Adult T-cell leukemia pathophysiology
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Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response. Development of adult T-cell leukemia is the result of multiple genetic mutations induced by an infection with human T‐cell lymphotropic virus (HTLV). On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic skin findings of adult T-cell leukemia. On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include pleomorphic, medium sized lymphocytes with a polylobulated nucleus and agranular cytoplasm.
The exact mechanism of pathogenesis of the adult T‐cell leukemia is unknown.
- Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune response.
- Adult T‐cell leukemia is mainly caused by an infection with human T‐cell lymphotropic virus (HTLV) .
- HTLV is usually transmitted via breast feeding early in life. Other minor routes of transmission for HTLV may include sexual contact, exposure to contaminated blood, or vertical maternal transmission .
- There appears to be a long latent period between HTLV-1 infection and the development of adult T‐cell leukemia.
- The oncogenesis of HTLV infection, which results in the development of adult T-cell leukemia, is due to:
- Adult T‐cell leukemia can manifests as either a leukemic form (75% of the cases) or a pure lymphomatous form (25% of the cases).
- Adult T‐cell leukemia is a widely disseminated disease which may involve the peripheral blood cells, bone marrow, lymph nodes, liver, spleen, skin, and CNS.
- Hematopathological features of adult T-cell leukemia are variable, which may include:
- Patchy bone marrow infiltration among adult T-cell leukemia patients may result in:
- Hypercalcemia among adult T-cell leukemia patients has been associated with elevated serum concentrations of:
- Infiltration of malignant leukemic cells results in the expansion of the lymph nodes paracortical region, which may lead to the development of peripheral lymphadenopathy among adult T-cell leukemia patients.
- Infiltration of the liver and spleen may lead to the development of organomegaly among adult T-cell leukemia patients.
- Cutaneous manifestations of adult T-cell leukemia is due to the infiltration of leukemic cells along the dermis layer of the skin.
- Cutaneous Pautrier's microabscesses formation (due to epidermotropism) may also be present among adult T-cell leukemia patients. These cutaneous lesions are indistinguishable from the ones found in Sézary syndrome and mycosis fungoides.
- Immune deficiency occurs in adult T-cell leukemia due to a defective cell-mediated immunity.
- Genes involved in the pathogenesis of adult T-cell leukemia include:
- On gross pathology, skin nodules, maculopapular eruption, and erythema are characteristic findings of adult T-cell leukemia.
- On microscopic histopathological analysis, characteristic findings of adult T-cell leukemia include:
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