Adult T-cell leukemia/lymphoma (ATL) is a human malignancy associated with infection by the retrovirus human T-cell leukemia virus type I (HTLV-I) (Takatsuki. Jpn Soc Res 25:97–103, 1985; Poiesz et al. Proc Natl Acad Sci USA 77:6815–6819, 1980). Approximately 90% of infected individuals remain asymptomatic carriers throughout their lifetime (Gonçalves et al. Clin Microbiol Rev 23(3):577–589, 2010). ATL involves many organs and systems, including the circulatory system, lymphatic system, skin, liver, bone marrow, and gastrointestinal tract. Furthermore, because ATL leads to immunodeficiency and immunoreactivity, it can indirectly cause many other problems, such as opportunistic lung infections, monoclonal gammopathy, chronic renal failure, and strongyloidiasis (Takatsuki et al. Gann Mono Can Res 32:1–15, 1992). HTLV-I also causes a chronic progressive myelopathy known as HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), which is linked to specific human leukocyte antigen (HLA) haplotypes, and leads to inflammatory disorders of organ systems other than the nervous system, which include uveitis, alveolitis (Osame et al. Lancet i:1013–1014, 1986; Gessain et al. Lancet ii: 407–409, 1985), Sjögren syndrome, polymyositis, and arthritis (Table 4.1) (Takatsuki et al. Gann Mono Can Res 32:1–15, 1992).
Table 4.1

Human T-cell leukemia virus type I (HTLV-1)-related disease

Neoplastic disorders

Reactive disorders

 Peripheral blood (leukemia)


  Smoldering type

  HTLV-I-associated myelopathy (HAM)

  Chronic type

  HTLV-I-associated uveitis

  Acute type

 Not confirmed

 Lymph node (Lymphoma)

  HTLV-I-associated lymphadenitis

  Hodgkin’s-like type

  HTLV-I-associated bronchopneumopathy (HAB)

  Pleomorphic small-cell type

  HTLV-I-associated arthropathy (HAAP)

  Pleomorphic (medium- and large-cell) type

  HTLV-I-associated nephropathy

  Anaplastic large-cell type

  Infective dermatitis




  Sjögren syndrome


  Autoimmune thyroiditis




Immunodeficiency association

 Gastrointestinal tract

 Strongyloidiasis (gastrointestinal tract)


 Varicella zoster (skin)


 Crusted scabies (skin)


 Opportunistic lung infection


  Pneumocystis carinii

  Portal or sinus infiltration


 Bone marrow

  Aspergillus fumigatus

  Infiltration with or without fibrosis

  Candida albicans


  Cryptococcus neoformans

  Interstitial infiltration

 Carcinoma (not confirmed)

Modified from Ref. [64]


Pleomorphic Lymph node Skin Gastrointestinal tract Bone marrow 


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Copyright information

© Springer Japan KK 2017

Authors and Affiliations

  1. 1.Department of Pathology, School of MedicineKurume UniversityKurumeJapan

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