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Non-Hodgkin Lymphomas, Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Marginal Zone Lymphoma (MALToma) -heterogeneous, tumor similar to marginal zone B-cells -can arise in LN, spleen, extranodal*(esp mucosal sites) Extranodal-middle-aged adults 1. in chronic inflam tissues of autoimmune or infx dz -Sjogren, Hashimoto, H. pylori 2. Remain localized long time->spread late in course 3. Various lymphoid stages, even plasma cells Process begins as reactive, polyclonal immune rxn ->monoclonal B-cell neoplasm emerges ->distant spread possible ->transformation to Diffuse LARGE B-CELL LYMPHOMA, Non-Hodgkin Lymphomas PERIPHERAL T-CELL/NK NEOPLASMS Periph T-cell Lymphoma, unspecified -'wastebasket'-no pathognomonic feature -LN architecture effaced -contain small, intermed, large malignant T cells -can have reactic eos, macs due to T-cell cytokines -can have angiogenesis -MATURE T-CELL PHENOTYPE-TdT(-) (+)T-cell a:b, g:d receptors (+)CD2, CD5, CD3 -lots w/ TCR rearrangements CLINICAL-generalized LNs, eosinophilia, pruritus, fever, wt loss HIGH RELAPSE-worse than diffuse large B-cell, Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Burkitt Lymphoma-CHILDREN-30% child NHL in US -African, sporadic, HIV-ass'd types -LNs effaced by diffuse intermed-sized lymphocyte infiltrate (10-25nm) -round-oval nuc w/ coarse chromatin -several nucleoli -moderate faint baso/amphophilic cytosol -many tingible body macs for phago->STARRY SKY -can be leukemic-clumped chromatin, 2-5 nucleoli -royal blue cytosol w/ clear vacuoles IMMUNOPHENOTYPE-surface IgM, CD19, CD20, CD10 -all w/ translocations -t(8;14)-IgH, c-myc, t(2;8), t(8;22) -EBV in almost all African, 25% of HIV-ass'd -infx before cellular transformation CLINICAL-most at EXTRANODAL sites -African-mandible, abd viscera (kidneys, ovaries, adrenal) -sporadic (non-endemic)-abd mass-ileocecum, peritoneum TX- curable w/ short-term high-dose CTX, Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Mantle Cell Lymphoma -3% of NHL, MALES 40-50s yo -resemble mantle zone cells 2 patterns: 1)Mantle zonenodular, surround NL B-cells 2)Diffuse Both-homogeneous pop of sm. lymphos -round-irreg cleaved nucleus -condensed chromatin, no nucleoli, scant cytosol NO centroblasts, prolif centers (follic. lym, SLL/CLL) Periph blood lymphocytosis in 20-40% Most w/ marrow involvement-non-and paratrabecular -Splenic white pulp, periportal liver, extranodal common ->most likely to cause lymphomatoid polyposis IMMUNOPHENOTYPE-CD19, CD20, CD5, surface IgD, IgM (-)CD23 (CLL/SLL), mantle cells usually CD5(-) -t(11;14)-IgH, BCL-1 (cyclin D1) in 70% CLINICAL-generalized LN, spleen (50%), liver, marrow B Sx in minority PROGNOSIS-3-4 yr medial survival-incurable -succumb to organ dysfcn -transformation less than SLL/CLL, follic lymphoma, Non-Hodgkin Lymphomas PERIPHERAL T-CELL/NK NEOPLASMS BOTH more common in Asia than US, Non-Hodgkin Lymphomas PERIPHERAL T-CELL/NK NEOPLASMS Adult T-cell Leuk/Lymphoma -CD4 T cells in HTLV-1-infx pts -S. Japan, Caribbean basin -virus in tumor cells -skin lesions, generalized LNs, big spleen, liver -periph blood lymphocytosis -hypercalcemia -multilobated nuclei-'cloverleaf/flower' -maybe some multinuc giant cells (mimic RS) FATAL əYR DESPITE CTX -if just in skin, resembles MF - indolent, Non-Hodgkin Lymphomas PERIPHERAL T-CELL/NK NEOPLASMS Mycosis Fungoides, Sezary Syndrome -cutaneous lymphoid neoplasm -neoplastic periph CD4 T-cells to epidermis, dermis -cerebriform nuc w/ infolding of nuc. Inflammatory PREMYCOTIC phase ->PLAQUE phase -> TUMOR phase ->LNs, marrow -25% have tumor cells in periph SEZARY SYNDROME-same, but w/ GENERALIZED EXFOLIATIVE ERYTHRODERMA -doesn't lead to tumefaction -ass'd leukemia of Sezary cells INDOLENT tumors w/ 8-9 yr median survival Transformation to LARGE CELL lymphoma occasionally -terminal event, Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Dyscrasias (gammopathies, dysproteinemia, paraproteinemia) Plasma Cell Neoplasms-monoclonal (mostly IgG>IgA)others rare -terminally diff'ated B cells -many malignancies->excess heavy &/or light chains -'M component'=mIg-in plasma, extracell. fluid -can be in urine w/ glom dmg -if just light chains->free light chains=Bence Jones proteins -totally cleared from blood in NL kidney in urine b/c small MULTIPLE MYELOMA-(plasma cell myeloma)-MOST COMMON -multiple tumor masses throughout skeleton, excess Ig -IL-1b, IL-6 activate osteoclasts -vertebrae>ribs>skull>pelvis (all start in medullary cavity) -can move to spleen, liver, kidney, LNs, lungs ->hypercalcemia -B-J proteinuria in 70% pts -MYELOMA KIDNEY-due to B-J proteins->amyloidosis -neoplastic cells-30% of mass-perinuclear hof(from golgi) -plasma cells or plasmablasts predominate -or multinuc cells, flame cells, Mott cells (blue cyto droplets) -other inclusions-fibrils, rods, Russell, Dutcher bodies(nuc) -can also spread to LNs, skin - 50-60yo MEN -NL humorla immunity suppressed->Strep, Staph, E.coli ETIOLOGY-less diff'ated than plasma cell -myelomonocytic, megakary, erythroid markers- -CD33, GpII/III, GLYCOPHORIN -depends on IL-6 to survive (from plasma & stromal cells) -has human herpesvirus 8 (Kaposi'slarge B-cell lymphoma) -deletion sof 13q, rearrangements of 14q -30% w/ t(4;14)-FGFR3 w/ IgH->cellular proliferation can ->plasma cell leukemia -POOR ə YR PROGNOSIS, NO CURE -'SOLITARY MYELOMA' if only one mass-3-5% plasma cell CAs -early stage of MM, but can be there fore 10-20 yrs -can have macroglobulinemia w/ IgA, IgG3 WALDENSTROM MACROGLOBULINEMIA-syndrome w/ lots of IgM HEAVY-CHAIN DZ-in CLL/SLL, LPL, Med. Lymphoma -heavy chain Ig fragments made and secreted PRIMARY or IMMUNOCYTE-MEDIATED AMYLOIDOSIS -from monoclonal prolif of plasma cells w/ LIGHT CHAINS (BENCE JONES-esp LAMBDA) MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE -MGUS-M components in blood for no good reason, no Sx -in 1-3% NL older pts=DYSPROTEINEMIA W/OUT DZ -MOST COMMON monoclonal gammopathy, no B-J -periodic check-up for progression, Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Diffuse Large B-Cell Lymphoma -20% of NHL, 60-70% of aggressive lymphoid neos -5% of childhood lymphoma -60 yo median age-MALE slightly MORPHOLOGY- Cells 4-5x small lymphocyte -diffuse growth -round/oval vesicular nucleus b/c chromatin at periph -can be multi-lobated or cleaved -2-3 nucleoli on periph, or 1 central -moderate cyto, pale or basophilic -if more anaplastic, can resemble RS cells IMMUNOPHENOTYPE-(+)CD19, CD20, CD10, Ig (-)TdT -30% w/ t(14;18) -20-30% w/ BCL6 breakpoint (extranodal types) -better prognosis SPECIAL SUBTYPES- -iMMUNODEFICIENCY-ASSOCIATED-W/ T-cell def -late HIV, SCID, x-plants ->latent EBV infx -BODY CAVITY LARGE...-malignant pleural/ascitic -late HIV, elderly -clonal IgH, no B, T markers -infx w/ human herpesvirus 8->Kaposi's Sarcoma CLINICAL-rapidly enlarging,(+)Sx mass at 1 node/site -GI, skin, bone, brain as presenting feature -oropharynx lymphoid -destructive masses in liver, spleen -bone marrow in late dz PROGNOSIS-rapidly fatal if unTx CTX->remission in 60-80%, 50% 'cured', Non-Hodgkin Lymphomas PERIPHERAL B-CELL NEOPLASMS Lymphoplasmacytic Lymphoma (LPL) (Waldenstrom Macroglobulinemia) -50-60 yo -sim to CLL/SLL but terminal diff'ation to plasma cells -mostly mIgM secreted->hyperviscosity syndrome (WM) -diffuse, sparse-heavy marrow w/ lymphos, plasmas, plymphos +reactive hyperplasia of mast cells -can be similar in liver, spleen, LNs if disseminated -can infiltrate meninges, nerve roots -Russell bodies in cytoplasm, Dutcher bodies in nucleus -no bone erosion CLINICAL -weakness, fatigue, wt loss -50% w/ LN, splenomegaly, hepatomegaly -anemia via marrow infiltration->autoimmune hemolysis in 10% -via COLD AGGLUTININS-IgM -hyperviscosity syndrome->visual impairment, neuro problems, bleeding, cryoglobulinemia(->Raynaud, cold hives) PROGNOSIS -incurable, progressive TX-plasmapheresis-median 4 yr survival -uncommon transformation to LARGE CELL LYMPHOMA