9827/3 Adult T-cell leukemia/lymphoma (HTLV-1 positive)


Synonyms

Adult T-cell lymphoma/leukemia
Adult T-cell lymphoma
Adult T-cell leukemia

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C91.5
           

/span> peripheral T-cell neoplasm most often composed of highly pleomorphic lymphoid cells. The disease is usually widely disseminated, and is caused by the human retrovirus known as human T-cell leukaemia virus type I (HTLV-I) (Table 11.01).

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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

A peripheral (mature) T-cell neoplasm caused by the human T-cell leukemia virus type 1 (HTLV-1). Adult T-cell lymphoma/leukemia is endemic in several regions of the world, in particular Japan, the Caribbean, and parts of Central Africa
1
 
Jaffe ES, Harris NL, Stein H, Vardiman JW (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues.
3rd Edition
IARC Press: Lyon 2001



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Primary gastric T-cell lymphomas are rare and heterogeneous.
> Adult T-cell leukaemia/lymphoma is common in areas of endemic infection with human T-cell leukaemia virus 1 (HTLV-1) infection, representing up to 7% of gastric lymphomas
2
Click to access Pubmed
Nakamura S, Matsumoto T, Iida M, Yao T, Tsuneyoshi M (2003)
Primary gastrointestinal lymphoma in Japan: a clinicopathologic analysis of 455 patients with special reference to its time trends.
Cancer 97: 2462-73



3
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Shimada-Hiratsuka M, Fukayama M, Hayashi Y, Ushijima T, Suzuki M, Hishima T, Funata N, Koike M, Watanabe T (1997)
Primary gastric T-cell lymphoma with and without human T-lymphotropic virus type 1.
Cancer 80: 292-303



. The lymphoma cells are typically CD4+, CD25+, and CCR4+ in addition to carrying pan T-cell markers. No cytotoxic granule-associated proteins are detected 4
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Hatano B, Ohshima K, Katoh A, Kanda M, Kawasaki C, Tsuchiya T, Shimazaki K, Haraoka S, Sugihara M, Suzumiya J, Kikuchi M (2002)
Non-HTLV-1-associated primary gastric T-cell lymphomas show cytotoxic activity: clinicopathological, immunohistochemical characteristics and TIA-1 expression in 31 cases.
Histopathology 41: 421-36



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> In areas where HTLV-1 infection is not endemic, most primary gastric T-cell lymphomas have a cytotoxic T-cell phenotype, similar to that seen in enteropathy associated T-cell lymphomas
5
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Lee SS, Jang JJ, Cho KJ, Khang SK, Kim CW (1997)
Epstein-Barr virus-associated primary gastrointestinal lymphoma in non-immunocompromised patients in Korea.
Histopathology 30: 234-42



6
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Yun WK, Ko YH, Kim DS, Kim WS, Rhee PL, Kwak SY, Jang TH, Choi WS (2008)
Composite marginal zone B cell lymphoma and enteropathy-type T cell lymphoma of the stomach: a case report.
Eur J Gastroenterol Hepatol 20: 791-5



7
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Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK (2007)
Enteropathy-type T-cell lymphoma.
Am J Clin Pathol 127: 701-6



. The neoplastic cells may display features of intraepithelial T-lymphocyte differentiation (e.g. expression of the human mucosal lymphocyte 1 antigen, CD103). These lymphomas may occur sporadically outside the setting of coeliac disease.
>Extranodal natural killer/ T-cell (NK/T-cell) lymphoma of nasal type (EBV+) is also seen in east Asian countries
8
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Chuang SS, Chang ST, Chuang WY, Huang WT, Hsieh PP, Tsou MH, Liao YL, Lin SH, Hsieh YC, Lu CL, Sheu MJ, Liu H (2009)
NK-cell lineage predicts poor survival in primary intestinal NK-cell and T-cell lymphomas.
Am J Surg Pathol 33: 1230-40



9
Click to access Pubmed
Shimada-Hiratsuka M, Fukayama M, Hayashi Y, Ushijima T, Suzuki M, Hishima T, Funata N, Koike M, Watanabe T (1997)
Primary gastric T-cell lymphoma with and without human T-lymphotropic virus type 1.
Cancer 80: 292-303



. Nasal-type NK/T-cell lymphomas and enteropathy-associated T-cell lymphomas have in common the expression of CD56 (NCAM1) and cytotoxic molecules such as granzyme B, TIA1, and perforin in addition to pan-T-cell markers. Therefore, the presence or absence of EBV is mandatory for their distinction. Some of the remainder are similar to peripheral T-cell lymphomas encountered in lymph nodes, including anaplastic large cell lymphoma that expresses anaplastic lymphoma kinase (ALK+)10
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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