Malignant Lymphoma: Non-Hodgkin Lymphoma
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| (T or F) There are no recognized benign neoplasma of the hematopoietic-lymphoid system. | True.
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| (T or F) Lymphomas are clonal. | True. These neoplasms contain clonal expansions of a single functional subpopulation
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| (T or F) The site of the primary disease of Non-Hodgkin Lymphomas is important. | True.
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| What is the most common type of non-Hodgkin's lymphoma in the Western World? | B-cell Non-Hodgkin Lymphomas make up 85-80% of all non-Hodgkin Lymphomas.
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| What is the characteristic immunophenotypic profile of follicular lymphoma? | CD20+ (pan B-cell), CD10+, and abundant monotypic surface immunoglobulin.
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| Type of NHL that classically presents with peripheral or retroperitoneal adenopathy. Histologic features include: effacement of nodal architecture by proliferation of neoplastic follicular nodules composed of small cleaved cells. | Follicular Lymphoma
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| What is the cell of origin in Follicular Lymphoma? | B follicular center cells
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| What is defined as Grade I follicular lymphoma? | Follicular lymphoma mostly composed of small cleaved cells
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| What is defined as Grade II follicular lymphoma? | Follicular lymphoma composed of a mixture of small cleaved and large transformed cells
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| What is defined as Grade III follicular lymphoma? | Follicular lymphoma largely made up of large cells.
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| What is the predominant genotype of follicular lymphomas? | t(14;18): This translocation juxtaposes the BCL-2 oncogene into the Ig heavy chain locus on chromosome 14 leading to the expression of Bcl-2 protein which blocks apoptotic cell death.
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| What is the immunophenotypic profile of B-Small Lymphocytic Lymphoma (B-SLL)? | Weak CD20, CD5, CD23, and weak surface immunoglobulin.
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| Type of NHL that occurs in older adults that presents with adenopathy and a leukemic blood picture identical to B-chronic lymphocytic leukemia. | B-Small Lymphocytic Lymphoma (B-SLL)
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| What are the histologic characteristics of B-Small Lymphocytic Lymphoma (B-SLL)? | Involved lymph nodes show diffuse effacement by a proliferation of small lymphocytes with round nuclei and dense chromatin.
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| Is a B-Small Lymphocytic Lymphoma (B-SLL) tumor considered high grade or low grade? | Low grade
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| What are common sites of MALT Lymphomas? | Extranodal tissue of the GI tract and lungs.
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| (T or F) MALT lymphomas only arise from lymphoid tissue that is a normal component of the site of origin (eg Peyer's patches of the small intestines). | False. MALT lymphomas may also arise from lymphoid tissue acquired as part of an autoimmune disorder or infeciton.
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| What is the immunophenotypic profile of Mantle Cell Lymphoma (MCL)? | CD20+, CD5+, CD23-, and Surface Ig+
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| What is the characteristic genotype of Mantle Cell Lymphomas? | t(11;14)(q13;q32) translocation
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| What is the molecular defect associated with t(11;14)(q13;q32) translocation? | Translocation of the immunoglobulin heavy chain gene next to the CYCLIN D1 (Bcl-1) gene, which leads to the overexpression of CYCLIN D1.
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| Type of NHL that occurs almost exclusively in adults characterized by diffuse proliferation of large neoplastic B-lymphoid cells. | Diffuse Large B-cell Lymphoma (DLBCL)
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| Where is the endemic form of Burkitt Lymphoma found? | Young children of Africa
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| Where is the sporadic form of Burkitt Lymphoma found? | Children of all ages in the United States
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| Type of NHL that presents in extra-nodal sites such as the jaws, distal ileum, and ovaries. Morphologically, it demonstrates a diffuse proliferation of small noncleaved cells with a very high mitotic rate and a background of "starry sky" macrophages. | Burkitt Lymphoma
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| What virus plays an important role int he pathogenesis of Burkitt Lymphoma? | Epstein-Barr Virus
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| What is the immunophenotypic profile of Burkitt Lymphoma neoplastic cells? | CD20+, CD10+, and surface Ig+
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| What is the genotype of Burkitt Lymphoma neoplastic cells? | Translocation of the MYC gene on chromosome 9 to the Ig heavy chain on chromsome 14. Or, the translocation of the MYC gene to the light chain loci on chromsome 2 or 22.
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| Type of NHL that classically presents as a rapidly growing anterior mediastinal mass in young patients, often male, who may have a respiratory or CV compromise and pleural/pericardial effusion. This lympohma disseminate rapidly to marrow, blood, and LNs. | Precursor T-lymphoblastic Lymphoma (T-LL)
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| What is the histologic findings of thymic tissue in Precursor T-lymphoblastic Lymphoma (T-LL)? | Thymic tissue is effaced by diffuse growth of monomorphic tumor cells that have high mitotic rate, and invade the capsule. The tumor cell hsave a blastic appearance.
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| What is the immunophenotypic profile of Precursor T-lymphoblastic Lymphoma (T-LL) neoplastic cells? | Immunophenotypic markers of immature T cells
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| Type of NHL that is a tumor of small, skin based T0cells, predominantly of the CD4+ subset that occurs in adults. The neoplastic lymphocytes are small and have a characteristic folded, cerebriform nucleus. | Mycosis Fungoides (MF)
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| What is the histologic findings of Mycosis Fungoides (MF)? | Band-like superifical dermal infiltrate adjacent to and invading the epidermis with formation of small collections of lymphocytes in the epidermis. The lymphocytes are small and have a characteristic folded, cerebriform nucleus.
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| Definition of epidermotropism | The invasion of the epidermis by a band-like superficial dermal infiltrate of neoplastic lymphocytes in mycosis fungoides (MF).
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| Defintion of Pauter's microabscess | Small collection of neoplastic lymphocytes in the epidermis in mycosis fungoides (MF).
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| Type of NHL that is characterized by constitutional symptoms with paraneooplastic features (eg eosinophilia and hemophagocytosis). Most cases demonstrate a T-phenotype with loss or aberrant expression of pan T-cell antigens. | Peripheral T-cell Lymphoma (PTCL)
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