Journal of Hematopathology

, Volume 6, Issue 3, pp 135–144 | Cite as

Adult T cell leukaemia/lymphoma in Kerala, South India: are we staring at the tip of the iceberg?

  • Rekha A. Nair
  • Priya Mary Jacob
  • Sreejith G. Nair
  • Shruti Prem
  • A. V. Jayasudha
  • Nair P. Sindhu
  • K. R. Anila
Original Article


This study comes from Regional Cancer Centre, Trivandrum, in the state of Kerala, South India. Regional Cancer Centre is a comprehensive cancer centre catering to the population of the State of Kerala and the adjoining states of Tamil Nadu and Karnataka. This study is an analysis of leukaemic phase, nodal and extranodal presentations of adult T cell leukaemia/lymphoma (ATLL) in our institute. This study aims to document the alarming incidence of ATLL in this part of South India. This study includes 32 cases, which were diagnosed as compatible with ATLL over a period of 26 months.Our diagnostic approach to a typical case starts with a detailed history, clinical examination with emphasis on skin lesions,evaluation of laboratory findings especially seurm calcium and lactate dehydrogenase values. A peripheral blood examination would be followed by ancillary tests like flow cytometric immunophenotyping of peripheral blood or histopathological examination with immunostaining of submitted tissue biopsy. The last step is the confirmatory serology test for serum human T cell lymphotropic virus type 1 (HTLV-1) antibody. We report for the first time from the state of Kerala, South India, 15 patients with HTLV-1-positive ATLL. This high incidence of ATLL in our state population hitherto not reported is worrisome and entails routine screening for serum HTLV-1 before invasive procedures and among voluntary blood donors in our population.


Non-endemic Adult T cell leukaemia/lymphoma Kerala, India 



Dr Mammen Chandy, eminent haematologist, presently Director, Tata Medical Center, Kolkata, India, for a thoughtful comment he made during a conference that most cases of ATLL he had seen were from the state of Kerala. Department of Radiotherapy, Medical college, Trivandrum for clinical photographs Fig. 3a–c. A part of this study was presented as a poster titled “Adult T-cell leukaemia / lymphoma (ATLL)—hot spot in South India” at the XVI Meeting of the European Association for Haematopathology October 20th–25th, 2012 in Lisbon, Portugal.

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Blattner WA, Blayney DW, Robert-Guroff M, Sarngadharan MG, Kalyanaraman VS, Sarin PS et al (1983) Epidemiology of human T-cell leukemia/lymphoma virus. J Infect Dis 147:406–416PubMedCrossRefGoogle Scholar
  2. 2.
    Proietti FA, Carneiro-Proietti AB, Catalan-Soares BC, Murphy EL (2005) Global epidemiology of HTLV-I infection and associated diseases. Oncogene 24:6058–6068PubMedCrossRefGoogle Scholar
  3. 3.
    Verdonck K, Gonzalez E, Van Dooren S, Vandamme AM, Vanham G, Gotuzzo E (2007) Human T-lymphotropic virus 1: recent knowledge about an ancient infection. Lancet Infect Dis 7:266–281PubMedCrossRefGoogle Scholar
  4. 4.
    Oshima K, Jaffe ES, Kikuchi M (2008) Adult T cell leukaemia/lymphoma. WHO classification of haematopoietic and lymphoid tissue. WHO, Lyon, pp 281–284Google Scholar
  5. 5.
    Roy M, Das MK, Ishida T, Das SK, Dey B, Banerjee S et al (1994) Absence of HTLV-I infection in some Indian populations. Indian J Med Res 100:160–162PubMedGoogle Scholar
  6. 6.
    Chaudhari CN, Shah T, Misra RN (2009) Prevalence of human T cell leukaemia virus amongst blood donors. Armed Forces Med J India 65:38–40CrossRefGoogle Scholar
  7. 7.
    Babu PG, Ishida T, Nesadoss J, John TJ (1995) Prevalence of HTLV-I/II antibodies in HIV seropositive and HIV seronegative STD patients in Vellore region in southern India. Scand J Infect Dis 27:105–108PubMedCrossRefGoogle Scholar
  8. 8.
    Ahmed F, Murthy SS, Mohan MK, Rajappa SJ (2012) HTLV1 associated adult T cell lymphoma/leukaemia a clinicopathologic, immunophenotypic tale of three cases from non endemic region of South India. Indian J Pathol Microbiol 55:92–96PubMedCrossRefGoogle Scholar
  9. 9.
    Gujral S, Polampalli S, Badrinath Y, Kumar A, Subramanian PG, Nair R, Sengar M, Nair C (2010) Immunophenotyping of mature T/NK cell neoplasm presenting as leukemia. Indian J Cancer 47(2):189–193PubMedCrossRefGoogle Scholar
  10. 10.
    Jain P, Gupta S, Prabhash K, Patkar N, Parikh PM (2008) Adult T cell leukemia. A typical case from India. Indian J Cancer 45:72–73PubMedGoogle Scholar
  11. 11.
    Udupa K, Prasanth G, Tenali GS, Sanju C, Urmila M (2011) Adult T-cell leukemia in India: report of two cases and review of literature. J Cancer Res Ther 7(3):338–340CrossRefGoogle Scholar
  12. 12.
    Uchiyama T, Yodoi J, Sagawa K (1977) Adult T-cell leukemia: clinical and hematologic features of 16 cases. Blood 50(3):481–492PubMedGoogle Scholar
  13. 13.
    Poiesz BJ, Ruscetti FW, Gazdar AF, Bunn PA, Minna JD, Gallo RC (1980) Detection and isolation of type C retrovirus particles from fresh and cultured lymphocytes of a patient with cutaneous T-cell lymphoma. Proc Natl Acad Sci USA 77:7415–7419PubMedCrossRefGoogle Scholar
  14. 14.
    Hinuma Y, Nagata K, Hanaoka M, Nakai M, Matsumoto T, Kinoshita K-I, Shirakawa S, Miyoshi I (1981) Adult T-cell leukemia: antigen in an ATL cell line and detection of antibodies to the antigen in human sera. Proc Natl Acad Sci USA 78:6476–6480PubMedCrossRefGoogle Scholar
  15. 15.
    Tamiya S, Matsuoka M, Etoh K (1996) Two types of defective human T-lymphotropic virus type I provirus in adult T-cell leukemia. Blood 88:3065–3073PubMedGoogle Scholar
  16. 16.
    Masao M, Green PL (2009) The HBZ gene, a key player in HTLV-1 pathogenesis. Retrovirology 6:71. doi: 10.1186/1742-4690-6-71 CrossRefGoogle Scholar
  17. 17.
    Hidaka T, Nakahata S, Hatakeyama K, Hamasaki M, Yamashita K, Kohno T, Arai Y (2008) Down-regulation of TCF8 is involved in the leukemogenesis of adult T-cell leukemia/lymphoma. Blood 112(2):383–393. doi: 10.1182/blood-2008-01-131185 PubMedCrossRefGoogle Scholar
  18. 18.
    Furukawa Y, Fujisawa J, Osame M et al (1992) Frequent clonal proliferation of human T-cell leukemia virus type 1 (HTLV- 1) -infected T cells in HTLV-1-associated myelopathy (HAMTSP). Blood 80(4):1012–1016PubMedGoogle Scholar
  19. 19.
    Ikeda S, Momita S, Kinoshita KI et al (1993) Clinical course of human T-lymphotropic virus type I carriers with molecularly detectable monoclonal proliferation of T lymphocytes: defining a low- and high-risk population. Blood 82(7):2017–2024PubMedGoogle Scholar
  20. 20.
    Yokote T, Akioka T, Oka S, Hara S et al (2005) Flow cytometric immunophenotyping of adult T-cell leukaemia/lymphoma using CD3 gating. Am J Clin Pathol 124:199–204PubMedCrossRefGoogle Scholar
  21. 21.
    LaGrenade L, Hanchard B, Fletcher V, Cranston B, Blattner W (1990) Infective dermatitis of Jamaican children: a marker for HTLV-I infection. Lancet 336:1345–1347PubMedCrossRefGoogle Scholar
  22. 22.
    Mochizuki M, Watanabe T, Yamaguchi K, Takatsuki K, Yoshimura K, Shirao M, Nakashima S, Mori S, Araki S, Miyata N (1992) HTLV-I uveitis: a distinct clinical entity caused by HTLV-I. Jpn J Cancer Res 83:236–239PubMedCrossRefGoogle Scholar
  23. 23.
    Nishioka K, I Maruyama, K Sato, I Kitajima, Y Nakajima, M Osame (1989) Chronic inflammatory arthropathy associated with HTLV-I. Lancet i:441Google Scholar
  24. 24.
    Gallo RC (2002) Human retroviruses after 20 years: a perspective from the past and prospects for their future control. Immunol Rev 185:236–265PubMedCrossRefGoogle Scholar
  25. 25.
    Vandamme AM, Bertazzoni U, Salemi M (2000) Evolutionary strategies of human T-cell lymphotropic virus type II. Gene 261:171–180PubMedCrossRefGoogle Scholar
  26. 26.
    Calattini S, Betsem E, Bassot S, Chevalier SA, Mahieux R, Froment A, Gessain A (2009) New strain of human T lymphotropic virus (HTLV) type 3 in a pygmy from Cameroon with peculiar HTLV serologic results. J Infect Dis 199:561–564PubMedCrossRefGoogle Scholar
  27. 27.
    Switzer WM, Salemi M, Qari SH, Jia H, Gray RR, Katzourakis A, Marriott SJ, Pryor KN, Wolfe ND, Burke DS, Folks TM, Heneine W (2009) Ancient, independent evolution and distinct molecular features of the novel human T-lymphotropic virus type 4. Retrovirology 6:9PubMedCrossRefGoogle Scholar
  28. 28.
    Wolfe ND, Heneine W, Carr JK, Garcia AD, Shanmugam V, Tamoufe U, Torimiro JN, Prosser AT, Lebreton M, Mpoudi-Ngole E, McCutchan FE, Birx DL, Folks TM, Burke DS, Switzer WM (2005) Emergence of unique primate T-lymphotropic viruses among central African bushmeat hunters. Proc Natl Acad Sci USA 102:7994–7999PubMedCrossRefGoogle Scholar
  29. 29.
    Vitone F, Gibellini D, Schiavone P, D’Antuono A, Gianni L, Bon I, Re MC (2006) Human T-lymphotropic virus type 1 (HTLV-1) prevalence and quantitative detection of DNA proviral load in individuals with indeterminate/positive serological results. BMC Infect Dis 6:41PubMedCrossRefGoogle Scholar
  30. 30.
    Mahieux R, Gessain A (2009) The human HTLV-3 and HTLV-4 retroviruses: new members of the HTLV family. Pathol Biol (Paris) 57:161–166CrossRefGoogle Scholar
  31. 31.
    Vandamme AM, Van Laethem K, Liu HF, Van Brussel M, Delaporte E, Castro Costa CM, Fleischer C, Taylor G, Bertazzoni U, Desmyter J, Goubau P (1997) Use of a generic polymerase chain reaction assay detecting human T-lymphotropic virus (HTLV) types I, II and divergent simian strains in the evaluation of individuals with indeterminate HTLV serology. J Med Virol 52:1–7PubMedCrossRefGoogle Scholar
  32. 32.
    Vet JA, Majithia AR, Marras SA, Tyagi S, Dube S, Poiesz BJ, Kramer FR (1999) Multiplex detection of four pathogenic retroviruses using molecular beacons. Proc Natl Acad Sci USA 96:6394–6399PubMedCrossRefGoogle Scholar
  33. 33.
    Venkataraman G, Berkowitz J, Morris JC, Janik JE, Raffeld MA, Pittaluga S (2011) Adult T-cell leukemia/lymphoma with EBV-positive Hodgkin-like cells. Hum Pathol 42(7):1042–1046. doi: 10.1016/j.humpath.2010.10.014 PubMedCrossRefGoogle Scholar
  34. 34.
    Ohtsubo H, Arima N, Tei C (1999) Epstein-Barr virus involvement in T-cell malignancy: significance in adult T-cell leukemia. Leuk Lymphoma 33:451–458PubMedGoogle Scholar
  35. 35.
    Ueda S, Maeda Y, Yamaguchi T, Hanamoto H, Hijikata Y, Tanaka M et al (2008) Influence of Epstein–Barr virus infection in adult T-cell leukemia. Hematology 13:154–162PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Rekha A. Nair
    • 1
  • Priya Mary Jacob
    • 1
  • Sreejith G. Nair
    • 2
  • Shruti Prem
    • 2
  • A. V. Jayasudha
    • 1
  • Nair P. Sindhu
    • 1
  • K. R. Anila
    • 1
  1. 1.Department of PathologyRegional Cancer CentreTrivandrumIndia
  2. 2.Department of Medical OncologyRegional Cancer CentreTrivandrumIndia

Personalised recommendations