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Lymphomas

QuestionAnswer
Where both T and B lymphocytes originate from ? * bone marrow
Lymphoid Neoplasms overview ? * Tumors of B-cell, T-cell and NK-cell origin ..... * Common causes: Genetic factors, environmental factors, viruses, smoking, and radiation/chemo
Leukemia = ? * bone marrow Dx made by sampling bd
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
Cx Features of both = ? * malaise and fatique
How to Dx ? * Biopsy of lymph node/BM biopsy, flow cytometry, cytogenetics and IHC necessary for diagnosis
Acute Lymphoblastic Leukemia/Lymphoma (ALL) cells involved ? * precursor T and B Cells
Childhood ALL = ? * B- ALL, but adults can have this too
ALL morpho ? * hypercellular marrow where lymphoblasts keep proliferating .... * see finely stippled chromatin
ALL IHC test ? * myeloperoxidase negative and PAS+
ALL immunophenotype ? * TdT+ with B-cells with CD19, CD10, PAX5
ALL chromosomes ? * translocations of 12;21
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
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
Cells in CLL/SLL we see ? * smudge cells in peripheral smear
CLL/SLL immunophenotypes and chromosomes? * CD23, CD5, surface Immunoglobulin + for both T and B cell markers ... * see trisomy 12q
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
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
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
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
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
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
Mantle Cell Lymphoma basics ? * in 5-6 decades and is very rare
Mantle Cell Lymphoma immunophenotype and Cx ? * Cyclin D1 and NO CD23.... * Molecular – t(11;14) .... * it is not curable
Marginal Zone Lymphoma basics ? * causes MALTomas and arrise to to chronic inflammation in such disorders as Sjogren, Hashimoto, H. pylori
Marginal Zone Lymphoma molecular ? * Up regulate expression & function of BCL10 and MALT1
Hairy Cell Leukemia immunophenotype, molecular, and prognosis ? * lymphocytes with fine hair-like projections.... * Immunophenotype - CD11c, CD25, CD103..... * prognosis is excellent
Hairy Cell Leukemia signs ? * dry tap when trying to get samples to Dx
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
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
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
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
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
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
Created by: thamrick800
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