid or plasmacytic differentiation is frequent. Lymphoid follicles are ubiquitous to MALT lymphoma but may be indistinct as they are often overrun or colonized by the neoplastic cells. Large transformed B cells are present scattered among the small cell population. If these large cells are present in clusters or sheets, a diagnosis of associated large B-cell lymphoma should be considered. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions.

MALT lymphoma may be difficult to distinguish from reactive infiltrates, and in some cases, multiple endoscopies are required before a confident diagnosis is reached. The Wotherspoon score, which grades the presence of histological features associated with MALT lymphoma, is useful in expressing confidence in diagnosis at presentation. Immunohistochemistry can be used to help distinguish MALT lymphoma from other small B-cell NHLs. B-cell-associated antigens such as CD19, CD20, CD22, and CD79a are usually expressed. In contrast to small lymphocytic lymphoma and MCL, staining for CD5 is usually negative, and these lymphomas can be further distinguished with CD23 (positive in small lymphocytic lymphoma) and CyclinD1 (positive in MCL).

Associations

Gastric MALT lymphoma is frequently associated (72–98%) with chronic inflammation as a result of the presence of Helicobacter pylori, potentially involving chronic inflammation, or the action of H. pylori virulence factors such as CagA.

The initial diagnosis is made by biopsy of suspicious lesions on esophagogastroduodenoscopy (EGD, upper endoscopy). Simultaneous tests for H. pylori are also done to detect the presence of this microbe.

In other sites, chronic immune stimulation is also suspected in the pathogenesis (e.g. association between chronic autoimmune diseases such as Sjögren's syndrome and Hashimoto's thyroiditis, and MALT lymphoma of the salivary gland and the thyroid).

Treatment

Due to the causal relationship between H. pylori infection and MALT lymphoma, identification of the infection is mandatory. Histological examination of GI biopsies yields a sensitivity of 95% with five biopsies, but these should be from sites uninvolved by lymphoma and the identification of the organism may be compromised by areas of extensive intestinal metaplasia. As proton-pump inhibition can suppress infection, any treatment with this class of drug should be ceased 2 weeks prior to biopsy retrieval. Serology should be performed if histology is negative, to detect suppressed or recently treated infections. Following the recognition of the association of gastric MALT lymphoma with H. pylori infection, it was established that early-stage gastric disease could be cured by H. pylori eradication, which is now the mainstay of therapy. Fifty to 95% of cases achieve complete response (CR) with H. pylori treatment.

A t(11;18)(q21;q21) chromosomal translocation, giving rise to an API2-MLT fusion gene, is predictive of poor response to eradication therapy.

Radiotherapy

Radiotherapy is a valid first option for MALT lymphoma. It provides local control and potential cure in localized gastric stage IE and II 1E disease with 5-year EFS of 85-100% reported in retrospective studies. However, the irradiation field is potentially large as it must include the whole stomach, which can vary greatly in size and shape. Irradiation techniques have improved considerably in the last 20 years, including treating the patient in a fasting state, decreasing the irradiated field and required dose. The moderate dose of 30 Gray (Gy) of involved-field radiotherapy administered in 15 fractions (doses) can be associated with tolerable toxicity and excellent outcomes. Hence, radiotherapy is the preferred approach for local disease where antibiotic therapy has failed, or is not indicated. Evidence also suggests that radiotherapy can be utilized to control localized relapses outside the original radiation field.

Chemotherapy

MALT lymphoma is exquisitely immunotherapy sensitive. Chemotherapy is reserved for those uncommon patients with disseminated disease at presentation or lack of response to local treatment. Rituximab, the anti-CD20 chimeric antibody, is a key component of therapy. Responses vary from 55% to 77% with monotherapy and 100% in combination with chemotherapy. Oral alkylating agents such as cyclophosphamide or chlorambucil have been administered for a median duration of 12 months with high rates of disease control (CR up to 75%) but appear not to be active in t(11;18) disease. The purine nucleoside analogs fludarabine and cladribine also demonstrate activity, the latter conferring a CR rate of 84% (100% in those with gastric primaries) in a small study. A pivotal study of rituximab plus chlorambucil compared with chlorambucil alone (IELSG-19 study, n = 227) demonstrated a significantly higher CR rate (78% vs. 65%; p = 0.017) and 5-year EFS (68% vs. 50%; p = 0.024) over chlorambucil alone. However, 5-year OS was not improved (88% in both arms). First-line treatment of choice is generally rituximab in combination with single alkylating agents or fludarabine, or a combination of all three drugs. The final results of this study, including the later addition of a rituximab-alone arm, are pending.

Two other genetic alterations are known:

• t(1;14)(p22;q32), which deregulates BCL10, at the locus 1p22.
• t(14;18)(q32;q21), which deregulates MALT1, at the locus 18q21.

These seem to turn on the same pathway as API2-MLT (i.e., that of NF-κB). They both act upon IGH, which is at the locus 14q32.

Epidemiology

Of all cancers involving the same class of blood cell, 8% of cases are MALT lymphomas.

See also

• Primary cutaneous marginal zone lymphoma

UpToDate Contents

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• 1. 辺縁帯（MALT）リンパ腫の治療 treatment of marginal zone malt lymphoma
• 2. 節外（MALT）および節性濾胞辺縁帯リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of extranodal malt and nodal marginal zone lymphomas
• 3. 脾辺縁帯リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of splenic marginal zone lymphoma
• 4. 消化管リンパ腫のマネージメント management of gastrointestinal lymphomas
• 5. 原発性消化管リンパ腫の臨床症状および診断 clinical presentation and diagnosis of primary gastrointestinal lymphomas

English Journal

• Activation of NF-κB and respiratory burst following Aspergillus fumigatus stimulation of macrophages.

• Sun H, Xu XY, Tian XL, Shao HT, Wu XD, Wang Q, Su X, Shi Y.SourceDepartment of Respiratory and Critical Care Medicine, Jinling Hospital, Clinical School of Nanjing, Second Military Medical University, Nanjing 210002, China.
• Immunobiology.Immunobiology.2014 Jan;219(1):25-36. doi: 10.1016/j.imbio.2013.06.013. Epub 2013 Jul 4.
• Dectin-2, a C-type lectin receptor (CLR), plays an essential role in mediating nuclear factor-kappa B (NF-κB) activation and anti-fungal immunity in response to Candida albicans infection. However, the molecular mechanisms and function of Dectin-2 signaling in response to infection by the pathogeni
• PMID 23886693

• Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal zone origin: is this a distinct entity?

• Xochelli A, Kalpadakis C, Gardiner A, Baliakas P, Vassilakopoulos TP, Mould S, Davis Z, Stalika E, Kanellis G, Angelopoulou MK, McIver-Brown N, Ibbotson R, Sachanas S, Korkolopoulou P, Athanasiadou A, Anagnostopoulos A, Papadaki HA, Papadaki T, Stamatopoulos K, Pangalis GA, Oscier D.SourceHematology Department and HCT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece;
• Blood.Blood.2013 Dec 3. [Epub ahead of print]
• The biological and clinical significance of a clonal B-cell lymphocytosis with an immunophenotype consistent with marginal zone origin (CBL-MZ) is poorly understood. We retrospectively evaluated 102 such cases with no clinical evidence to suggest a concurrent MZ lymphoma. Immunophenotyping revealed
• PMID 24300853

• Whole exome sequencing in splenic marginal zone lymphoma reveals mutations in genes involved in marginal zone differentiation.

• Martínez N, Almaraz C, Vaqué JP, Varela I, Derdak S, Beltran S, Mollejo M, Campos-Martin Y, Agueda L, Rinaldi A, Kwee I, Gut M, Blanc J, Oscier D, Martinez-Lopez J, Salar A, Sole F, Rodriguez-Peralto JL, Díez C, García JF, Fraga M, Sebastián E, Alvés J, Menárguez J, González-Carreró J, Casado LF, Bayes M, Bertoni F, Gut I, Piris MA.SourceCancer Genomics, IFIMAV Instituto de Formación e Investigación Marqués de Valdecilla, Santander, Spain.
• Leukemia.Leukemia.2013 Dec 3. doi: 10.1038/leu.2013.365. [Epub ahead of print]
• Splenic marginal zone lymphoma (SMZL) is a B cell neoplasm whose molecular pathogenesis remains fundamentally unexplained, requiring more precise diagnostic markers. Previous molecular studies have revealed 7q loss and mutations of NF-kB, BCR and Notch signalling genes. We performed whole exome sequ
• PMID 24296945

Japanese Journal

• 症例報告 腫瘍内部に結核性病変が並存した肺MALTリンパ腫

• 井上 盛浩,井脇 康宣,華 見
• 臨床血液 53(11), 1921-1925, 2012-11
• NAID 40019507297

• アミロイド沈着を伴った播種性肺MALTリンパ腫の1剖検例

• 阪下 健太郎,高森 幹雄,村田 研吾,和田 曉彦,藤田 明
• 気管支学 : 日本気管支研究会雑誌 34(5), 479-485, 2012-09-25
• 背景.アミロイド沈着はMALTリンパ腫における予後不良因子であるとの報告が散見される.異時性に診断された本例の肺と胃のMALTリンパ腫はともにALアミロイドの沈着を伴っていた.アミロイド沈着とMALTリンパ腫について,文献的考察を加え報告する.症例. 70歳男性.右肺門部腫瘤影を指摘され紹介. 58歳時,胃MALTリンパ腫に対し胃全摘術施行.気管支鏡にて,右主気管支粘膜の不整隆起と発赤を認め,同部 …
• NAID 110009518314

Related Pictures

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★リンクテーブル★

リンク元	「MALTリンパ腫」「marginal zone B-cell lymphoma」「lymphoma of mucosa-associated lymphoid tissue」
関連記事	「associate」「associated」「lymphoid」「lymphoid tissue」

「MALTリンパ腫」

　　[★]

英

mucosa-associated lymphoid tissue lymphoma MALToma

同

粘膜関連リンパ組織リンパ腫

関

胃MALTリンパ腫、悪性リンパ腫

概念

• 比較的緩徐な経過をたどる低悪性度リンパ腫

リンパ濾胞(リンパ小節)の胚中心：{被膜側}頂部明調域(生存を許された分化したB細胞がとどまる。記憶細胞か形質細胞になってリンパ小節を去る)～基底側明調域(sIgを発現しクラススイッチを起こして中心細胞となる。中心細胞は濾胞樹状細胞から抗原提示をうける。)～暗調域(sIgを持たないB細胞(中心芽細胞)が密集。増殖盛ん)～帽状域{中心側})

• リンパ濾胞外側領域(おそらく胚中心の頂部明調領域であろう)のB細胞層にある記憶B細胞に由来する。
• 形態は小型胚中心細胞に似ており、広く明調な細胞質と切れ込みを認める。

原因

• 胃MALTリンパ腫

• Helicobacter pyloriの感染
• 染色体異常：t(11;18)(q21;q21)

病理

• 胃MALTリンパ腫の場合、胃癌0-IIc型(表面感凹型)に似ている。
• 胃炎型の粘膜は萎縮性胃炎像、多発びらん、顆粒状、敷石状粘膜

細胞表面マーカー

• CD5 -、CD10 -、CD19 +、CD23 -、CD43 -/+、sIgG -、sIgM +

特徴 YN.A-46

• 胚中心細胞に類似したcentrocyte-like cellの浸潤
• 粘膜上皮の破壊像
• 単クローン性免疫グロブリンを有する形質細胞の浸潤
• リンパ濾胞の残存

検査

染色体

• t(11;18)(q21;q21)

API2(11q21)、MALT1(18q21)

診断

• 胃生検

治療

ヘリコバクター・ピロリ菌の除菌により消失することがある

• 薬物療法：

• 抗菌薬：ヘリコバクターの除菌　→　第一選択とすることが推奨されている(参考1)

• 除菌治療の有効性予測因子：　H. pylori感染の有無、臨床病期、深達度、API2-MALT1 染色体転座　(参考1)

• 抗悪性腫瘍薬：(病変が広範囲に及んでいる場合 more extensive disease)chlorambucil単剤。rituximabを加えて使用するレジメンもある。(HIM.694)
• 放射線療法
• リツキシマブ

• 手術療法：

• (病変が限局している場合)有効

up2date

Management of gastrointestinal lymphomas (This topic last updated: 6月 2, 2010)

• (Lugano I/II)

• H. pylori陽性：H.pylori除菌を行う
• H. pylori陰性 or t(11;18)転座：局所放射線療法

• (Lugano IV)

• H. pylori陽性：H.pylori除菌を行う。免疫療法や化学療法が必要になるまで症状が進むまで経過観察。

• 消化管の切除は消化管穿孔や消化管閉塞を合併するまでreserveされる。

予後

• リンパ節転移は少なく、臨床的進行は遅い。
• 中間～良好 (LAB.1219)

参考

• 1. Helicobacter pylori 感染の診断と治療のガイドライン2009改訂版 - 日本ヘリコバクター学会

http://www.jshr.jp/pdf/journal/guideline2009-2.pdf

• 2. 写真

http://pathol.ncc.go.jp/matsuno/images/matsuno-43.htm

• 3. 写真

http://user.shikoku.ne.jp/hirata/geca/sub48.html

「marginal zone B-cell lymphoma」

　　[★]

辺縁帯B細胞リンパ腫

関

lymphoma of mucosa-associated lymphoid tissue、MALT lymphoma、mucosa-associated lymphoid tissue lymphoma

「lymphoma of mucosa-associated lymphoid tissue」

　　[★] 粘膜関連リンパ組織リンパ腫

関

MALT lymphoma、marginal zone B-cell lymphoma、mucosa-associated lymphoid tissue lymphoma

「associate」

　　[★]

• vt.

• 結合する、連合させる。連想によって結びつける。(人を)仲間/友達などとしての関係に置く。(化)会合させる

• vi.

• 仲間/友人などとして交際する(with)。一つにまとめる、連合する

• n.

• 仕事仲間、提携者、同僚。友人、朋友。準会員。従業員、社員
• 密接な蒸す偽付きのある物、付随する物、つきもの。準国家
• 連想によって心に浮かぶ言葉/思い、連想物

• adj.

• 仲間の、同僚の。製紙機械員に次ぐ資格の
• 付随する、連想される

関

accompany、association、associative、attach、bearing、bind、binding、bond、bonding、coassemble、colleague、combine、conjoin、conjugate、conjugation、conjunction、connect、connection、correlate、correlation、couple、dock、engage、engagement、federation、implication、join、joint、ligate、link、linkage、peer、pertinent、reference、relate、relation、relationship、relative、relevance、relevant、symphysial、union

　 　 　 　 　 　

「associated」

　　[★]

• adj.

• 随伴の、随伴性の

関

concomitant、erratic、erratically

associate

「lymphoid」

　　[★]

• adj.

• リンパ様、リンパ系の

関

lymphatic system

　 　

「lymphoid tissue」

　　[★]

リンパ組織、リンパ系組織

関

lymphatic tissue