Adult T-cell leukemia lymphoma

Last Updated: 2021-06-28

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

ICD11: 2A90.5

  • Frequency peaks at the age of 60 to 70 years.
  • Mostly found in Japan and the Caribbean.

  • Trigger: human T-cell leukemia virus-1 (HTLV-1), but only a few HTLV-1 positive patients develop adult T-cell leukemia/lymphoma.
  • Transmission paths:
  • Blood contact
  • IV drug abuse
  • Transfusions
  • Intercourse
  • Mother's milk

  • Erythematous, non-scaling papules, plaques and nodes, in some cases purpura is also described.
  • Acute to chronic
  • Leucocytosis
  • HOTS (hypercalcemia, osteolysis, T-cell neoplasia and skin)
  • Lymphadenopathy
  • Hepatosplenomegaly
  • Spastic paresis

  • Clinic
  • Lab
    • ESR/CRP, Diff.-BB (leukocytosis, rel. lymphocytosis), liver and kidney values, LDH, electrolytes.
    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7 cells
    • Possibly immune electrophoresis.
    • HTLV serology, if applicable
    • If necessary, Lyme Serology
  • Biopsy
    • Dermatopathology
    • Immunohistology: CD4-, CD8+, CD45RA+, TIA-1+
    • Molecular biological: Clonal rearrangment of T-cell receptor genes.
  • Whole-body CT, lymph node sonography, PET-CT if necessary 

     

Unfavorable.

  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.