Adult T-cell leukaemia/lymphoma

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2A90.5

  • 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.