Lymphomas
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| Where both T and B lymphocytes originate from ? | * bone marrow
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| Lymphoid Neoplasms overview ? | * Tumors of B-cell, T-cell and NK-cell origin
..... * Common causes: Genetic factors, environmental factors, viruses, smoking, and radiation/chemo
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| Leukemia = ? | * bone marrow Dx made by sampling bd
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| Lymphoma = ? | * Dx made by sampling a lymph node.... * Le and Ly can turn in to each other, it basically depends where the Dx sample was taken
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| Cx Features of both = ? | * malaise and fatique
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| How to Dx ? | * Biopsy of lymph node/BM biopsy, flow cytometry, cytogenetics and IHC necessary for diagnosis
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| Acute Lymphoblastic Leukemia/Lymphoma (ALL) cells involved ? | * precursor T and B Cells
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| Childhood ALL = ? | * B- ALL, but adults can have this too
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| ALL morpho ? | * hypercellular marrow where lymphoblasts keep proliferating .... * see finely stippled chromatin
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| ALL IHC test ? | * myeloperoxidase negative and PAS+
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| ALL immunophenotype ? | * TdT+ with B-cells with CD19, CD10, PAX5
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| ALL chromosomes ? | * translocations of 12;21
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| ALL prognosis ? | * 75-85 % curable when of the B cell type and Dx between 2-10 y/o, and when there is no CNS involvement
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| Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL) basics ? | * same thing, just one was Dx from lymph nodes and the other by blood... * usu in older ppl > 55 and see SUPER high WBC counts 100,000-300,000
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| Cells in CLL/SLL we see ? | * smudge cells in peripheral smear
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| CLL/SLL immunophenotypes and chromosomes? | * CD23, CD5, surface Immunoglobulin + for both T and B cell markers ... * see trisomy 12q
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| CLL/SLL Cx and prognosis ? | * can progress to prolymphocytic leukemia or diffuse
large B-cell lymphoma (Richter syndrome)... * survive for extended periods of time and if ZAP-70 is expressed, there is a poor pronosis
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| Follicular Lymphoma basics ? | * arise from Germinal Center B cells, and forms follicles... * Cells we see: Centrocytes and Centroblasts with Spleen white pulp and hepatic portal triads involved
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| Immunophenotype, Molecular and Cx prognosis of Follicular Lymphoma ? | * + BCL2..... * translocation of 14;18 chromosome.... * Clinical – histologic transformation to diffuse large B-cell lymphoma with less than 1 year
survival after this
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| Diffuse Large B-Cell Lymphoma immunophenotype, molecular, and Cx outcomes ? | * Immunophenotype – CD19, CD20 .... * Molecular – Dysregulation of BCL6 --> normally silences p53 expression ... * Clinical – rapid course; rapidly/aggressive enlarging mass – nodal or extranodal; rarely leukemic/found in the blood
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| Burkitt Lymphoma cells we see ? | * benign macrophages with nuclear remnants of numerous apoptotic cells --> starry sky pattern... * In Bone Marrow – cells have royal blue cytoplasm and clear cytoplasmic vacuoles
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| Immunophenotype, Molecular and Cx prognosis of Burkitts Lymphoma ? | * Immunophenotype – surface IgM, CD19, CD20, CD10, BCL6; Almost never have BCL2 .... * Molecular 3 translocation possibilities 8;14, 8;2, 8;22.... *Prognosis is aggressive, but respond very well
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| Mantle Cell Lymphoma basics ? | * in 5-6 decades and is very rare
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| Mantle Cell Lymphoma immunophenotype and Cx ? | * Cyclin D1 and NO CD23.... * Molecular – t(11;14) .... * it is not curable
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| Marginal Zone Lymphoma basics ? | * causes MALTomas and arrise to to chronic inflammation in such disorders as Sjogren, Hashimoto, H. pylori
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| Marginal Zone Lymphoma molecular ? | * Up regulate expression & function of BCL10 and MALT1
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| Hairy Cell Leukemia immunophenotype, molecular, and prognosis ? | * lymphocytes with fine hair-like projections.... * Immunophenotype - CD11c, CD25, CD103..... * prognosis is excellent
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| Hairy Cell Leukemia signs ? | * dry tap when trying to get samples to Dx
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| Peripheral T-Cell Lymphoma, Unspecified basics ? | * Clinical – generalized lymphadenopathy; eosinophilia, pruritis, fever & weight loss
Worse prognosis than aggressive mature B-cell neoplasms
.... * rule out everything else first
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| Anaplastic Large Cell Lymphoma (ALK Positive) basics ? | * large anaplastic cells with horseshoe-shaped nuclei .... * rearrangement of ALK gene on chromo 2p23 , which trigger JAK/STAT
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| Adult T-Cell Leukemia/Lymphoma basics ? | * Adults infected with HTLV-1 (Human T-cell Leukemia retrovirus type 1).... * multilobated nuclei (clover leaf or flower cells)..... * see mature T-cell markers and CD4 only (NOT CD8)... * can see skin lesions/scabbing look
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| Mycosis Fungoides/Sezary Syndrome basics ? | * Cutaneous T-Cell Lymphoma (CTCL) – presents in the skin and may evolve into generalized
lymphoma.... * Stages: patches, plaques, nodules.... * see Sezary cells and Pautrier microabscesses.... * see CLA, CCR4 and CCR10.... * epidermotropism in skin
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| Large Granular Lymphocytic Leukemia basics ? | * T-cell and NK-cell variants.... * abundant blue cytoplasm containing a few coarse
azurophilic granules (red)(called LGLs)... ** See CD56 **..... * course is dependent on degree of cytopenias
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| Extranodal NK/T-Cell Lymphoma basics ? | * Destructive nasopharyngeal mass but can be in testis and skin.... * get ischemic necrosis... * see EBV episomes in tumor cells
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