Sandbox: T cell: Difference between revisions
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Long latency, virus exposure usually occurs very early in life | Long latency, virus exposure usually occurs very early in life | ||
Transmission of HTLV-1 is believed to occur from mother to child; by sexual contact; and through exposure to contaminated blood, either through blood transfusion or sharing of contaminated needles | Transmission of HTLV-1 is believed to occur from mother to child; by sexual contact; and through exposure to contaminated blood, either through blood transfusion or sharing of contaminated needles | ||
HTLV-I p40 tax viral protein: non structural protein that causes transcriptional activation of many genes in infected lymphocytes | |||
Enhancement of c-AMP response element binding transcription factor (CREB) phosphorylation | |||
HTLV-I basic leucine zipper factor (HBZ): causes T cell proliferation and oncogenesis | |||
JAK/STAT pathway constitutively activated in HTLV-I infected cells | |||
The disease manifests in 75% of cases with leukaemia and in the remaining as a pure lymphomatous form | The disease manifests in 75% of cases with leukaemia and in the remaining as a pure lymphomatous form | ||
widely disseminated disease which may involve liver, skin, blood | widely disseminated disease which may involve liver, skin dermis layer, peripheral blood involvement , bone, and CNS | ||
anaemia and thrombocytopenia is variable | anaemia and thrombocytopenia is variable | ||
patchy infiltrates Bone marrow infiltration | |||
Neutrophilia and eosinophilia Present | Neutrophilia and eosinophilia Present | ||
lytic bone lesions | lytic bone lesions | ||
tumor-induced osteolysis hypercalcaemia | tumor-induced osteolysis hypercalcaemia | ||
increased osteoclastic activity | |||
elevated serum levels of IL-1, TGFβ, PTHrP, macrophage inflammatory protein (MIP-1α), and receptor activator of nuclear factor-κB ligand (RANKL) have been associated with hypercalcemia | elevated serum levels of IL-1, TGFβ, PTHrP, macrophage inflammatory protein (MIP-1α), and receptor activator of nuclear factor-κB ligand (RANKL) have been associated with hypercalcemia | ||
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Infiltration of the dermis skin infiltration, epidermotropism present and Pautrier's microabcesses | Infiltration of the dermis skin infiltration, epidermotropism present and Pautrier's microabcesses | ||
antibodies to HTLV‐I are demonstrable | antibodies to HTLV‐I are demonstrable | ||
defects of cell-mediated immunity recurrent infections | defects of cell-mediated immunity recurrent infections | ||
+3, +7, +21, monosomy X,deletion of chromosome Y and chromosomes 6 and 14q; | +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 | ||
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mutations of tumour‐suppressor genes CDKN2A (p16), CDKN2B (p15) and TP53 (p53) | mutations of tumour‐suppressor genes CDKN2A (p16), CDKN2B (p15) and TP53 (p53) | ||
Gross | |||
Nodules skin | |||
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cytoplasm agranular | cytoplasm agranular | ||
“flower cell” | “flower cell” | ||
Reed-Sternberg like cells may also be present | |||
CD4 positive CD8 positive | CD4 positive CD8 positive | ||
Revision as of 13:06, 3 November 2015
Adult T‐cell leukaemia/lymphoma (ATLL) is a mature T‐cell neoplasm of post‐thymic lymphocytes Etiologically linked to the human T‐cell lymphotropic virus, HTLV‐I, HTLV‐I serology is a mandatory investigation Long latency, virus exposure usually occurs very early in life Transmission of HTLV-1 is believed to occur from mother to child; by sexual contact; and through exposure to contaminated blood, either through blood transfusion or sharing of contaminated needles HTLV-I p40 tax viral protein: non structural protein that causes transcriptional activation of many genes in infected lymphocytes Enhancement of c-AMP response element binding transcription factor (CREB) phosphorylation HTLV-I basic leucine zipper factor (HBZ): causes T cell proliferation and oncogenesis JAK/STAT pathway constitutively activated in HTLV-I infected cells
The disease manifests in 75% of cases with leukaemia and in the remaining as a pure lymphomatous form widely disseminated disease which may involve liver, skin dermis layer, peripheral blood involvement , bone, and CNS anaemia and thrombocytopenia is variable patchy infiltrates Bone marrow infiltration Neutrophilia and eosinophilia Present
lytic bone lesions
tumor-induced osteolysis hypercalcaemia
increased osteoclastic activity
elevated serum levels of IL-1, TGFβ, PTHrP, macrophage inflammatory protein (MIP-1α), and receptor activator of nuclear factor-κB ligand (RANKL) have been associated with hypercalcemia
Diffuse infiltration of the lymph node leading expansion of the paracortical area Infiltration of the dermis skin infiltration, epidermotropism present and Pautrier's microabcesses antibodies to HTLV‐I are demonstrable defects of cell-mediated immunity recurrent infections
+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
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