Term
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Definition
| Leukemias are primarily bone marrow/peripheral blood involvement; Lymphomas are primarily tumor masses in lymph nodes/other organs |
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Term
| What are the two broad categories of Lymphomas? |
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Definition
| Hodgkin Lymphoma (HL) and Non-Hodgkin Lymphoma (NHL) |
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Term
| Features of Hodgkin Lymphomas in terms of Age, Location, Spread, and Tissues affected |
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Definition
| Young patients (BIMODAL old too), Localized, Waldeyer's ring rarely involved, Contiguous spread |
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Term
| Features of Non-Hodgkin Lymphomas Features of Hodgkin Lymphomas in terms of Age, Location, Spread, and Tissues affected |
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Definition
| Older patients, Disseminated disease with Non-contiguous spread and commonly involves Waldeyer's ring |
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Term
| Most Lymphomas are of what cell type? |
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Definition
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Term
| What infectious agents are implicated in the cause of Lymphomas? |
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Definition
| EBV, HHV8, HCV, H. pylori |
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Term
| What two mutations can occur to cause B and T cell NHL? |
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Definition
| myc gene for uncontrolled proliferation and Bcl-2 gene for lack of apoptosis |
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
| B cell Germinal center marker |
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Definition
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Term
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Definition
| Mature cells that have left the BM |
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Term
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Definition
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Term
| What is the main predisposing factor for acquiring a Lymphoma? |
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Definition
| Alterations (impaired) immune status |
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Term
| How much pain is associated with Lymph node enlargement in lymphomas? |
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Definition
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Term
| What are the clinical symptoms of Lymphomas? |
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Definition
| Fever, night sweats, Weight loss (aka "B" symptoms) as well as generalized lymphadenopathy |
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Term
| What is the main method for diagnosing Lymphomas? |
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Definition
| Excisional Biopsy with Fresh tissue |
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Term
| What are the goals of Ancillary studies on biopsy tissue? |
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Definition
| To confirm lymphoma and to determine which subtype of B cell lymphoma it is |
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Term
| Why is it important to know the subtype of B cell lymphoma present? |
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Definition
| The prognosis for NHL/Leukemia is dependent on the specific type |
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Term
| How is lineage of the lymphoma determined? |
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Definition
| Immunohistochemistry/Flow cytometry of the cell surface markers |
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Term
| What markers are associated with Follicular Lymphoma? |
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Definition
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Term
| What is Flow cytometry most useful for? |
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Definition
| Identifying specific markers via antibodies with fluorochromes to establish B vs T lymphoma and Clonality (Kappa vs Lambda) |
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Term
| Burkitt lymphoma translocation |
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Definition
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Term
| Follicular lymphoma translocation |
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Definition
| t(14;18) leading to overexpression of bcl-2 and thus inhibition of apoptosis |
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Term
| Gene rearragment studies on a B cell lymphoma should reveal what about immunoglobulin production? |
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Definition
| Large amount of one type of Ig due to monoclonality of a specific heavy chain gene rearrangement |
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Term
| How are NHL's broadly classified? |
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Definition
| Low-grade lymphomas vs High-grade lymphomas |
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Term
| Characteristics of Low grade NHL and give example |
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Definition
| Indolent growth, difficult to cure but not often fatal; Follicular lymphoma |
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Term
| Characteristics of High grade NHL and give example |
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Definition
| Aggressive growth, more likely to be cured from chemotherapy; Burkitt Lymphoma |
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Term
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Definition
| Involved in Single Lymph node region |
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Term
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Definition
| Two or more lymph node regions on the Same Side of the diaphragm |
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Term
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Definition
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Term
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Definition
| Involvement in extralymphatic organs |
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Term
| What are the 4 Low-Grade B-cell lymphomas? |
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Definition
| 1) Chronic Lymphocytic Leukemia/Small Lymphocytic lymphoma, 2) Follicular lymphoma, 3) Extranodal Marginal Zone Lymphoma (MALT), 4) Mantle Cell Lymphoma |
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Term
| What are the 2 main High-Grade B-cell lymphomas? |
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Definition
| 1) Burkitt Lymphoma, 2) Diffuse large cell lymphoma |
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Term
| What are the 3 main T-cell Lymphomas? |
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Definition
| 1) Peripheral T-cell Lymphoma, 2) Mycosis Fungoides/Sezary Syndrome, 3) Anaplastic Large Cell Lymphoma |
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Term
| Chronic Lymphocytic Lymphoma predominantly involves what tissue? |
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Definition
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Term
| Small Lymphocytic Lymphoma predominantly involves what tissue? |
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Definition
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Term
| What are the clinical symptoms of Chronic Lymphocytic Lymphoma/Small Lymphocytic Lymphoma? |
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Definition
| Asymptomatic (mostly an Indolent disorder), Hypogammaglobulinemia, AIHA, Splenomegaly |
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Term
| Peripheral smear of Chronic Lymphocytic Lymphoma/Small Lymphocytic Lymphoma shows what cells? |
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Definition
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Term
| Lymph nodes appear how in Chronic Lymphocytic Lymphoma/Small Lymphocytic Lymphoma? |
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Definition
| They show Diffuse Effacement and Proliferation centers |
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Term
| What is the immunophenotype of CLL/SLL? |
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Definition
| CD5 (normally a T cell marker) |
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Term
| What is Richter's syndrome and what is it's incidence? |
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Definition
| Transformation of CLL/SLL into Large B-cell Lymphoma that is very aggressive; happens 3.5% of the time |
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Term
| What age group typically acquires CLL/SLL? |
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Definition
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Term
| Describe the Morphology of Follicular Lymphomas |
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Definition
| Effacement of Nodal architecture with Nodular Growth patterns (reflects its derivation from germinal cell centers) |
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Term
| What featurs of normal germinal centers does Follicular Lymphoma lack? |
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Definition
| "Tingible body" macrophages and no Preserved Mantle zone (Dark ring around the germinal center) |
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Term
| What marker is characteristic of Follicular Lymphomas? |
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Definition
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Term
| What genetic mutation is present in most Follicular Lymphomas and what genes does it occur between? |
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Definition
| t(14;18) between IgH and bcl2 genes |
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Term
| What location does Most Extranodal Marginal Zone Lymphoma occur? |
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Definition
| MALT (stomach and GI tract) |
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Term
| What pathogen is the cause of most Extranodal Marginal Zone Lymphoma of the stomach and how is it treated? |
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Definition
| H. pylori; it is the only example where treatment of infection with antibiotics can eradicate the lymphoma |
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Term
| What is the key histological feature of Extranodal Marginal Zone Lymphoma? |
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Definition
| Lymphoepithelial lesions in the glands that invade epithelial cells |
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Term
| Are there any key surface immunophenotypes for Extranodal Marginal Zone Lymphomas? |
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Definition
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Term
| Most common Lymphoma in adults |
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Definition
| Diffuse Large B-Cell Lymphoma |
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Term
| Second most common Lymphoma in adults? |
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Definition
| Diffuse Large B-cell Lymphoma |
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Term
| How does Diffuse Large B-cell Lymphoma progress clinically (what organ is typically involved)? |
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Definition
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Term
| ~30% of Diffuse Large B-cell Lymphomas have what translocation? |
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Definition
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Term
| Describe the Clinical Course of Diffuse Large B-Cell Lymphoma and how it responds to chemotherapy |
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Definition
| Aggressive clinical course; Responds well to Chemotherapy |
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Term
| What are the two key clinical symptoms of Mantle Cell Lymphoma? |
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Definition
| Fatigue and Diffuse Lymphadenopathy |
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Term
| What tissues/locations are most frequently involved in Mantle Cell Lymphoma? |
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Definition
| GI tract and Waldeyer's ring |
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Term
| What is the morphology of Mantle Cell Lymphoma? |
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Definition
| Diffuse effacement or nodular pattern with small cells and irregular nuclei (a lot like follicular lymphoma) |
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Term
| What is the key Immunophenotype of Mantle Cell Lymphoma? |
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Definition
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Term
| What translocation is associated with Mantle Cell Lymphoma? |
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Definition
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Term
| What is the prognosis and treatment response of Mantle Cell Lymphoma |
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Definition
| Aggressive clinical course that is resistant to Chemotherapy |
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Term
| Where does Burkitt Lymphoma manifest in the US? |
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Definition
| Bowels, Retroperitoneum, and Ovaries |
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Term
| How well does Burkitt Lymphoma respond to chemotherapy? |
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Definition
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Term
| What is the Characteristic Morphology of Burkitt Lymphoma? |
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Definition
| "Starry-Sky" pattern with lots of Tingible Body Macrophages |
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Term
| What is the Immunophenotype of Burkitt Lymphoma? |
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Definition
| CD20+, CD10+, slg+ and EBV antigens in Endemic cases |
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Term
| What translocation is present in Burkitt Lymphoma? |
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Definition
| t(8;14), overexpression of myc gene on chromosome 8 |
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Term
| What is the immunophenotype of Peripheral T-cell lymphoma, Not Otherwise Specified (NOS)? |
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Definition
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Term
| Anaplastic Large Cell Lymphoma (ALCL) is usually characterized by the presence of what protein? |
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Definition
| Anaplastic Lymphoma Kinase (ALK) |
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Term
| ALK+ is more common in what age group and ALK- is more common in what age group in Anaplastic Large Cell Lymphoma? |
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Definition
| ALK+ in first 3 decades of life and ALK- in older individuals |
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Term
| Anaplastic Large Cell Lymphoma mostly involves what tissues? |
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Definition
| Lymph nodes and Bone Marrow |
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Term
| Anaplastic Large Cell Lymphoma has neoplastic cells with what cytoplasmic and nuclear features? |
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Definition
| Abundant Pink Cytoplasm with Horseshoe shaped nuclei called Hallmark cells |
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Term
| Tumor cells in Anaplastic Large Cell Lymphoma express what immunophenotypically? |
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Definition
| CD30 as well as one or more T-cell antigen like CD2 or CD4 |
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Term
| Why is expression of the ALK protein important in Anaplastic Large Cell Lymphoma? |
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Definition
| It is a measure of prognosis; ALK+ cases have 80% 5 year survival compared to 40% for ALK- |
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Term
| What is the most common cutaneous lymphoma? |
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Definition
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Term
| Mycosis Fungoides that affects the spleen is known as what? |
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Definition
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Term
| What is the key immunophenotype of Mycosis Fungoides? |
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Definition
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Term
| Describe the morphology of the nucleus in Sezary cells |
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Definition
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Term
| Acute Lymphoblastic Leukemia (ALL) is mostly a disease of what age group and involves what cell phenotype mostly? |
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Definition
| Children; 85% B-cell phenotype |
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Term
| What two tissues are always involved in ALL? |
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Definition
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Term
| What is the morphology of B-cell Acute Lymphoblastic Leukemia (B-ALL)? |
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Definition
| Small blasts with scant cytoplasm |
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Term
| What is the Immunophenotype of B-cell Acute Lymphoblastic Leukemia? |
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Definition
| TdT+, CD34+ (and also typical B-cell markers like CD19,20,10) |
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Term
| How do you differentiate Burkitt Lymphoma (a mature B-cell lymphoma) from B-cell Acute Lymphoblastic Leukemia (B-ALL)? |
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Definition
| Burkitt Lymphoma has TdT- and SIg+; B-ALL is TdT+ and SIg- |
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Term
| How does T-Lymphoblastic Lymphoma/Leukemia typically present on gross exam and what is the immunophenotype? |
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Definition
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