ATCL | FlowcytometryNet
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Adult T‐cell leukaemia/lymphoma (ATLL)

Adult T‐cell leukaemia/lymphoma (ATLL) is a mature T‐cell neoplasm of post‐thymic lymphocytes aetiologically linked to the human T‐cell lymphotropic virus, HTLV‐I and  found in the blood (leukemia), lymph nodes (lymphoma), skin, or multiple areas of the body.. The cytological picture is pleomorphic, with the predominant cell being a medium size lymphocyte with condensed chromatin and a convoluted or polylobated nucleus, designated a “flower cell”,nucleoli are not visible, and when identified are often small.

Immunophentyping:

The immunophenotype of the ATLL cell is that of an activated mature T‐lymphocyte.

positive for CD2, CD5, CD4, CD3, CD25(identifies alpha chain of the interleukin‐2  orIL‐2) ,T‐cell receptor (TCR)‐β (weak expression or even negative in the cell membrane)(few cases of CD4+CD8+ and CD4-CD8+ cases also seen with  worse clinical course), variable expression of HLA-DR, CD38 and CD56  and Negative for CD7, CD7, CD8, ALK, cytotoxic molecules, CD10, CXCL13, TCL1, NK cells, CD15, epithelial membrane antigen (EMA) and the anaplastic lymphoma kinase (ALK1)

Reference:

  • Kikuchi M, Jaffe E S, Ralfkiaer E. Adult T‐cell leukaemia/lymphoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. World Health Organization classification of tumours. Tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press, 2001200–203.

  • Matutes E, Catovsky D. Adult T cell leukaemia lymphoma. In: Whittaker JA, ed. Leukaemia, 3rd edn. Oxford: Blackwell Scientific Publications 1998416–433.

  • Shimomaya M. Diagnostic criteria and clinical subtypes of ATLL. A report from the Lymphoma Study Group (1984–87). Br J Haematol 199179428–437. [PubMed]

  • Hattori T, Asou N, Suzushima H. et al Leukaemia of novel gastrointestinal T‐lymphocyte population infected with HTLV‐I. Lancet 199133776–77. [PubMed]

  • Cabrera M E, Labra S, Ford A. et al HTLV‐I induced intestinal lymphoma. Leuk Lymphoma 199935637–640. [PubMed]

  • Dungerwalla M, Osuji N, Waldman A D. et al Isolated central nervous system involvement in adult T‐cell lymphoma/leukaemia. Br J Haematol 2005130511–515. [PubMed]

  • Taylor G. Molecular aspects of TLV‐I infection and ATLL. J Clin Pathol 2007601392–1396. [PMC free article] [PubMed]

  • Pombo de Oliveira M S, Matutes E, Famadas L C. et al Adult T‐cell leukaemia/lymphoma in Brazil and its relation to HTLV‐I. Lancet 1990336987–990. [PubMed]

  • Yamaguchi K, Sung Yul L, Shimizu T. et al Concurrence of lymphoma type adult T‐cell leukemia in three sisters. Cancer 1985561688–1690. [PubMed]

  • Matutes E, Spittle M F, Smith N P. et al The first report of familial adult T‐cell leukaemia lymphoma in the United Kingdom. Br J Haematol 199589615–619. [PubMed]

  • Cartier L, Castillo J L, Cabrera M E. et al HTLV‐I positive spastic paraparesis (TSP) associated with a lymphoid disorder in three Chilean patients. Leuk Lymphoma 199517459–464. [PubMed]

  • Aouba A, Lambotte O, Vasiliu V. et al Hemophagocytic syndrome as a presenting sign of transformation of smouldering to acute adult T‐cell leukemia/lymphoma: efficacy of anti‐retroviral and interferon therapy. Am J Hematol 200476187–189. [PubMed]

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