Sandbox: T cell: Difference between revisions
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==Genetic== | ==Genetic== | ||
* | * Development of adult T-cell leukemia is the result of multiple genetic mutations. | ||
* +3, +7, +21, monosomy X,deletion of chromosome Y and chromosomes 6 and 14q; | * Genes involved in the pathogenesis of adult T-cell leukemia include: | ||
:* 14q11 gene mutation | |||
:* TCR‐alpha chain gene mutation | |||
:* TCR‐delta chain gene mutation | |||
* +3, +7, +21, monosomy X, deletion of chromosome Y and chromosomes 6 and 14q; | |||
* 14q11 and break points e TCR‐alpha and ‐delta chain genes TCRA and TCRD | * 14q11 and break points e TCR‐alpha and ‐delta chain genes TCRA and TCRD | ||
* 14q32 of TCL1 | * 14q32 of TCL1 | ||
| Line 65: | Line 71: | ||
* CD3 and T‐cell receptor (TCR)‐β may be down‐regulated | * CD3 and T‐cell receptor (TCR)‐β may be down‐regulated | ||
* CD2, CD3, CD4, CD5, CD25, TCR α/β, CD45ROCD56 expressionCCR4, FOXP3, HLA-DR, L-selectin (CD62), MUM-1 | * CD2, CD3, CD4, CD5, CD25, TCR α/β, CD45ROCD56 expressionCCR4, FOXP3, HLA-DR, L-selectin (CD62), MUM-1 | ||
==References== | |||
{{Reflist}} | |||
Revision as of 13:28, 23 November 2015
Overview
Pathogenesis
- Adult T‐cell leukemia arises from post‐thymic lymphocytes, which are normally involved in the process of cell-mediated immune responses.[1]
- Adult T‐cell leukemia is mainly caused by an infection with human T‐cell lymphotropic virus (HTLV‐I).
- HTLV-1 is usually transmitted via breast feeding early in life.
- Other minor routes of transmission for HTLV-1 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‐I infection, which results in the development of adult T-cell leukemia, is due to:
- HTLV-I basic leucine zipper factor
- HTLV-I p40 tax viral protein
- Activation of JAK/STAT signaling pathway by HTLV-I
- Enhancement of CREB transcription factor by HTLV-I
- 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.
- Haematopathological features of adult T-cell leukemia are variable, patients may present with:
- Anemia
- Thrombocytopenia
- Neutrophilia
- Eosinophilia
- Patchy bone marrow infiltration among adult T-cell leukemia patients may result in:
- Tumor-induced osteolysis due to increased osteoclastic activity
- Multiple lytic bone lesions
- Hypercalcemia
- Hypercalcemia among adult T-cell leukemia patients has been associated with elevated serum concentrations of:
- IL-1
- TGFβ
- PTHrP
- MIP-1α
- RANKL
- 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 organomegally among adult T-cell leukemia patients.
- Cutaneous manifestations of adult T-cell leukemia is due to the infiltration of leukmeic cells along the dermis layer of the skin.
- Cutaneous Pautrier's microabcesses 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.
Genetic
- Development of adult T-cell leukemia is the result of multiple genetic mutations.
- Genes involved in the pathogenesis of adult T-cell leukemia include:
- 14q11 gene mutation
- TCR‐alpha chain gene mutation
- TCR‐delta chain gene mutation
- +3, +7, +21, monosomy X, deletion of chromosome Y and chromosomes 6 and 14q;
- 14q11 and break points e TCR‐alpha and ‐delta chain genes TCRA and TCRD
- 14q32 of TCL1
- mutations of tumour‐suppressor genes CDKN2A (p16), CDKN2B (p15) and TP53 (p53)
Gross
- Nodules skin
Micro
- pleomorphic, a medium size lymphocyte conndensed chromatin
- convoluted or polylobated nucleus
- nucleoli are not visibl
- cytoplasm agranular
- “flower cell”
- Reed-Sternberg like cells may also be present
- CD4 positive CD8 positive
- CD2 and CD5 positive
- CD7 negativ
- CD3 and T‐cell receptor (TCR)‐β may be down‐regulated
- CD2, CD3, CD4, CD5, CD25, TCR α/β, CD45ROCD56 expressionCCR4, FOXP3, HLA-DR, L-selectin (CD62), MUM-1
References
- ↑ 1.0 1.1 Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
- ↑ Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015
- ↑ Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015
- ↑ Lymphoma. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Lymphoma#Adult_T-cell_leukemia.2Flymphoma Accessed on November, 3 2015
- ↑ Adult T-cell Leukemia. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/lymphomanonBatlv.html Accessed on November, 3 2015