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

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

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

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

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

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

Therapy
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Progression/forecast
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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.

Literature
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  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

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020