Term
| Majority of T-Lineage Lymphomas belong to what class? |
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Definition
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Term
| t (2;5) chromosomal translocation is associated with what disease? |
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Definition
| Anaplastic Large Cell Lymphoma |
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Term
| What is the usual of a T-lineage lymphoma compared to a B-lineage lymphoma? |
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Definition
| There is a poorer prognosis, and the cancer is typically more aggressive |
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Term
| What is the most common cutanaous T-cell lymphoma? |
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Definition
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Term
| What is it about the morphology of Mycosis Fungoides that confirms definitively that MF is the right answer? |
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Definition
| Small-to-medium sized T cells with cerebriform nuclei |
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Term
| Name 2 out of 3 skin characteristics found in mycosis fungoides |
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Definition
1. Epidermotropic T-cell infiltrates
2. Pautrier Microabscesses
3. T cells with cerebriform nuclei |
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Term
| Does mycosis fungoides ever present with lymphadenopathy? If so, how would it be caused? |
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Definition
| Lymphadenopathy is possible in MF. Pieces of skin plaques can deposit in the lymph nodes, provoking an immune response and subsequent enlargement of the nodes |
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Term
| What is the cell of origin for mycosis fungoides? |
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Definition
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Term
| What is the diagnostic triad for Sezary syndrome? |
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Definition
1. Generalized erythroderma
2. Lymphadenopathy
3. Circulating tumor cells in blood |
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Term
| What happens to the bone marrow in Sezary syndrome? |
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Definition
| The bone marrow is spared, and the spread of Sezary cells usually occurs into the interstitial space, not the bone marrow |
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Term
| What markers are positive in an immunophenotype for Sezary syndrome? |
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Definition
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Term
| What is the prognosis for Sezary syndrome? |
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Definition
| It is incurable, and the median survival is 5 years. Patients usually succumb to opportunistic infections |
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Term
| In individuals with Adult T-cell Leukemia/Lymphoma, what is the only way they could have gotten it? |
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Definition
| Those individuals were infected by the human T-cell leukemia retrovirus type-1 (HTLV-1) |
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Term
| In which geographic locations is Adult T-cell leukemia/lymphoma endemic to? |
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Definition
| Japan, Central Africa, and the Caribbean nations |
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Term
| What cells are classically observed in Adult T-cell leukemia? |
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Definition
| flower cells- cells with multilobated nuclei |
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Term
| Name the clinical variants of ATL that are commonly found |
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Definition
| Acute, Lymphomatous, Chronic, and Smoldering |
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Term
| What are some common clinical findings in acute ATL? |
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Definition
hypercalcemia with bone lesions
skin rash
hepatosplenomegaly
marked leukemic phase (increase in WBC with eosinophils)
constitutional symptoms |
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Term
| In which variants of ATL is the median survival greater than 2 years? |
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Definition
| Chronic and Smoldering variants |
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Term
| In the Smoldering variant of ATL, what two diseases are commonly present, and what classical characteristic of acute ATL is not present? |
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Definition
| Skin and Lung diseases are common, but hypercalcemia is not featured |
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Term
| In addition the usual T-cell markers present, what additional CD is present in ATL? |
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Definition
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Term
| What cells are classically featured in Anaplastic Large-Cell Lymphoma (ALK positive)? |
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Definition
| hallmark cells with horeshoe-shaped nuclei and voluminous cytoplasm |
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Term
| The rearrangement of the ALK gene in Anaplastic disease has the effect of doing what? |
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Definition
| ALK rearrangement leads to constitutive activation of tyroskine kinases that trigger the JAK/STAT pathway |
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Term
| What age group is commonly affected in Anaplastic disease? |
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Definition
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Term
| In the primary systemic type of Anaplastic Large-Cell lymphoma, what are some common clinical indications? |
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Definition
| There is enlargement of the lymph nodes, but the bone marrow is typically unaffected. There is involvement of extranodal sites, but there is little mediastinal mass formation. |
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Term
| Besides the usual T-cell antigens, what other marker is found in Anaplastic leukemia that is also found in Hodgkin's Lymphoma? |
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Definition
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Term
| What is the prognosis for Anaplastic Leukemia? |
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Definition
| Unlike the other aggressive peripheral T-cell neoplasms, the five-year survival rate is 80%/ soft tissue involvement and children being the ones affected are factors that contribute to the favorable survival odds. |
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