Term
| Leukemia/Lymphoma - which is characterized as a proliferation of neoplastic cells in the bone marrow? |
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Definition
| Leukemia. Lymphoma is in the LNs. |
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Term
| How is leukemia categorized? |
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Definition
| Lymphoid or myeloid cells. |
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Term
| What type of lymphoid cells are neoplastic in leukemia/lymphoma? |
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Definition
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Term
| What type of myeloid cells are neoplastic in leukemia? |
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Definition
| Only leukemia has myeloid cells - Granulocytes, monocytes, megakaryocytes, erythrocytes. |
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Term
| Describe acute leukemia cells. |
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Definition
| More common in younger pts, immature, classically have distinct lymphoid and myeloid cell origins, more progressive/aggressive. |
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Term
| Describe chronic leukemia cells. |
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Definition
| More common in older pts, more mature cells, more indolent, longer disease course. |
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Term
| What is the site of involvement of AML? |
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Definition
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Term
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Definition
| Acute Myeloid Leukemia - monoclonal proliferation of immature usually blastic myeloid cells. |
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Term
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Definition
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Term
| Describe acute leukemia w/ t(15:17)(q22:q12) - what is it associated with? |
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Definition
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Term
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Definition
| Acute Promyelocytic Leukemia. Have bilobed dumbbell shaped nuclei. |
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Term
| T/F APL cells are blasts. |
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Definition
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Term
| What is the treatment for APL? |
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Definition
| All-trans retinoic acid (ATRA) that lets the leukemic cells differentiate beyond the promyelocytic stage. |
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Term
|
Definition
| Disseminated Intravascular Coagulation - DIC. |
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Term
| What does treatment of APL w/ ATRA cause? |
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Definition
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Term
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Definition
| AML, Not Otherwise Specified - so it includes all other myeloid cell types. |
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Term
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Definition
| Myeloproliferative Neoplasms. These are abnormal clonal proliferations where excessive numbers of cells of 1 or more myeloid cell lines. |
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Term
| What is the difference between MPN and AML? |
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Definition
| Complete maturation of the cell lines. |
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Term
| What causes the abnormal proliferation of MPN cells? |
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Definition
| Intra-cellular signalling activated by Tyrosine kinases. |
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Term
| Which tyrosine kinase is the prototype of MPNs? |
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Definition
| BCR-ABL1 - it's activated and always turned on leading to abnormal proliferation. |
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Term
| What chromosome is mutated in MPN? |
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Definition
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Term
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Definition
| Imanitab - specifically targes the BCR-ABL1 gene and its tyrosine kinase product. |
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Term
| Why could someone with CML end up getting an acute leukemia w/ myeloid blasts or lymphoid blasts? |
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Definition
| As CML progresses, there can be an accumulation of add'l cytogenetic abnormalities - cytogenetic evolution. |
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Term
| What is found in cases of Polycythemia Vera (PV) Thrombocythemia (ET), and Primary Myelofibrosis? |
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Definition
| JAK2 Used for diagnosis of MPN. This is a mutation. Without it, diagnosis was much more difficult. |
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Term
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Definition
| Chronic leukemia of RBCs. |
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Term
| T/F Venous and arterial thrombosis can occur w/ PV. |
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Definition
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Term
| T/F Hemorrhage and thrombosis both occur with PV. |
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Definition
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Term
| T/F Most pts w/ ET have JAK2 mutation. |
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Definition
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Term
| What is the replacement of bone marrow by fibrotic tissue? |
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Definition
| Extramedullary hematopoeisis. Necessitates the production of RBCs elsewhere. |
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Term
| Where does extramedullary hematopoesis typically occur? |
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Definition
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Term
| What is effacement of lymph nodes? |
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Definition
| Normal architecture is partially or completely effaced depending on where the lymphoma occurs. REPLACED AND OBLITERATED. Typical in Non-HL, also HL. |
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Term
| What 2 patterns are seen in non-Hodgkin Lymphoma. |
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Definition
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Term
| Which pattern of non-HL has B-cell involvement? |
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Definition
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Term
| What cells are involved w/ diffuse non-HL? |
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Definition
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Term
| Describe follicular non-HL. |
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Definition
| Composed of cells w/ morphologies similar to benign germinal ctr-like structures. They form these center-like structures. |
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Term
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Definition
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Term
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Definition
| Acute Lymphoblastic Leukemia. |
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Term
| What is the predominant cell type in ALL? |
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Definition
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Term
| What are the symptoms of ALL? |
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Definition
| Fever (secondary to neutropenia), Bleeding (secondary to thrombocytopenia), Fatigue (secondary to anemia), Bone pain (secondary to infiltratin of the periosteum or compression fractures). |
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Term
| What are the symptoms of LBL? |
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Definition
| B-LBL - no bone marrow involvement - usually asymptomatic. T-LBL - Thymus involved. Skin, tonsils, LNs, liver, spleen, CNS also involved. |
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Term
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Definition
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Term
| What is the primary site of involvement in ALL? Secondary? |
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Definition
| Bone marrow, peripheral blood. Also CNS, Spleen, liver, testis. |
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Term
| What is the primary site of involvement in LBL? |
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Definition
| Lymph nodes. T-LBL - thymus, skin, tonsils, liver, spleen, CNS. |
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Term
| With B-ALL/LBL - which has a better prognosis - Hyper or Hypo ploidy? |
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Definition
| Hyper - typical in children, rare in adults. Hypo is more severe, not frequent in adults or kids. |
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Term
| What chromosome is the precursor for ALL/LBL neoplasms? |
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Definition
| Philadelphia - causes BCR-ABL2 mutations. Same as in CML except ABL2 instead of 1. |
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Term
| Is the transolaction of the Philadelphia chromosome more frequent in kids or adults? |
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Definition
| Adults - not frequent at all in kids. Unfavorable prognosis. |
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Term
| What is the sanctuary site for ALL/LBL? Why? |
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Definition
| Testis and CNS - impervious to chemotherapeutic agents. |
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Term
| What is the most common leukemia in the Western world? |
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Definition
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Term
| What symptoms do most people with CLL have? |
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Definition
| Asymptomatic typically, but if symptoms present - lymphadenopathy or hepatosplenomegaly. Also anemia, neutropenia, or thrombocytopenia. |
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Term
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Definition
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Term
| What is the predominant cell in CLL/SLL? |
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Definition
| Small, round to slightly irregular B-cell. There are also larger B-cells w/ nucleoli called prolymphocytes and para-immunoblasts. |
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Term
| How are CLL/SLL diagnosed? |
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Definition
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Term
| Who gets Follicular Lymphoma? |
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Definition
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Term
| What is the cell of origin of FL? |
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Definition
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Term
| What is the disease process w/ FL? |
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Definition
| Germinal center cells contain heterogenous collection of many diff. cells, also macs, containing apoptotic debris, dendritic cells, T-cells. |
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Term
| What cells are contained in FL neoplastic germinal centers? |
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Definition
| More monotonous pop - centrocytes and centroblasts - but usually cytes. |
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Term
| T/F the neoplastic cells in FL germinal ctrs are packed loosely. |
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Definition
| False - packed tightly - lack the cuff of small mature lymphocytes that surround normal germinal ctrs. Thus when lymph node architecture becomes effaces by neoplastic germinal ctrs - it's called follicular. |
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Term
| T/F The higher the grade of FL - the more centroblasts that are present. |
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Definition
| True - then it behaves more aggressively w/ more blasts. |
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Term
| T/F Diffuse large B-cell lymphoma (DLBCL) is most common in kids. |
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Definition
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Term
| What is the cell of origin in DLBCL? |
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Definition
| Many cells that arise from diff. points of antigen stimulated B-cell development. |
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Term
| What is the morphology of DLBCL? |
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Definition
| Large - bigger than a mac nucleus - size of 2 mature small lymphocytes. |
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Term
| What B-Cell antigens are typical in DLBCL? |
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Definition
| CD19, CD20, and antigens that reflect the pt of antigenic stimulated dev. where they arose. |
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Term
| T/F Burkitt Lymphoma is benigh. |
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Definition
| False very aggressive. 2-3 days can cause death if near trachea. |
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Term
| T/F Burkitt lymphoma is a neoplasm of B-cells. |
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Definition
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|
Term
| What are the 3 types of BL? |
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Definition
| Burkitt Lymphoma - Endemic BL, Sporadic BL, Immunodeficient BL. |
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Term
| What are areas of involvement w/ Endemic BL? |
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Definition
| Jaws - other facial bones. |
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Term
| Who is affected with Endemic BL? |
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Definition
| Children - mostly in Africa. |
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Term
| What virus is seen with BL? |
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Definition
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|
Term
| What is the site of infection w/ Sporadic BL? |
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Definition
| Ileo-cecal area - leading to presenatation w/ an abdominal mass. |
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Term
| What causes Immunodeficient BL? |
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Definition
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|
Term
| What is the cell of origin of BL? |
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Definition
| Small non-cleaved germinal center cell. |
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Term
| What is the morphology of a BL cell? |
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Definition
| Medium sized cell w/ basophilic cytoplasm that contains prominent vacuoles. Nucleus is round and contains finely clumped chromatin and multiple medium sized nucleoli. |
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Term
| What are the immunophenotypic findings w/ BL? |
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Definition
| B-cell antigens and antigens that reflect its germinal ctr origins. |
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Term
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Definition
| Monoclonal plasma cells that all secrete a single Ig or portions that are the same size. |
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Term
| What is the order of frequency of diff. types of paraprotein in myeloma? |
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Definition
| IgG > IgA > Bence Jones protein > IgD > IgE > IgM. |
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Term
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Definition
| Igs found in the gamma region. Normal people have a smear, people w/ monoclonal populations have a spike that is a single dark band. |
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|
Term
| What is CRAB associated with? |
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Definition
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Term
|
Definition
| End organ dmg from PCM - hyperCalcemia, Renal failure, Anemia Bony lesions. |
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|
Term
| T/F Patients w/ Smoldering Myloma have end organ dmg. |
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Definition
| False - only those w/ PCM. |
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|
Term
| What are the symptoms of SM? |
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Definition
| Smoldering Myeloma - asymptomatic. Can progress to PCM. |
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|
Term
| What is the site of proliferation of PCM and SM? |
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Definition
| Bone marrow - both involved and uninvolved areas. |
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|
Term
| What do cells of PCM look like? |
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Definition
| Abnormal - atypical - immature. |
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|
Term
| What are the radiographic findings with PCM? |
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Definition
| Osteoclasts are activated - so lytic bony lesions are a feature of PCM. |
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|
Term
| What is the immunophenotypic finding w/ a proliferation of neoplastic plasma cells (PCM)? |
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Definition
| Only kappa/lambda light chains in the cytoplasm due to monoclonal Igs. |
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Term
| What's the difference btw PCM and SM? |
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Definition
| Presence of end organ dmg. With SM you nee dto see monoclonal plasma cell proliferaiton for SM to diagnose (w/ no end organ dmg). |
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|
Term
| What is the cell of origin of a NLPHL? |
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Definition
| Germal ctr, centroblastic B-cell. |
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|
Term
| What is the morphology of NLPHL cells? |
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Definition
| Large folded multilobulated nuclei w/ a small nucleolus. |
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|
Term
| What disease has "popcorn cells"? |
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Definition
| NLPHL - Large folded, multi-lobulated nuclei w/ a small nucleolus. |
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|
Term
| What cells are predominant in an NLPHL LN? |
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Definition
| Small histocytes, dendritic cells, popcorn cells. |
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|
Term
| What antigen do LP cells exhibit? |
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Definition
| CD45. Called lukocyte common antigen b/c it's express by almost all myeloid and lymphoid cells. |
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Term
| T/F LP (Popcorn cells) cells express CD20, CD15, CD45, and CD30. |
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Definition
| False - Only 20 and 45, not 15 or 30. These are expressed by CHL cells. |
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|
Term
| What accounts for the vast majority of HL? |
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Definition
|
|
Term
| What is the morphology of CHL cells? |
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Definition
| 2 kinds - Reed Steinberg and Hodgkin cells. Collectively called HRS cells. |
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|
Term
| What is the cell of origin of CHL cells? |
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Definition
| B- cells in germinal centers. |
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|
Term
| Which cells are diagnostic in CHL? |
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Definition
|
|
Term
| What is the shape of Reed-Steinberg cells? |
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Definition
| Large w/ large bilobed nucleus, eosinophilic. Central nucleolus w/ a clear area surrounding the nucleolus. |
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|
Term
| What cell has pink cytoplasm? |
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Definition
|
|
Term
| What is the diff. between a Reed-Steinberg cell and a Hodgkin Lymphoma cell? |
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Definition
| The same, except a HL cell has a non-lobated nucleus. |
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|
Term
| T/F CHL Hodgkin cells are in the majority. |
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Definition
| False - in minority to inflammatory cells that surround it. Include eosinophils, lymphocytes, plasma cells and histiocytes. |
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|
Term
| Which, CHL or NLPHL has a full-fledged B-cell immunophynotype? |
|
Definition
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