Human T-cell leukemia virus type 1 (HTLV-1) causes adult T-cell leukemia (ATL) after a long latency period of about 60 years. As the mature T-cell neoplasms that emerge in patients infected with HTLV-1 are often ATL, T-cell neoplasms developing in such patients tend to be diagnosed simply as ATL without further investigation. However, not all T-cell neoplasms that develop in HTLV-1-infected cases are ATL. Mature T-cell malignancies other than ATL should be carefully excluded in patients infected with HTLV-1, as these sometimes closely resemble ATL in their clinical, morphological, and histological features. Here, we present a case of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in an HTLV-1 carrier. Confirmation of monoclonal integration of the virus with Southern blotting leads to a definite diagnosis of ATL. Although we did not detect the monoclonal integration band of HTLV-1 in this case, the high HTLV-1 proviral load complicated the diagnosis. Multicolor flow cytometric analysis clearly showed that HTLV-1 was not integrated in the tumor cells, and facilitated discrimination of PTCL-NOS from ATL.
Human T-cell leukemia virus type 1 (HTLV-1) Adult T-cell leukemia (ATL) Multicolor flow cytometry Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS)
This is a preview of subscription content, log in to check access.
Conflict of interest
The authors declare no conflicts of interest.
Matsumoto M, Nomura K. Clinical and hematological features of adult T cell leukemia in southern part of Kyushu, Japan (author’s transl). Rinsho Ketsueki. 1979;20:1040–7.PubMedGoogle Scholar
Yoshida M, Miyoshi I, Hinuma Y. Isolation and characterization of retrovirus from cell lines of human adult T-cell leukemia and its implication in the disease. Proc Natl Acad Sci USA. 1982;79:2031–5.PubMedCrossRefGoogle Scholar
Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984–87). Br J Haematol. 1991;79:428–37.PubMedCrossRefGoogle Scholar
Ishitsuka K, Tamura K. Treatment of adult T-cell leukemia/lymphoma: past, present, and future. Eur J Haematol. 2008;80:185–96.PubMedCrossRefGoogle Scholar
Nakano-Akamatsu S, Takahashi R, Sekioka Y, et al. CD20- and CD56-positive T-cell large granular lymphocyte leukemia in a human T-cell leukemia virus type 1 carrier. Int J Hematol. 2007;86:348–51.PubMedCrossRefGoogle Scholar
Ohmatsu H, Sugaya M, Fujita H, et al. Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma in a human T-cell leukaemia virus type-1 carrier. Acta Derm Venereol. 2010;90:324–5.PubMedGoogle Scholar
Chuang SS, Ichinohasama R, Chu JS, et al. Differential diagnosis of angioimmunoblastic T-cell lymphoma with seropositivity for anti-HTLV antibody from adult T-cell leukemia/lymphoma. Int J Hematol. 2010;91:687–91.PubMedCrossRefGoogle Scholar
Iwanaga M, Watanabe T, Utsunomiya A, et al. Human T-cell leukemia virus type I (HTLV-1) proviral load and disease progression in asymptomatic HTLV-1 carriers: a nationwide prospective study in Japan. Blood. 2010;116:1211–9.PubMedCrossRefGoogle Scholar
Ohshima K. Pathological features of diseases associated with human T-cell leukemia virus type I. Cancer Sci. 2007;98:772–8.PubMedCrossRefGoogle Scholar
Kamihira S, Atogami S, Sohda H, et al. Significance of soluble interleukin-2 receptor levels for evaluation of the progression of adult T-cell leukemia. Cancer. 1994;73:2753–8.PubMedCrossRefGoogle Scholar
Tian Y, Kobayashi S, Ohno N, et al. Leukemic T cells are specifically enriched in a unique CD3(dim) CD7(low) subpopulation of CD4(+) T cells in acute-type adult T-cell leukemia. Cancer Sci. 2011;102:569–77.PubMedCrossRefGoogle Scholar
Kobayashi S, Tian Y, Ohno N, et al. The CD3 versus CD7 plot in multicolor flow cytometry reflects progression of disease stage in patients infected with HTLV-I. PLoS ONE. 2013;8:e53728.PubMedCrossRefGoogle Scholar
Kozuru M, Uike N, Muta K, et al. High occurrence of primary malignant neoplasms in patients with adult T-cell leukemia/lymphoma, their siblings, and their mothers. Cancer. 1996;78:1119–24.PubMedCrossRefGoogle Scholar
Suefuji H, Ohshima K, Hayabuchi N, et al. HTLV-1 carriers with B-cell lymphoma of localized stage head and neck: prognosis, clinical and immunopathological features. Br J Haematol. 2003;123:606–12.PubMedCrossRefGoogle Scholar
Sugata K, Satou Y, Yasunaga JI, et al. HTLV-1 bZIP factor impairs cell-mediated immunity by suppressing production of Th1 cytokines. Blood. 2012;119(2):434–44.Google Scholar
Imaizumi Y, Tsukasaki K, Tsushima H, et al. Lymphoma cases without detectable monoclonal HTLV-1 integration in HTLV-1 carrier. Rinsho Ketsueki. 2012;53:1359.Google Scholar
Lotan I, Khlebtovsky A, Inbar E, et al. Primary brain T-cell lymphoma in an HTLV-1 serologically positive male. J Neurol Sci. 2012;314:163–5.PubMedCrossRefGoogle Scholar