Adult T-cell leukaemia/lymphoma

• Peak in frequency at the age of 60-70 years.
• Common especially in Japan and the Caribbean.

• Causative agent: human T-cell leukaemia virus-1 (HTLV-1), but only a few HTLV-1 positive patients develop adult T-cell leukaemia/lymphoma
• Modes of transmission:
• Blood contact
• i.v. drug abuse
• Transfusion
• Intercourse
• Breast milk

• Erythematous, non-scaling papules, plaques and nodules, in some cases also purpura are described
• Acute to chronic
• Leukocytosis
• HOTS (hypercalcaemia, osteolysis, T-cell neoplasia and skin)
• Lymphadenopathy
• Hepatosplenomegaly
• Spastic paresis

• Clinic
• Laboratory
  • BSG/CRP, diff.-BB (leukocytosis, rel. lymphocytosis), liver and kidney values, LDH, electrolytes.
  • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7- cells
  • Immunelectrophoresis if necessary
  • If applicable, HTLV serology
  • If applicable, Borrelia serology
• Biopsy
  • Dermatopathology
  • Immunohistology: CD4-, CD8+, CD45RA+, TIA-1+
  • Molecular biology: clonal rearrangment of T-cell receptor genes

Unfavourable.

1. Sugimoto KJ, Shimada A, Wakabayashi M, et al. CD56-positive adult T-cell leukemia/lymphoma: a case report and a review of the literature. Med Mol Morphol. 2015;48(1):54-59.
2. Kato K, Akashi K. Recent Advances in Therapeutic Approaches for Adult T-cell Leukemia/Lymphoma. Viruses. 2015;7(12):6604-6612.
3. Qayyum S, Choi JK. Adult T-cell leukemia/lymphoma. Arch Pathol Lab Med. 2014;138(2):282-286.