adult T-cell leukemia

成人T細胞白血病 ATL

WordNet

1. small room in which a monk or nun lives (同)cubicle
2. a device that delivers an electric current as the result of a chemical reaction (同)electric cell
3. a room where a prisoner is kept (同)jail cell, prison cell
4. (biology) the basic structural and functional unit of all organisms; they may exist as independent units of life (as in monads) or may form colonies or tissues as in higher plants and animals
5. any small compartment; "the cells of a honeycomb"
6. a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
7. any mature animal
8. a fully developed person from maturity onward (同)grownup
9. (of animals) fully developed; "an adult animal"; "a grown woman" (同)big, full-grown, fully grown, grown, grownup
10. malignant neoplasm of blood-forming tissues; characterized by abnormal proliferation of leukocytes; one of the four major types of cancer (同)leukaemia, leucaemia, cancer of the blood
11. the 20th letter of the Roman alphabet (同)t
12. a tough youth of 1950s and 1960s wearing Edwardian style clothes (同)Teddy boy

PrepTutorEJDIC

1. (刑務所の)『独房』;(修道院の)小さい独居室 / (ミツバチの)みつ房,巣穴 / 小さい部屋 / 『細胞』 / 電池 / 花粉室 / (共産党などの)細胞
2. 『おとなの』,成人した,成熟した / 成人向きの / 『おとな』,成人
3. 白血病
4. tritium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）』「2015/05/23 18:05:41」(JST)

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Adult T-cell leukemia/lymphoma
Classification and external resources
ICD-10	C83-C88
ICD-9	204.0-208.9
ICD-O	M9827/3
DiseasesDB	29486
MeSH	D015459

Adult T-cell leukemia/lymphoma (ATL or ATLL) is a rare cancer of the immune system's own T-cells.

Human T cell leukemia/lymphotropic virus type 1 (HTLV-1) is believed to be the cause of it, in addition to several other diseases.

Signs and symptoms

ATL is usually a highly aggressive non-Hodgkin's lymphoma with no characteristic histologic appearance except for a diffuse pattern and a mature T-cell phenotype. Circulating lymphocytes with an irregular nuclear contour (leukemic cells) are frequently seen. Several lines of evidence suggest that HTLV-1 causes ATL. This evidence includes the frequent isolation of HTLV-1 from patients with this disease and the detection of HTLV-1 proviral genome in ATL leukemic cells. ATL is frequently accompanied by visceral involvement, hypercalcemia, skin lesions, and lytic bone lesions. Bone invasion and osteolysis, features of bone metastases, commonly occur in the setting of advanced solid tumors, such as breast, prostate, and lung cancers, but are less common in hematologic malignancies. However, patients with HTLV-1–induced ATL and multiple myeloma are predisposed to the development of tumor-induced osteolysis and hypercalcemia. One of the striking features of ATL and multiple myeloma induced bone disease is that the bone lesions are predominantly osteolytic with little associated osteoblastic activity. In patients with ATL, elevated serum levels of IL-1, TGFβ, PTHrP, macrophage inflammatory protein (MIP-1α), and receptor activator of nuclear factor-κB ligand (RANKL) have been associated with hypercalcemia. Immunodeficient mice that received implants with leukemic cells from patients with ATL or with HTLV-1–infected lymphocytes developed hypercalcemia and elevated serum levels of PTHrP. Most patients die within one year of diagnosis.

Infection with HTLV-1, like infection with other retroviruses, probably occurs for life and can be inferred when antibody against HTLV-1 is detected in the serum.

Transmission

Transmission of HTLV-1 is believed to occur from mother to child; by sexual contact; and through exposure to contaminated blood, either through blood transfusion or sharing of contaminated needles.

Treatment

Treatment options that have been tried include zidovudine and the CHOP regimen. Pralatrexate has also been investigated. Most therapy is directed towards the cancer rather than the virus itself. Recently, it has been reported that the traditional glucocorticoid-based chemotherapy toward ATL are largely mediated by thioredoxin binding protein-2 (TBP-2/TXNIP/VDUP1), suggesting the potential use of a TBP-2 inducer as a novel therapeutic target.

Recently, mogamulizumab, has been approved for the treatment of ATL in Japan.

At a medical conference in December 2013, researchers reported anywhere from 21-50% of ATL patients have disease expressing CD30. This suggests treatment with CD30-targeting brentuximab vedotin may be beneficial.

Epidemiology

HTLV-1 infection in the United States appears to be rare. Although little serologic data exist, prevalence of infection is thought to be highest among blacks living in the Southeast. A prevalence rate of 30% has been found among black intravenous drug abusers in New Jersey, and a rate of 49% has been found in a similar group in New Orleans. It is possible that prevalence of infection is increasing in this risk group. Studies of HTLV-1 antibody indicate that the virus is endemic in southern Japan, in the Caribbean, South America, and in Africa.

ATL is relatively uncommon among those infected with HTLV-1. The overall incidence of ATL is estimated at about 1 per 1,500 adult HTLV-1 carriers per year. Those cases that have been reported have occurred mostly among persons from the Caribbean or blacks from the Southeast (National Institutes of Health, unpublished data). There appears to be a long latent period between HTLV-1 infection and the start of ATL.

Research

Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation. Pralatrexate is one compound currently under investigations for the treatment of PTCL.

In pregnancy

Leukemia is rarely associated with pregnancy, affecting only about 1 in 10,000 pregnant women. How it is handled depends primarily on the type of leukemia. Acute leukemias normally require prompt, aggressive treatment, despite significant risks of pregnancy loss and birth defects, especially if chemotherapy is given during the developmentally sensitive first trimester.

References

External links

• Franchini, Genoveffa; Nicot, Christophe; Johnson, Julie M. (2003). "Seizing of T Cells by Human T-Cell Leukemia/Lymphoma Virus Type 1". In Vande Woude, George F.; Klein, George. Advances in Cancer Research 89. pp. 69–132. doi:10.1016/S0065-230X(03)01003-0. ISBN 978-0-12-006689-6. PMID 14587871. 
• Centers for Disease Control (CDC) (1987). "Adult T-cell leukemia/lymphoma associated with human T-lymphotropic virus type I (HTLV-I) infection--North Carolina". Morbidity and Mortality Weekly Report 36 (49): 804–6, 812. PMID 2891025. 
• Genoveffa Franchini's NCI page: Human Retroviral Diseases: Pathogenesis and Prevention
• International Retrovirology Association

UpToDate Contents

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• 1. 成人T細胞性白血病/リンパ腫の治療及び予後 treatment and prognosis of adult t cell leukemia lymphoma
• 2. 成人T細胞性白血病/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 adult t cell leukemia lymphoma
• 3. ヒトTリンパ球好性ウイルスI型：疾患関連、診断、および治療 human t lymphotropic virus type i disease associations diagnosis and treatment
• 4. Sézary症候群の臨床症状、病理学的特徴、および診断 clinical presentation pathologic features and diagnosis of sezary syndrome
• 5. 末梢性T細胞リンパ腫の初期治療 initial treatment of peripheral t cell lymphoma

English Journal

• Alpha2beta1 integrin promotes T cell survival and migration through the concomitant activation of ERK/Mcl-1 and p38 MAPK pathways.

• Naci D1, Aoudjit F2.
• Cellular signalling.Cell Signal.2014 Sep;26(9):2008-15. doi: 10.1016/j.cellsig.2014.05.016. Epub 2014 May 29.
• Integrin-mediated attachment to extracellular matrix (ECM) is crucial for cancer progression. Malignant T cells such as acute lymphoblastic leukemia (T-ALL) express β1 integrins, which mediate their interactions with ECM. However, the role of these interactions in T-ALL malignancy is still poorly e
• PMID 24880062

• Microarray analysis of gene expression by microdissected epidermis and dermis in mycosis fungoides and adult T-cell leukemia/lymphoma.

• Hashikawa K1, Yasumoto S1, Nakashima K2, Arakawa F2, Kiyasu J2, Kimura Y2, Saruta H1, Nakama T1, Yasuda K3, Tashiro K4, Kuhara S4, Hashimoto T1, Ohshima K2.
• International journal of oncology.Int J Oncol.2014 Sep;45(3):1200-8. doi: 10.3892/ijo.2014.2524. Epub 2014 Jun 26.
• The characteristic histopathological feature of mycosis fungoides (MF) and adult T-cell leukemia/lymphoma (ATLL) is epidermotropism. To identify the mechanism for epidermotropism of lymphoma cells, total RNAs were obtained from skin biopsies of epidermis and dermis of MF and ATLL patients by means o
• PMID 24970722

• Therapeutic potential of targeting mTOR in T-cell acute lymphoblastic leukemia (Review).

• Evangelisti C1, Evangelisti C2, Chiarini F1, Lonetti A2, Buontempo F2, Bressanin D2, Cappellini A3, Orsini E2, McCubrey JA4, Martelli AM2.
• International journal of oncology.Int J Oncol.2014 Sep;45(3):909-18. doi: 10.3892/ijo.2014.2525. Epub 2014 Jun 26.
• T-cell acute lymphoblastic leukemia (T-ALL) is a heterogeneous neoplastic disorder of immature hematopoietic precursors committed to the T-cell lineage. T-ALL comprises about 15% of pediatric and 25% of adult ALL cases. Even if the prognosis of T-ALL has improved especially in the childhood due to t
• PMID 24968804

Japanese Journal

• Cord Blood Transplantation Provided Long-term Remission in a Case of Adult T-cell Leukemia-lymphoma (ATL) with Myelofibrosis

• Internal Medicine 55(2), 197-201, 2016
• NAID 130005119768

• 症例 モガムリズマブ治療中の成人T細胞白血病リンパ腫患者に生じたSweet病様皮疹の1例

• 皮膚科の臨床 57(13), 2030-2032, 2015-12
• NAID 40020682494

• 成人T細胞白血病/リンパ腫におけるトリプトファン代謝の予後に関する意義 (第75回日本血液学会学術集会 学会奨励賞受賞論文)

• 臨床血液 56(11), 2295-2304, 2015-11
• NAID 40020667965

Related Links

• Adult T-cell leukemia-lymphoma. - National Center for ...

Abstract Adult T-cell leukemia-lymphoma, an aggressive T-cell leukemia, is characterized by the presence in the peripheral blood of malignant T cells that have highly indented or lobulated nuclei. Phenotypically the cells are usually ...

• Adult T-Cell Leukemia/Lymphoma (HTLV-1) - Home ...

Adult T-Cell Leukemia/Lymphoma (HTLV-1) What is Lymphoma? Lymphoma is a cancer of the white blood cells, namely lymphocytes, that constitute the lymphatic system. The two main types of lymphoma are Hodgkin lymphoma

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★リンクテーブル★

リンク元	「成人T細胞白血病」「T-cell acute lymphoblastic leukemia」「T-ALL」「T-cell acute lymphocytic leukemia」「acute T-cell leukemia」
拡張検索	「adult T-cell leukemia-lymphoma」「adult T-cell leukemia-derived factor」「adult T-cell leukemia/lymphoma」
関連記事	「adult」「T」「cell」

「成人T細胞白血病」

　　[★]

英

adult T cell leukemia, adult T-cell leukemia ATL

同

成人T細胞白血病リンパ腫 adult T-cell leukemia/lymphoma ATLL

関

ヒトTリンパ球向性ウイルス

特徴

• CD4陽性Tリンパ球に感染して白血病/リンパ腫を起こす　　→　　成熟したT細胞に感染し、それが腫瘍性に増殖

(CD2⁺, CD3⁺, CD4⁺, CD25⁺, HLA-DR⁺, CD8^-)

病型

• 急性型、慢性型、くすぶり型、リンパ腫型

	急性型	リンパ腫型	慢性型	くすぶり型
典型的な症状	腫瘍随伴症状(腫瘍熱、高カルシウム血症(意識障害・脱水・腎機能障害) (50%で認められる))が初発症状であることが多い。 臓器浸潤による症状(消化管浸潤(下痢・腹痛)、肝臓浸潤(黄疸)、 肺浸潤(呼吸困難)、中枢神経浸潤(脳神経症状))	リンパ節腫大は全例で認められる。 腫瘍熱、高カルシウム血症を初発症状とする場合もある。 末梢血中の形態的異常細胞は1%以下	多くの場合、自覚症状を欠き、健康診断などで偶然診断される。 日和見感染症や腫瘍の浸潤に伴う皮膚症状(皮疹、皮膚腫瘤)、 肺病変(労作時息切れ、咳嗽)が診断の発端となる場合もある。	リンパ球増多を伴わないため、血液検査では診断に至らない。 無症候キャリアーと同様、多くの患者では症状を欠く。
PS>1	67.6％	45.4％	27.2％	22.7％
感染性併発症	26.9％	10.9％	35.5％	35.6％
皮膚病変	40.2％	25％	46.1％	48.9％
肺病変	20.2％	10.9％	15.1％	15.6％
肝腫大	35.9％	16％	13.8％	0％
脾腫大	29.9％	15.4％	11.2％	0％
4個以上の腫大リンパ節	80.7％	62.2％	54％	6.7％
高カルシウム血症	50.3％	16.7％	0％	0％
貧血(Hb<10g/dl)	10.1％	4.5％	4％	0％
血小板減少(10万/ul)	19.4％	4.5％	2％	6.7％

疫学

• 日本に約120万人の感染者
• 男性に多い。
• 1000-2000人に1。50歳以上で発症(平均58歳)。

病原体

• ヒトTリンパ球向性ウイルス HTLV-1

感染経路

• 母乳感染　←　哺乳の停止・人工乳による感染防止
• 輸血
• 性交

病理

参考1

• 末梢血、骨髄：花弁状、脳回転状に分葉した異型細胞　→　ATL細胞：CD4陽性、CD8陰性

[show details]

• リンパ節
• その他：(皮膚)皮膚T細胞リンパ腫のように表皮向性をもち、ポートリエ微小膿瘍を伴って皮膚に浸潤することがある。このばあい、緩徐進行型の経過をたどる
• 免疫表現型
• 遺伝子：決まった核型異常や遺伝子異常はない。

病態

• ATL細胞はPTHrP、IL-1α、TNF-βを産生　→　高カルシウム血症、全身症状？(発熱、全身倦怠感など)
• 正常T細胞の減少　→　易感染性

症状

• 初発症状は皮膚症状、リンパ節腫脹、肝脾腫、高カルシウム血症など。貧血や出血症状の頻度は少ない。
• 皮膚症状 → PMID: 24438140 [1]
• リンパ節腫脹
• 肝脾腫
• 高カルシウム血症：ATL細胞の産生する副甲状腺ホルモン関連蛋白質(PTHrP)により破骨細胞の活性化が起こるため。

検査

• 血液塗沫

• 花弁状の腫瘍細胞

合併症

• HTLV-1 associated myelopath(HAM) = tropical spastic paraparesis(TSP) →まとめて「HAM/TSP」と呼ばれることがある。

治療

• 慢性(進行型を除く)・くすぶり型

• 経過観察

• 急性・リンパ腫型・慢性進行型

予後

• 急性型とリンパ腫型は予後不良
• LDH、血清Ca濃度が予後と関連(PMID 16122659)

参考

• 1. [charged] Clinical manifestations, pathologic features, and diagnosis of adult T cell leukemia-lymphoma - uptodate [2]

国試

• 103I007

「T-cell acute lymphoblastic leukemia」

　　[★] T細胞性急性リンパ性白血病、T細胞性急性リンパ芽球性白血病

関

acute T-cell leukemia、adult T-cell leukemia、adult T-cell leukemia-lymphoma、ATLL、HTLV-I-associated acute T-cell leukemia-lymphoma、T-ALL、T-cell acute lymphocytic leukemia

「T-ALL」

　　[★] T細胞性急性リンパ性白血病

関

acute T-cell leukemia、adult T-cell leukemia、adult T-cell leukemia-lymphoma、ATLL、HTLV-I-associated acute T-cell leukemia-lymphoma、T-cell acute lymphoblastic leukemia、T-cell acute lymphocytic leukemia

「T-cell acute lymphocytic leukemia」

　　[★] T細胞性急性リンパ性白血病

関

acute T-cell leukemia、adult T-cell leukemia、adult T-cell leukemia-lymphoma、ATLL、HTLV-I-associated acute T-cell leukemia-lymphoma、T-ALL、T-cell acute lymphoblastic leukemia

「acute T-cell leukemia」

　　[★] 急性T細胞白血病

関

adult T-cell leukemia、adult T-cell leukemia-lymphoma、ATLL、HTLV-I-associated acute T-cell leukemia-lymphoma、T-ALL、T-cell acute lymphoblastic leukemia、T-cell acute lymphocytic leukemia

「adult T-cell leukemia-lymphoma」

　　[★] 成人T細胞白血病リンパ腫、成人T細胞性白血病リンパ腫

関

acute T-cell leukemia、adult T-cell leukemia、ATLL、HTLV-I-associated acute T-cell leukemia-lymphoma、T-ALL、T-cell acute lymphoblastic leukemia、T-cell acute lymphocytic leukemia

「adult T-cell leukemia-derived factor」

　　[★] ヒトチオレドキシン。成人T細胞白血病由来因子

「adult T-cell leukemia/lymphoma」

　　[★] 成人T細胞白血病リンパ腫 ATLL

「adult」

　　[★]

• n.

• 成人、成体、大人

• adj.

• 成体型の、成人型の、大人の、成人性の

関

adult human、adult type

　 　 　 　

「T」

　　[★]

• T波 T wave
• チミン thymine
• ツイスト数
• スレオニントレオニン threonine

　 　

「cell」

　　[★] 細胞