WordNet

small room in which a monk or nun lives (同)cubicle
a device that delivers an electric current as the result of a chemical reaction (同)electric cell
a room where a prisoner is kept (同)jail cell, prison cell
(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
any small compartment; "the cells of a honeycomb"
a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
any mature animal
a fully developed person from maturity onward (同)grownup
(of animals) fully developed; "an adult animal"; "a grown woman" (同)big, full-grown, fully grown, grown, grownup
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
the 20th letter of the Roman alphabet (同)t
a neoplasm of lymph tissue that is usually malignant; one of the four major types of cancer

PrepTutorEJDIC

(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
『おとなの』,成人した,成熟した / 成人向きの / 『おとな』,成人
白血病
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）』「2015/12/05 11:32:58」(JST)

wiki en

Adult T-cell leukemia/lymphoma (ATL or ATLL) is a rare cancer of the immune system's own T-cells.^[1]^[2]^[3]

Human T cell leukemia/lymphotropic virus type 1 (HTLV-1) is believed to be the cause of it,^[4] 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.^[5] Most patients die within one year of diagnosis.^[6]

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.^{[citation needed]}

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.^[7]

Treatment

Treatment options that have been tried include zidovudine and the CHOP regimen.^[8] Pralatrexate has also been investigated.^[9] Most therapy is directed towards the cancer rather than the virus itself.^{[citation needed]} 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.^[10]^[11]

Recently, mogamulizumab, has been approved for the treatment of ATL in Japan.^[12]

At a medical conference in December 2013, researchers reported anywhere from 21-50% of ATL patients have disease expressing CD30.^[13] 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.^{[citation needed]}

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.^{[citation needed]}

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.^{[citation needed]}

In pregnancy

Leukemia is rarely associated with pregnancy, affecting only about 1 in 10,000 pregnant women.^[14] 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.^[14]

References

^ Yodoi, J; Takatsuki, K; Masuda, T (1974). "Still More on NBT Technic". New England Journal of Medicine 290 (10): 572–3. doi:10.1056/NEJM197403072901018. PMID 4544052.
^ Uchiyama, T; Yodoi, J; Sagawa, K; Takatsuki, K; Uchino, H (1977). "Adult T-cell leukemia: Clinical and hematologic features of 16 cases". Blood 50 (3): 481–92. PMID 301762.
^ Yodoi, J; Maeda, M (2011). "The discovery of ATL: an odyssey in restrospect". International Journal of Hematology 94 (5): 423–8. doi:10.1007/s12185-011-0957-x. PMID 22068231.
^ Nicot, Christophe (2005). "Current views in HTLV-I-associated adult T-cell leukemia/lymphoma". American Journal of Hematology 78 (3): 232–9. doi:10.1002/ajh.20307. PMID 15726602.
^ bloodjournal.hematologylibrary.org/content/106/13/4294.full HTLV-1 Tax transgenic mice develop spontaneous osteolytic bone metastases prevented by osteoclast inhibition
^ "Treatment and prognosis of adult T cell leukemia-lymphoma". Retrieved 27 July 2012.
^ "HTLV-1: CLINICAL IMPACT OF A CHRONIC INFECTION". Retrieved 22 July 2013.
^ Taylor, Graham P; Matsuoka, Masao (2005). "Natural history of adult T-cell leukemia/lymphoma and approaches to therapy". Oncogene 24 (39): 6047–57. doi:10.1038/sj.onc.1208979. PMID 16155611.
^ Marneros, A. G.; Grossman, M. E.; Silvers, D. N.; Husain, S.; Nuovo, G. J.; Macgregor-Cortelli, B.; Neylon, E.; Patterson, M.; O'Connor, O. A. (2009). "Pralatrexate-induced tumor cell apoptosis in the epidermis of a patient with HTLV-1 adult T-cell lymphoma/leukemia causing skin erosions". Blood 113 (25): 6338–41. doi:10.1182/blood-2009-03-210989. PMID 19389878.
^ Chen, Z; Lopez-Ramos, D (2011). "Thioredoxin-binding protein-2 (TBP-2/VDUP1/TXNIP) regulates T-cell sensitivity to glucocorticoid during HTLV-I-induced transformation.". Leukemia 25 (3): 440–8. doi:10.1038/leu.2010.286. PMC 3072512. PMID 21151022.
^ Chen, Z; Yoshihara E (2010). "Differential roles of Annexin A1 (ANXA1/lipocortin-1/lipomodulin) and thioredoxin binding protein-2 (TBP-2/VDUP1/TXNIP) in glucocorticoid signaling of HTLV-I-transformed T cells.". Immunology Letters 131 (1): 11–18. doi:10.1016/j.imlet.2010.04.003. PMID 20398702.
^ Subramaniam, JM; Whiteside, G; McKeage, K; Croxtall, JC (June 2012). "Mogamulizumab: first global approval.". Drugs 72 (9): 1293–8. doi:10.2165/11631090-000000000-00000. PMID 22686619.
^ Campuzano-Zuluaga, G; Pimentel, A; Diaz, L; Chapman-Fredricks, JR; and Ramos, JC " CD30 Expression Is Associated With Decreased Survival In Patients With Acute and Unfavorable Chronic Types Of Adult T-Cell Leukemia-Lymphoma" December 2013 https://ash.confex.com/ash/2013/webprogram/Paper64702.html
^ ^a ^b Shapira T, Pereg D, Lishner M (September 2008). "How I treat acute and chronic leukemia in pregnancy". Blood Rev. 22 (5): 247–59. doi:10.1016/j.blre.2008.03.006. PMID 18472198.

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
2. 成人T細胞性白血病/リンパ腫の治療および予後 treatment and prognosis 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. 末梢性T細胞リンパ腫の初期治療 initial treatment of peripheral t cell lymphoma
5. 好酸球の生物学および好酸球増多症の原因 eosinophil biology and causes of eosinophilia

English Journal

Interferon regulatory factor-4 activates IL-2 and IL-4 promoters in cooperation with c-Rel.

Shindo H, Yasui K, Yamamoto K, Honma K, Yui K, Kohno T, Ma Y, Chua KJ, Kubo Y, Aihara H, Ito T, Nagayasu T, Matsuyama T, Hayashi H.SourceDivision of Cytokine Signaling, Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki 852-8523, Japan; Division of Surgical Oncology, Department of Translational Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki 852-8523, Japan.
Cytokine.Cytokine.2011 Dec;56(3):564-72. Epub 2011 Sep 3.
Interferon regulatory factor (IRF)-4 is a member of the IRF transcription factor family, whose expression is primarily restricted to lymphoid and myeloid cells. In T-cells, IRF-4 expression is induced by T-cell receptor (TCR) cross-linking or treatment with phorbol-12-myristate-13-acetate (PMA)/Iono
PMID 21890374

Vesiculobullous variant of adult T-cell leukemia/lymphoma in a Caribbean émigré.

Mouzakis J, Black W, Messina J, Cherpelis B.AbstractAdult T-cell leukemia/lymphoma (ATLL) results from human T-cell lymphotropic virus (HTLV) type I infection and may present as a diverse array of cutaneous findings. Often these clinical manifestations are non-specific and overlap significantly with cutaneous T-cell lymphoma (CTCL). However, it is exceedingly rare for a patient suffering from ATLL to develop vesicular or bullous pathology and only a handful of such cases have been reported in the literature. The authors describe a patient of Jamaican descent afflicted with ATLL who developed an impressive vesiculobullous eruption. This case provides further support of the near complete clinical overlap between ATLL and CTCL. Patients from HTLV endemic areas with consistent clinical manifestations should have viral serologies drawn as the treatment and prognosis of ATLL and CTCL differ greatly. J Drugs Dermatol. 2011;10(12):1469-1471.
Journal of drugs in dermatology : JDD.J Drugs Dermatol.2011 Dec 1;10(12):1469-71.
Adult T-cell leukemia/lymphoma (ATLL) results from human T-cell lymphotropic virus (HTLV) type I infection and may present as a diverse array of cutaneous findings. Often these clinical manifestations are non-specific and overlap significantly with cutaneous T-cell lymphoma (CTCL). However, it is ex
PMID 22134574

Japanese Journal

HTLV-1 bZIP Factor Enhances T-Cell Proliferation by Impeding the Suppressive Signaling of Co-inhibitory Receptors

PLoS Pathogens 13, 2017-01-03
NAID 120005973331

ATL異種移植モデルにおけるAKT signaling pathwayの役割

血液内科 = Hematology 74(1), 99-102, 2017-01
NAID 40021065728

ATLに対するHTLV-1 Tax標的樹状細胞ワクチン療法の現状 (特集血液腫瘍に対する免疫療法の新たな展開)

血液内科 = Hematology 74(1), 13-18, 2017-01
NAID 40021065370

Related Pictures

Adult T-cell leukemia / lymphoma Adult T-cell Leukemia/Lymphoma - 2.

★リンクテーブル★

リンク元	「成人T細胞白血病」「成人T細胞白血病リンパ腫」「ATLL」「成人T細胞白血病/リンパ腫」
関連記事	「adult」「T」「cell」

「成人T細胞白血病」

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

　　[★]

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％