Adult t-cell lymphoma/leukemia (htlv+) C91.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 28.03.2022

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Adult T-cell leukaemia-lymphoma; Adult T-cell Leukemia/lymphoma; Adult T-cell leukemia/lymphoma (HTLV+); Adult T-cell leukemia/lymphoma (HTLV positiv); Adult T-cell lymphoma/leukemia; Adult T-cell lymphoma/leukemia (HTLV+); ATLL; Human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma

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With a lifetime risk of 1-3%, virus carriers (HTLV-1 infection) develop an acute lymphoproliferative disease after a latency period of several decades: adult T-cell lymphoma/leukemia (ATLL). The initial symptoms are often abdominal pain, prolonged diarrhoea and coughing.

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ATLL can be classified into several types:

  • Smoldering ATLL: (5% lymphoma cells in peripheral blood;total leukocyte count not increased). Only skin and lungs are affected
  • Chronic ATLL (mild lymphocytosis): Affects skin, lungs, furthermore liver, spleen and lymph nodes.
  • Lymphoma type of ATLL (no lymphocytosis): marked lymphadenopathy with extranodal lesions.
  • Acute type of ATLL(Most common variant): most aggressive form of ATLL with the full picture of all symptoms. Prognosis is poor.

In chronic and smouldering ATLL, skin lesions resembling T cell lymphoma of the mycosis furunculoid type are frequently observed, with patches (extensive erythema) papules and plaques.

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Occurrence: ATLL is endemic in countries with high prevalence of HTLV-1, such as the southwest of Japan, the Caribbean islands and countries in central Africa.

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Age: 20-40 years, m&g;

Clinical features
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Symptoms include ascites, swelling of lymph nodes, mycosis fungoides-like cutaneous lesions, enlarged liver and spleen, and hypercalcemia.

Many of the symptoms are caused by infiltration of the open organs. In advanced disease, patients often become immunodeficient and develop interstitial pneumonia, from which they often die again.

The detection of HTLV-1 is essential for the diagnosis of ATLL. Western blotting and PCR allow the differential diagnosis between HTLV -1 and HTLV-2.

The leukemia cells show the phenotype of helper T cells. They are predominantly CD 4-positive, rarely also CD 8-positive and express a large amount of the alpha chain of the interleukin-2 receptor. The cell morphology is altered; characteristic are enlarged cells with lobed nuclei (flower-shaped)

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Different levels of expression of T-cell leukemia. Hypercalcemia;

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Superficial or even deep nodular or diffuse infiltration of the dermis with small, medium or large pleomorphic T cells, usually with marked epidermotropism. The histologic picture is indistinguishable from mycosis fungoides with certainty. The cells expressed CD3, CD4, CD25; they are CD8 negative.

General therapy
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The treatments are performed by internal oncologists.

Chemotherapeutic combination therapy which is oriented therapy of non-Hogkin's lymphoma. This increases the average life expectancy to 7-8 months. Furthermore: combination of interferon and zidovudine (ACT).

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  1. Ishitsuka K et al (2014) Human T-cell leukaemia virus type I and adult T-cell leukaemia-lymphoma. Lancet Oncol 15:e517-526
  2. Marchetti MA et al (2015) Cutaneous manifestations of human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma: a single-center, retrospective study. J Am Acad Dermatol 72:293-301
  3. Torres-Cabala CA et al (2015) HTLV-1-associated infective dermatitis demonstrates low frequency of FOXP3-positive T-regulatory lymphocytes. J Dermatol Sci 77:150-155
  4. Tsukasaki K et al (2014) Human T-cell lymphotropic virus type I-associated adult T-cell leukemia-lymphoma: new directions in clinical research. Clin Cancer Res 20: 5217-5225
  5. Yoshida N et al (2015) Incidence of adult T-cell leukemia/lymphoma in nonendemic areas. Curr Treat Options Oncol 16:321


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Last updated on: 28.03.2022