Chapter 105. Malignancies of Lymphoid Cells (Part 5) pdf

Malignancies of Lymphoid Cells Part 5 Table 105-5 Diseases or Exposures Associated with Increased Risk of Development of Malignant Lymphoma Inherited immunodeficiency disease Klinefe

Chapter 105 Malignancies of

Lymphoid Cells

(Part 5)

Table 105-5 Diseases or Exposures Associated with Increased Risk of Development of Malignant Lymphoma

Inherited immunodeficiency disease

Klinefelter's syndrome

Chédiak-Higashi syndrome

Ataxia telangiectasia syndrome

Wiscott-Aldrich syndrome

Common variable immunodeficiency disease

Acquired immunodeficiency diseases

Iatrogenic immunosuppression

HIV-1 infection

Acquired hypogammaglobulinemia

Autoimmune disease

Sjögren's syndrome

Celiac sprue

Rheumatoid arthritis and systemic lupus erythematosus

Chemical or drug exposures

Phenytoin

Dioxin, phenoxyherbicides

Radiation

Prior chemotherapy and radiation therapy

Immunology

All lymphoid cells are derived from a common hematopoietic progenitor that gives rise to lymphoid, myeloid, erythroid, monocyte, and megakaryocyte lineages Through the ordered and sequential activation of a series of transcription factors, the cell first becomes committed to the lymphoid lineage and then gives rise to B and T cells About 75% of all lymphoid leukemias and 90% of all lymphomas are of B cell origin A cell becomes committed to B cell development when it begins to rearrange its immunoglobulin genes The sequence of cellular changes, including changes in cell-surface phenotype, that characterizes normal B

cell development is shown in Fig 105-2 A cell becomes committed to T cell differentiation upon migration to the thymus and rearrangement of T cell antigen receptor genes The sequence of the events that characterize T cell development is depicted in Fig 105-3

Figure 105-2

Pathway of normal B cell differentiation and relationship to B cell lymphomas HLA-DR, CD10, CD19, CD20, CD21, CD22, CD5, and CD38 are

cell markers used to distinguish stages of development Terminal transferase (TdT) is a cellular enzyme Immunoglobulin heavy chain gene rearrangement (HCR) and light chain gene rearrangement or deletion (κR or D, λR or D) occur early in B cell development The approximate normal stage of differentiation associated with particular lymphomas is shown ALL, acute lymphoid leukemia; CLL, chronic lymphoid leukemia; SL, small lymphocytic lymphoma