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 t o 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 delet ion (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. . Chapter 105. Malignancies of Lymphoid Cells (Part 5) Table 105- 5 Diseases or Exposures Associated with Increased Risk of Development of Malignant Lymphoma Inherited. 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. 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