T-cell leukemia/lymphoma, adult (htlv-i+)C91.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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DefinitionThis section has been translated automatically.

Adult leukemic T-cell lymphoma caused by infection with the human pathogenic retrovirus HTLV-I+ (but also occurring in HTLV-I negative AIDS patients).

Occurrence/EpidemiologyThis section has been translated automatically.

Endemic areas for this virus are southern Japan, the Caribbean, southern USA, South America and Equatorial Africa.

EtiopathogenesisThis section has been translated automatically.

HTLV 1 is transmitted through sexual intercourse, breastfeeding or blood contact.

ManifestationThis section has been translated automatically.

Start of the disease between the ages of 24 and 85.

Clinical featuresThis section has been translated automatically.

Often begins with skin changes; monomorphic papular exanthema, nodules and tumours, sometimes also picture of mycosis fungoides or Sézary syndrome.

HistologyThis section has been translated automatically.

Nodular, medium-sized, large-cell infiltrates of varying density, penetrating the entire corium and the adjacent subcutis.

DiagnosisThis section has been translated automatically.

The diagnosis is made clinically, histologically and serologically (HTLV-I positive serology).

TherapyThis section has been translated automatically.

S.u. cutaneous T-cell lymphoma, see also Therapy of pleomorphic T-cell lymphomas.

Progression/forecastThis section has been translated automatically.

Good prognosis if skin changes are the main focus, the mean survival time is then > 20 years. With the acute (leukemic) form there is a poor prognosis. The mean survival time after diagnosis is < 1 year.

LiteratureThis section has been translated automatically.

  1. Bangham CR (2003) Human T-lymphotropic virus type 1 (HTLV-1): persistence and immune control. Int J Hematol 78: 297-303
  2. Franchini G et al (2003) T-cell control by human T-cell leukemia/lymphoma virus type 1 Int J Hematol 78: 280-296
  3. Hanchard B et al (1991) Childhood infective dermatitis evolving into adult T-cell leukaemia after 17 years. Lancet 338: 1593-1594
  4. Kottilil S et al (2000) Hypercalcemia and T-cell-lymphoma with acquired immunodeficiency syndrome: occurrence without human T-cell leukaemia virus-I South Med J 93: 894-897
  5. Yamaguchi K (1994) Human T-lymphotropic virus type in Japan. Lancet 343: 213-216

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer