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28 Cards in this Set
- Front
- Back
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Multiple Myeloma is a neoplasm of what cells?
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Plasma cells
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What are the main clinical features of multiple myeloma?
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- Destroys bone at site of active hematopoiesis (vertebrae, ribs, skull)
- malignant cells destroy normal marrow environment - hypercalcalcemia, anemia |
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What is seen histologically in the BM in multiple myeloma?
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- Hypercellular bone marrow consisting of plasma cells
- Plasma cells in clusters and sheets - Destruction/Resorption of bone |
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In MM what 2 special cell types are seen histologically?
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Flame cells and Mott cells
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Most of the clinical findings in multiple myeloma are due to what feature?
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Monoclonoal expansion of plasma cells that secrete a single homogenous IgG product
PARAPROTEIN |
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What is a Bence-Jones protein?
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Light chain IgG found in urine - measure of severity of myeloma
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What is a paraprotein?
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Monoclonal AB produced by a myeloma
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What are the 2 leading causes of death from multiple myeloma?
Caused by what? |
1. Immunodeficiency
2. Renal failure --> anemia Caused by excess production of paraprotein |
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What is seen on the blood smear of multiple myeloma?
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Hyperviscosity causing RBC's to line up like "stacked coins"
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What cytogenetic trait gives a favorable prognosis in CLL but a more aggressive prognosis in multiple myeloma?
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13q Deletion
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What is the second most common lymphoid malignancy in caucasians?
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Multiple Myeloma
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What are the 3 hallmark features of Mutiple Myeloma?
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1. Bone destruction - fractures
2. Marrow Replacement - anemia 3. Paraprotein production |
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Diffuse Large B Cell Lymphomas and Burkitts Lymphomas are what types of lymphomas?
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Very aggressive neoplasms
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DLBCL arises in what 2 settings?
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1. Progression of a more indolent tumor
2. De Novo |
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How does DLBCL usually present?
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Usually with pain so bring patients to doctors early
As rapidly enlarging mass at single nodal/extranodal site (GI most common) |
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Where does DLBCL usually arise?
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Often in extra-nodal sites (40%) such as GI tract
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DLBCL is associated with what virus?
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Epstein-Barr Virus (EBV)
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What does DLBCL look like histologically?
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- Diffuse architectural effacement
- Large Cells - Mitotically active - Invasive - Necrotic |
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What are the 2 genetic categories of DLBCL?
Which one does worse? |
1. GCB (germinal center B cell)
2. ABC (activated B Cell) phenotype ABC does WORSE |
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What disease is associated with EBV and is the most common childhood malignancy in equatorial Africa?
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Burkitt Lymphoma
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What are the 3 variants of Burkitts Lymphoma?
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1. Endemic: seen in Africa
2. Sporadic - throughout the world 3. Immunodeficiency associated - seen with those with HIV |
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What are the sites of involvement for the 3 variants of Burkitts?
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1. Endemic - facial bones
2. Sporadic: abdominal masses 3. Immunodeficient: Lymph Nodes and BM involvement |
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Is Burkitt lymphoma usually indolent or aggressive?
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EXTREMELY Aggressive - medical emergency!
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What is seen histologically in Burkitts lymphoma?
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1. "starry sky" pattern of malignant cells with number scattered histiocytes
2. Tingible Body Macrophages - abundant cytoplasm with dark staining cellular remnants of necrotic tumor cells 3. Deeply basophilic |
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What is the cellular precursor for Burkitts lymphoma?
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Germinal Center B Cell
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What is the main difference between Burkitts lymphoma and a follicular lymphoma in terms of proliferation?
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Burkitts lymphoma is a problem because of its tremendous proliferative drive
Follicular Lymphoma has an inability to undergo apoptosis |
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What is the central event in all Burkitts lymphomas?
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Activation of cMYC
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What cytogenetic event is characteristic of Burkitts lymphoma?
What does it result in? |
T(8:14): IgH - cMYC
- Results in inappropriate OVEREXPRESSION of cMYC |
