Term
| Mantle Cell Lymphoma t (11;14) results in what? |
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Definition
| Over-expression of Cyclin D-CDK 4 |
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Term
| What is the mean age for B lineage lymphomas? |
|
Definition
| 50-60 years with a subset for children |
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Term
| What are the primary risk factors for B lineage lymphomas/leukemias? |
|
Definition
| Primary immunodeficiency syndrome, Autoimmune disorders, and AIDS |
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Term
| What cancer commonly results from infection of the gut by helicobacter pylori? |
|
Definition
| extranodal marginal zone lymphoma (Gastric MALT type) |
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Term
| What are the two most common B-lineage leukemias/lymphomas? |
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Definition
| Diffuse Large B-Cell Lymphoma and Follicular Lymphoma |
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Term
| In Chronic Lymphocytic Leukemia, what happens to the DNA of the B cell? |
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Definition
| The DNA is damaged, so the B cell can't produced antibodies, will continue to proliferate, and grow out of control until it infiltrates the interstitial space |
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Term
| In Small Lymphocytic Lymphoma, what is unique about the lymph nodes? |
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Definition
| In SLL, we have lymphadenoapthy and proliferation centers in the lymph nodes |
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Term
| What are the markers that are positive in the immunophenotype for CLL/SLL? |
|
Definition
|
|
Term
| What markers are not present in CLL/SLL? |
|
Definition
|
|
Term
| Compare the survival lengths between non-somatically mutated CLL/SLL and somatically-mutated CLL/SLL |
|
Definition
Non-somatic mutations: 8 years
Somatic mutations: 20 years |
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|
Term
| What is it called when CLL/SLL transforms into DLBCL? |
|
Definition
| Richter transformation (2-8% chance of it happening) |
|
|
Term
| What is the prognosis for CLL/SLL? |
|
Definition
| It is incurable and has a indolent. Median survival time is 7 years |
|
|
Term
| How frequent are chromosomal translocations in CLL/SLL? |
|
Definition
| Not frequent. Rare, actually |
|
|
Term
| What marker is notably absent in mantle cell lymphoma? |
|
Definition
|
|
Term
| What is the degree of somatic mutation in MCL? |
|
Definition
| There is none. MCL cell of origin is the naive, pre-germinal center B cell. |
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|
Term
| What is the most common clinical presentation in Mantle Cell Lymphoma? |
|
Definition
|
|
Term
| In MCL, are proliferation centers present or absent in the lymph nodes? |
|
Definition
|
|
Term
| What the sites of extranodal involvement in MCL? |
|
Definition
| Lok at the spleen, liver, and gut; occasionally there may be polyps in the colon |
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|
Term
| As opposed to CLL/SLL, what is the prognosis for Mantle Cell Lymphoma? |
|
Definition
| It is aggressive and incurable; the median survival is 3-5 years. Most patients eventually succumb to organ dysfunction caused by tumor infiltration. |
|
|
Term
| What is the gender distribution for follicular lymphoma? |
|
Definition
|
|
Term
| What is the median age in Follicular Lymphoma? |
|
Definition
|
|
Term
| What are the more common markers expressed in FL? |
|
Definition
|
|
Term
| Besides the lymph nodes, what are other sites in the body are affected by follicular lymphoma? |
|
Definition
| Extranodal involvement is relatively uncommon |
|
|
Term
| What percentage of low-grade lymphomas does FL represents? |
|
Definition
|
|
Term
| What is effacement in FL due to? |
|
Definition
| Effacement due to closely-packed, poorly formed neoplastic follicles in germinal centers, often without mantle zones |
|
|
Term
| What are the two principal cell types in FL? |
|
Definition
Small cells with cleaved nuclear contours- centrocytes
Larger cells with open nuclear chromatin, modest cytoplasm- centroblasts |
|
|
Term
| What occurs in the marrow in FL? |
|
Definition
| There is the formation of paratrabecular aggregates |
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|
Term
| What is the prognosis and course of FL? |
|
Definition
| Although incurable, it follows an indolent waxing and waning course- survival (median 7-9 years) is not improved by aggressive therapy |
|
|
Term
| What percentage of follicular lymphomas transforms to DLBCL, and what is the consequence of that transformation |
|
Definition
| 25-30%; increasingly aggressive course, refractory to therapy; impending death |
|
|
Term
| What is the age range for DLBCL? |
|
Definition
| It has a broad age range and evenly affects men and women |
|
|
Term
| Describe the morphology common in DLBCL? |
|
Definition
| Large transformed cells 2x small lymphocyte size; often centroblastic, but can also be immunoblastic or anaplastic |
|
|
Term
| How does DLBCL clinically present? |
|
Definition
| Frequently presents as a rapidly enlarging mass at a nodal or extranodal mass |
|
|
Term
| For DLBCL, what does DNA microarray frequently show? |
|
Definition
Shows two major sub-groups
Germinal center- better prognosis
Advanced blood type- not so good |
|
|
Term
| What are the two most common chromosomal/genetic anomalies that present with DLBCL? |
|
Definition
| t(14;18) like you find in follicular lymphoma and dysregulation of Bcl6 |
|
|
Term
| What therapy is commonly used to treat DLBCL? |
|
Definition
| Rituximab (anti -CD 20; a common marker in DLBCL) |
|
|
Term
| Burkitt's Lymphomas makes up what percentage of childhood lymphomas? |
|
Definition
| 30-50%; disease associated with younger people and children |
|
|
Term
| In sporadic cases of Burkitt's, what percentage is attributed to Epstein-Barr Virus (EBV)? |
|
Definition
|
|
Term
| In endemic cases of EBV, what are the most common extranodal sites that are involved? |
|
Definition
| jaw, breast, omentum, iliocecum, rare in lymph nodes, abdomen |
|
|
Term
| Characterize the morphology of a slide preparation of Burkitt's lymphoma |
|
Definition
| you'll find that the tumor has a high mitotic index and numerous apoptotic cellst that are gobbled up by macrophages, creating a starry sky pattern |
|
|
Term
| What are the common markers of Burkitt's Lymphoma? |
|
Definition
| CD 10, Bcl6, CD 19, 20, 22 |
|
|
Term
| In what B cell lineage lymphoma/leukemia is there a heavy slant toward males (5:1)? |
|
Definition
| Hairy cell leukemia (men are hairy creatures) |
|
|
Term
| What is unique about the pancytopenia found in hairy cell leukemia? |
|
Definition
| Normally, neutropenia is compensated with monocytosis; in HCL, there is also monocytopenia |
|
|
Term
| Is there splenomegaly in HCL? |
|
Definition
| There is very extensive splenomegaly, and red splenic pulp disease |
|
|
Term
| Pancytopenia in HCL, so there's gotta be something wrong with marrow. What does it look like? |
|
Definition
| there is extensive reitculin fibril formation in marrow,with fried egg appearance; often times, the cells cannot be aspirated ("dry tap" condition) |
|
|
Term
| What are the common markers and stains used to indicate positive HCL? |
|
Definition
| Cyclin D1, CD 103, 25, 11, and TRAP stain |
|
|
Term
| What is the recommended treatment for HCL? |
|
Definition
| deoxycoformycin, not classical therapy |
|
|
Term
| In lymphoplasmacytic lymphoma, Waldestrom macroglobulinemia is common. What is it? |
|
Definition
| high levels of IgM in blood leading to hyperviscosity |
|
|
Term
| In blood, what dyscrasia is common in Lymphoplasmacytic lymphoma? |
|
Definition
| rouleaux formation (spleen red pulp disease as well, atrophy of white pulp) |
|
|
Term
| What is the cell of origin for HCL? |
|
Definition
| late-activated memory B cell |
|
|
Term
| Describe the cell of origin and the prognosis for Lymphoplasmacytic lymphoma |
|
Definition
| originates from post-follicular B cell differentiating to plasma cell; incurable progressive disease/ median survival is 4 years (much of IgM is intravascular) |
|
|
Term
| What is the age range commonly affected in multiple myeloma? |
|
Definition
|
|
Term
| Plasma cell myeloma is the most common lymphoid tumor in what ethnic group? |
|
Definition
| African Americans (often have higher physiologic Ig) |
|
|
Term
| What is monoclonal gammopathy? |
|
Definition
| It's a local population of clonal cells with a M spike that doesn't fulfill the criteria yet for multple myeloma due to fact that serum M protein level is less than 3 gm/dl |
|
|
Term
| What two tests must be confirmed to diagnose plasma myeloma? |
|
Definition
|
|
Term
| What two markers are common indicators of multiple myeloma? |
|
Definition
|
|
Term
| Characterize marrow involvement in multiple myeloma |
|
Definition
| Multiple myeloma affects sites of active hematopoiesis like the flat bones, vertebrae, skull, pelvis, and femur |
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