Analysis Tools
neoplasm
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• 36% of heterozygotes show tumors at 4-5 months of age and by 8-15 months of age about 50% of heterozygotes develop T-cell lymphoma
• prevalence of tumors is 1.6 times higher in females (65%) than in males (41%), regardless of age
• affected lymph nodes exhibit effacement of the nodal architecture, prominent vasularization, atypical T cells, and large B cells, features of angioimmunoblastic T-cell lymphoma, however they do not show expanded FDC networks
• clonal rearrangements in the TCR-beta genes are present in tumors
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hematopoietic system
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• 36% of heterozygotes show tumors at 4-5 months of age and by 8-15 months of age about 50% of heterozygotes develop T-cell lymphoma
• prevalence of tumors is 1.6 times higher in females (65%) than in males (41%), regardless of age
• affected lymph nodes exhibit effacement of the nodal architecture, prominent vasularization, atypical T cells, and large B cells, features of angioimmunoblastic T-cell lymphoma, however they do not show expanded FDC networks
• clonal rearrangements in the TCR-beta genes are present in tumors
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• slight but significant increase in spleen weight; no difference in spleen size is seen between heterozygotes with or without tumors, indicating that splenomegaly does not correlate with lymphadenopathy
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• non-tumor lymph nodes exhibit an increased frequency of follicular helper T cells compared to wild-type mice
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• heterozygotes, both with and without tumors, develop hypergammaglobulinemia, showing a 2- to 3-fold increase in serum IgG levels
• heterozygotes with tumors have a 1.5-fold increase in total serum IgG levels compared to heterozygotes without tumors
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• follicular T cells exhibit increased clonality compared with non-follicular T cells from the same lymph node, even in nontumor lymph nodes
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immune system
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• 36% of heterozygotes show tumors at 4-5 months of age and by 8-15 months of age about 50% of heterozygotes develop T-cell lymphoma
• prevalence of tumors is 1.6 times higher in females (65%) than in males (41%), regardless of age
• affected lymph nodes exhibit effacement of the nodal architecture, prominent vasularization, atypical T cells, and large B cells, features of angioimmunoblastic T-cell lymphoma, however they do not show expanded FDC networks
• clonal rearrangements in the TCR-beta genes are present in tumors
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• slight but significant increase in spleen weight; no difference in spleen size is seen between heterozygotes with or without tumors, indicating that splenomegaly does not correlate with lymphadenopathy
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• non-tumor lymph nodes exhibit an increased frequency of follicular helper T cells compared to wild-type mice
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• heterozygotes, both with and without tumors, develop hypergammaglobulinemia, showing a 2- to 3-fold increase in serum IgG levels
• heterozygotes with tumors have a 1.5-fold increase in total serum IgG levels compared to heterozygotes without tumors
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• follicular T cells exhibit increased clonality compared with non-follicular T cells from the same lymph node, even in nontumor lymph nodes
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• tumor lymph nodes exhibit an increase in the number of macrophages within the paracortex with occasional foci of myeloid metaplasia, dense aggregates of small PAX5+ lymphocytes all over the parenchyma forming follicles without germinal centers, reactive B-blasts and small clusters of mature plasma cells in the interfollicular areas, polymorphic infiltrate composed of small- to medium-sized proliferating CD3+ lymphocytes in the interfollicular compartment, T cells that occasionally form rosettes around large blasts, sinusoidal dilatation, vessels filled with T cells, an increase in the proportion of B cells, an increase in the frequency of myeloid and dendritic cells with monocyte-derived dendritic cells showing a 2- to 3-fold expansion, a decrease in CD8+ dendritic cells, and an increase in proliferation of follicular helper T cells
• nonenlarged lymph nodes from tumor-bearing heterozygotes exhibit preserved nodal architecture, having both primary and secondary follicles with reactive germinal centers as well as an increase of mature plasma cells in the interfollicular area
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• germinal centers are hyperplastic in nontumor lymph nodes of heterozygotes with tumors
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• 53% of heterozygotes develop 1 to 4 enlarged lymph nodes, whereas other lymph nodes remain normal
• while some lymph nodes are larger due to tumor development, nontumor lymph nodes in heterozygotes are also often larger than control lymph nodes
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growth/size/body
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• slight but significant increase in spleen weight; no difference in spleen size is seen between heterozygotes with or without tumors, indicating that splenomegaly does not correlate with lymphadenopathy
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endocrine/exocrine glands
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• 36% of heterozygotes show tumors at 4-5 months of age and by 8-15 months of age about 50% of heterozygotes develop T-cell lymphoma
• prevalence of tumors is 1.6 times higher in females (65%) than in males (41%), regardless of age
• affected lymph nodes exhibit effacement of the nodal architecture, prominent vasularization, atypical T cells, and large B cells, features of angioimmunoblastic T-cell lymphoma, however they do not show expanded FDC networks
• clonal rearrangements in the TCR-beta genes are present in tumors
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| peripheral T-cell lymphoma | DOID:0050749 | J:189087 | ||
