Linfoma hepatoesplénico de células T gamma y delta (γδ)

  • Es un tipo de linfoma raro, agresivo (con tendencia a la recidiva) que afecta sobre todo a varones jóvenes o adolescentes.
  • Algunos pacientes presentan historia de inmunosupresión o enfermedad de Crohn en tratamiento con el anticuerpo monoclonal infliximab.
  • Presenta infiltración en: hígado, bazo y médula ósea.
  • Inmunofenotipo y genotipo es: CD3+, CD4-, CD8+, TIA1+, TCRdg, isocromosoma 7q. EBV-

Linfoma T del tipo paniculitis subcutánea

  • Es un linfoma infrecuente (≤ 1% de todos los LNH).
  • Los síntomas son variables desde una afectación nula a su asociación con un síndrome hemofagocítico que puede ser letal (fiebre, pancitopenia y hepatoesplenomegalia) de muy mala evolución.
  • Las formas de comportamiento benigno puede tratarse con monoterapia (metotrexate, prednisona, ciclosporina, clorambucil, ciclofosfamida).
  • En caso de recidiva o debut agresivo debe de valorarse la poliquimioterapia seguida de consolidación con TAPH.
  • Su inmunofenotipo y genotipo es TCR r, EBV-, CD8+, TIA1+.

Nasal NK/T-cell lymphoma

  • This is an aggressive type of lymphoma that is generally rare in the United States and Europe but more common in Asia and South America.

  • It gets its name from the fact that it infiltrates the nasopharynx.

  • The disease often manifests as localized disease with no adenopathy, though there have been cases of systemic disease and involvement of the orbit, mouth, skin, and testicles.

  • The disease can affect people of all ages, though the average age is around 50; men are more likely to develop the disease than women.

  • The immunophenotype and genotype is: TCR r-, EBV+, CD2+, surface CD3-, CD3e+, CD56+, CD4-, CD8-, TIA1+.

  • Prognosis is variable (depending on tumor location and extension).

    Treatmentà CHOP is administered for localized disease (5-year survival rates are around 60-90%). New treatment approached with other drugs such as the SMILE regimen with L-asparaginase have made for improved outcomes. If the disease is localized, consolidation therapy consisting of radiotherapy will be given. Autologous stem-cell transplantation is given in advanced disease.

Enteropathy-associated T-cell lymphoma

  • This very rare disease is mostly seen in areas with high rates of celiac disease.
  • Clinical course consists of abdominal pain, lower calorie absorption, and deteriorated state of overall health.
  • In most cases the disease developed in a localized stage, though it can also spread to the liver, lung, spleen, skin, and bone marrow.
  • It is aggressive and shows little response to chemotherapy, and patients are at risk of intestinal perforation.
  • The immunophenotype and genotype is TCR r. EBV-, CD3+,CD4-, CD8-/+, CD103+, CD56-/+.