{"id":5351,"date":"2021-05-15T23:53:42","date_gmt":"2021-05-15T21:53:42","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/aggressive-lymphomas-dlbcl-burkitt-mantle-cell-t-cell\/"},"modified":"2021-05-27T15:39:51","modified_gmt":"2021-05-27T13:39:51","slug":"aggressive-lymphomas-dlbcl-burkitt-mantle-cell-t-cell","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/lymphomas\/aggressive-lymphomas-dlbcl-burkitt-mantle-cell-t-cell\/","title":{"rendered":"Aggressive lymphomas (DLBCL, Burkitt, mantle cell, T-cell)"},"content":{"rendered":"\n

Make sure you cover the lymphomas<\/a> article first.<\/em><\/p>\n\n\n\n

Aggressive lymphomas are those lymphomas that are fast-growing and progressing, but they are generally considered curable.<\/div><\/p>\n\n\n\n

They include:<\/p>\n\n\n\n

  • DLBCL<\/li>
  • Burkitt<\/li>
  • Mantle-cell<\/li><\/ul>\n\n\n\n

    Diffuse large B-cell lymphoma<\/h4>\n<\/span>\n\n\n

    A group of B-cell lymphomas with a morphology resembling large and diffuse germinal centers in which there are large cells (3-4 times more than normal) with prominent nuclei.<\/p>\n\n\n\n

    • The most common type of Non-Hodgkin lymphomas (NHL) in adults.<\/li>
    • Divided into several groups with the largest group being DLBCL, NOS (non-specified).<\/li>
    • Presents with rapidly progressing lymphadenopathy typically involving extra-lymphatic tissues as well (bone marrow, GI, CNS, kidneys), with the GIT being most common.<\/li>
    • Can arise from follicular lymphoma.<\/li>
    • Associated with t(14;18), BCL2, BCL6, and MYC mutations.<\/li>
    • Treatment:
      • First line: R-CHOP<\/li>
      • Salvage: R-DHAP, R-ICE<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

        Burkitt’s lymphoma<\/h4>\n<\/span>\n\n\n

        A rapidly-growing B-cell lymphoma (the fastest growing tumor) appearing in the germinal center; divided into endemic (African) and non-endemic forms.<\/p>\n\n\n\n

        • “Starry-sky” histological appearance.<\/li>
        • The typical patient is an African child seen with a massive swelling in his jaw. <\/li>
        • Associated with EBV<\/li>
        • Associated with t(8;14) MYC mutations.<\/li>
        • Excellent prognosis; treatment includes CODOX-M\/IVAC + intrathecal therapy.<\/li><\/ul>\n\n\n<\/span>\n

          Mantle cell lymphoma<\/h4>\n<\/span>\n\n\n

          Mantle cell lymphom is a B-cell lymphoma composed of cells resembling the naive B cells found in the mantle zones of normal lymphoid follicles.<\/p>\n\n\n\n

          • Moderately aggressive and incurable.<\/li>
          • Distinguished from CLL\/SLL by the lack of proliferation centers.<\/li>
          • t(11;14) fusing the cyclin D1 gene onto the IgH locus leading to the overexpression of cyclin D1 and hyperproliferation.<\/li>
          • Treatment includes R-CHOP, proteasome inhibitors (Bortezomib), BTK inhibitors (Zanubrutinib).<\/li><\/ul>\n\n\n<\/span>\n

            T-cell lymphomas<\/h4>\n<\/span>\n\n\n

            Lymphomas that originate in the T-cells are commonly aggressive and can be fast-growing.<\/p>\n\n\n\n

            • Adult T-cell leukemia\/lymphoma<\/strong>
              • A very aggressive tumor caused by HTLV-1 (human T-cell leukemia virus 1).<\/li>
              • Associated with skin lesions, lymphadenopathy, hepatosplenomegaly, hypercalcemia, and variable lymphocytosis.<\/li>
              • Treatment includes interferon-alpha (IFN) and zidovudine (AZT).<\/li>
              • 8 months survival.<\/li><\/ul><\/li>
              • Peripheral T-cell lymphoma<\/strong>
                • A diverse group of unclassifiable, aggressive tumors arising from peripheral (post-thymic) T-cells.<\/li>
                • These are tumors of functional T cells, patients often suffer from symptoms related to tumor-derived inflammatory products.<\/li>
                • Treatment: CHOP<\/li><\/ul><\/li>
                • S\u00e9zary syndrome<\/strong>
                  • A type of cutaneous T-cell lymphoma presenting with a widespread itchy rash, erythroderma, and malignant cells in the blood (S\u00e9zary cells).<\/li>
                  • Treatment:
                    • PUVA<\/strong> and UVB<\/strong><\/li>
                    • Retinoids<\/strong><\/li>
                    • Interferon-alpha<\/strong><\/li>
                    • Anti-CD52<\/strong> (alemtuzumab) mAb<\/li>
                    • Anti-CD30<\/strong> (brentuximab vedotin) mAb<\/li><\/ul><\/li>
                    • If diagnosed late, 1-3 year survival.<\/li><\/ul><\/li><\/ul>\n<\/span>

                      Diffuse large B-cell lymphoma<\/h4>

                      Burkitt’s lymphoma<\/h4>

                      Mantle cell lymphoma<\/h4>

                      T-cell lymphomas<\/h4><\/div>","protected":false},"excerpt":{"rendered":"

                      Make sure you cover the lymphomas article first. They include: DLBCL Burkitt Mantle-cell Diffuse large B-cell lymphoma A group of B-cell lymphomas with a morphology resembling large and diffuse germinal centers in which there are large cells (3-4 times more than normal) with prominent nuclei. The most common type of Non-Hodgkin lymphomas (NHL) in adults. […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5478,"menu_order":1,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5351","page","type-page","status-publish","hentry"],"yoast_head":"\nAggressive lymphomas (DLBCL, Burkitt, mantle cell, T-cell) – Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/lymphomas\/aggressive-lymphomas-dlbcl-burkitt-mantle-cell-t-cell\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" 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