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LymphocyticNeoplasia

lecture 17 fuda

QuestionAnswer
reasons for reactive proliferation (lymphocytosis) chronic infection with M. tuberculosis or B. pertussis, viral infections or stress from CVA, MVC or other traumas
pathogenetic/etiological factors of lymphocytic neoplasias radiation/chemo, gene arrangements involving proto-oncogene translocations, oncogenic viral infection (HTLV-1 or EBV), genomic instability syndromes, chronic infection and immune dysregulation (H. pylori MALT lymphoma)
nl B cell markers CD19, 20, 22, 79a (like CD3 for T cells), kappa and lambda Ig light chains
nl T cell markers remember SINGLE DIGITS: CD2, 3, 4, 5, 7, 8
CD45 leukocyte common antigen, expressed on all nl hematolymphoid opulations
Virchow's node aka sentinel or signal node L supraclavicular node that receives drainage from thoracic duct, if it's swollen this indicates an abd ca
markers of immaturity in lymphoid populations (thus seen most only in immature neoplasms) CD34 and TdT also CD1a in T cells
important markers for making distinction btwn classical Hodgkin's lymphoma and nodular lymphocyte predominant Hodgkin lymphoma CD30 & 15, CD45, CD20, CD3
important NK cell markers CD2, CD7, CD8, CD16, CD5
markers useful in subcategorizing B-lineage lymphomas CD10 (expressed by immature B cells [aka hematogones] or mature germinal center B cells) and T-cell marker CD5
marker of cell proliferation, determines the percentage of neoplastic cells actively growing Ki-67 or Mib-1
terms used to describe neoplasms of immature lymphocytes acute, blastic, central or precursor
terms used to describe neoplasms of mature lymphocytes chronic or peripheral
immunoblast transformed, reactive and mature lymphocyte
B-ALL/LBL (B-cell acute lymphoblastic leukemia or just lymphoblastic lymphoma) cells typically express what markers? CD19 (earliest recognizable B cell marker), CD79a (like CD3 co-receptor for T cells), CD22 (inhibits B cell signaling) and at times CD20 (starting at late pro-B cell stage). normally don't express Ig light chains, (+) CD10 at times (immature B cells)
distribution of types of cells in ALL 85% B cell and 15% T cell blasts, also associated marrow suppression
T-ALL/LBL (T-cell acute lymphoblastic leukemia or just lymphoblastic lymphoma) cells typically express what markers? CD1a, 2, 3, 4, 5, 7, 8. since these are blasts, they can also express nonspecific lymphoid immature markers TdT and CD34
morphology of ALL/LBL cells very large and round nuclei, high N/C ratio with more cytoplasm than a nl lymphocyte would have, usually able to see nucleoli
common clinical features of ALL/LBL (usually in young children not older adults) cytopenias due to lymphoblasts filling up marrow and suppressing growth of other lineages (this rapid growth causes bone pain), LAD, organomegaly, fatigue/weakness, petechiae or other hemorrhages, infections. anterior mediastinal masses, IVC syndrome?
lymphoma definition and its 2 broad categories tumor of genetically altered lymphocytes that show evidence of maturation; non-Hodgkins and Hodgkins lymphomas
"grading system" for NHL indolent - comprised of small cells that grow slowly but are disseminated at time of dx /// aggressive - large cells that proliferate quickly but are usually localized
types of indolent NHLs Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL), follicular lymphoma and extranodal marginal zone B-cell lymphoma of MALT
types of aggressive NHLs Burkitt lymphoma and diffuse large B-cell lymphoma
characteristics of follicular lymphoma pts present with advanced dz, painless and progressive LAD. various proportions of centrocytes and centroblasts that express CD10, nodular with disorganized, crowded follicles; involves t(14;18) moving Ig heavy chain to bcl-2 anti-apoptotic locus
centroblasts vs centrocytes blasts: without nuclear grove, round; // cytes: with nuclear groove, "cleaved" cells -- REMEMBER: cyte, cleaved
pathogenetic mechanism behind extranodal marginal zone B cell lymphoma of MALT thought to be due to chronic immune stimulation causing MALT like organization of B cells that acquire mutations in various sites like thyroid, salivary glands, skin, stomach, etc.
characteristics of extranodal marginal zone B cell lymphoma of MALT CD10 and CD5 negative, one of the few curable indolent lymphomas, lymphoepithelial lesions - infiltrative cell into glands,
translocations involved with extranodal marginal zone B cell lymphoma of MALT t(14:18) IGH and MALT1 genes, t(11:18) AP12 and MALT1 genes
characteristics of CLL/SLL indolent, but diffuse leukemia composed of cells which are indistinguishable morphologically from normal mature lymphocytes, CD5+, pts present with anemia, thrombocytopenia, LAD and possibly hepatosplenomegaly
characteristics of diffuse large B cell lymphoma (DLBCL) diffuse infiltration of large lymphoid cells, pts present with rapidly growing mass of extranodal or LN tissue, only CD10+ in 25-50% of cases. 20% of cases have bcl-2 translocation, 30% with bcl-6 translocation
characteristics of Burkitt lymphoma often associated with HIV or EBV infection , diffusely infiltrates tissues and leaves "starry sky" appearance (lots of histiocytes in neoplastic lymphocyte background), CD10 and 38+, Ki-67 usually 100%, involves myc translocation of chr 8 with Ig chain
morphologic salient features of Burkitt lymphoma scattered histiocytes with abundant cytoplasm set in a background of uniformly medium-sized lymphoma cells with round nuclei, peripheral small nucleoli, deeply basophilic cytoplasm, lipid-containing vacuoles, numerous mitotic bodies
classical Hodgkin lymphoma relatively few large neoplastic cells, arises in LN of neck or medastinum, half of pts have B sx, spreads in an orderly fashion as one would expect lymph flow to travel, commonly cured
histological appearance of CHL Reed-Sternberg cells, very few large neoplastic cells (Hodgkin cells), immunophenotype is unclear but usually B-cell origin, diffuse or nodular infiltrate
typical CD marker profile for CHL CD15+ and 30+ // CD 45-, 20-, 3-
Nodular Lymphocyte Predominant Hodgkin Lymphoma indolent dz that usually presents with just one enlarged LN in the neck, axilla or inguinal region; (+) are CD45 and 20, (-) are CD3, 15, 30
plasma cell myeloma near or terminally differentiated plasma cells are hyperproliferative within the bone marrow, make tons of Ig, hyperimmunoglobulinemia is what causes sx
various types of morphologic forms of neoplastic cells seen with Hodgkin lymphoma popcorn cells with bizarre nuclei, Reed-Sternberg cells with bi or multi-lobulated nuclei, monolobate nuclei are Hodgkin cells which are usually very sparse
Created by: sirprakes
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