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Lecture 22

Malignant Lymphoma: Non-Hodgkin Lymphoma

(T or F) There are no recognized benign neoplasma of the hematopoietic-lymphoid system. True.
(T or F) Lymphomas are clonal. True. These neoplasms contain clonal expansions of a single functional subpopulation
(T or F) The site of the primary disease of Non-Hodgkin Lymphomas is important. True.
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.
What is the characteristic immunophenotypic profile of follicular lymphoma? CD20+ (pan B-cell), CD10+, and abundant monotypic surface immunoglobulin.
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
What is the cell of origin in Follicular Lymphoma? B follicular center cells
What is defined as Grade I follicular lymphoma? Follicular lymphoma mostly composed of small cleaved cells
What is defined as Grade II follicular lymphoma? Follicular lymphoma composed of a mixture of small cleaved and large transformed cells
What is defined as Grade III follicular lymphoma? Follicular lymphoma largely made up of large cells.
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.
What is the immunophenotypic profile of B-Small Lymphocytic Lymphoma (B-SLL)? Weak CD20, CD5, CD23, and weak surface immunoglobulin.
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)
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.
Is a B-Small Lymphocytic Lymphoma (B-SLL) tumor considered high grade or low grade? Low grade
What are common sites of MALT Lymphomas? Extranodal tissue of the GI tract and lungs.
(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.
What is the immunophenotypic profile of Mantle Cell Lymphoma (MCL)? CD20+, CD5+, CD23-, and Surface Ig+
What is the characteristic genotype of Mantle Cell Lymphomas? t(11;14)(q13;q32) translocation
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.
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)
Where is the endemic form of Burkitt Lymphoma found? Young children of Africa
Where is the sporadic form of Burkitt Lymphoma found? Children of all ages in the United States
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
What virus plays an important role int he pathogenesis of Burkitt Lymphoma? Epstein-Barr Virus
What is the immunophenotypic profile of Burkitt Lymphoma neoplastic cells? CD20+, CD10+, and surface Ig+
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.
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)
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.
What is the immunophenotypic profile of Precursor T-lymphoblastic Lymphoma (T-LL) neoplastic cells? Immunophenotypic markers of immature T cells
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)
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.
Definition of epidermotropism The invasion of the epidermis by a band-like superficial dermal infiltrate of neoplastic lymphocytes in mycosis fungoides (MF).
Defintion of Pauter's microabscess Small collection of neoplastic lymphocytes in the epidermis in mycosis fungoides (MF).
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)
Created by: UVAPATH4