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This chapter outlines the category of neoplastic or preneoplastic lymphocyte and plasma cell disorders. It introduces a framework for evaluating neoplastic lymphocyte and plasma cell disorders, outlines clinical syndromes associated with such disorders, and presents a road map to the chapters in the text that discuss each of these disorders in greater detail. Chapter 80 outlines the diseases caused by nonneoplastic disorders of lymphocytes and plasma cells.

Acronyms and Abbreviations

Acronyms and abbreviations that appear in this chapter include: α/β TCR, T-cell-receptor genes encoding the α and β chains of the T-cell receptor (see Chap. 78); ALK, gene encoding anaplastic lymphoma kinase; BCL2, gene encoding B-cell chronic lymphocytic leukemia (CLL)/lymphoma 2; BCL6, gene encoding B-cell chronic lymphocytic leukemia (CLL)/lymphoma 6; cIg, cytoplasmic immunoglobulin; EBV, Epstein-Barr virus; γ/δ TCR, T-cell-receptor genes encoding the γ and δ chains of the T-cell receptor (see Chap. 78); HL, Hodgkin lymphoma; HLA, human leukocyte antigen; HTLV-1, human T-cell leukemia virus type 1; Ig, immunoglobulin; IgR, immunoglobulin gene rearrangement (see Chap. 77); IL, interleukin; MALT, mucosa-associated lymphoid tissue; MUM1, gene encoding multiple myeloma oncogene 1; neg., negative; NK cell, natural killer cell; NPM, gene encoding nucleophosmin; PAX5, paired box gene 5; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes; REAL, revised European-American lymphoma; R-S, Reed-Sternberg; sIg, surface immunoglobulin (see Chap. 77); sIgD, surface IgD; sIgM, surface IgM; TAL1, gene encoding T-cell acute leukemia-1; TCR, T-cell receptor; TdT, terminal deoxynucleotidyl transferase; WHO, World Health Organization.

Lymphocyte and plasma cell malignancies compose a wide spectrum of different morphologic and clinical syndromes (Table 92–1). Lymphocyte neoplasms can originate from cells that are at a stage prior to T- and B-lymphocyte differentiation from a primitive stem cell or from cells at stages of maturation after stem cell differentiation. Thus, acute lymphoblastic leukemias arise from an early lymphoid progenitor cell that may give rise to cells with either B or T cell phenotypes (see Chap. 93). On the other hand, chronic lymphocytic leukemia arises from a more mature B-lymphocyte progenitor (see Chap. 94) and myeloma from progenitors at even later stages of B-lymphocyte maturation (see Chap. 109). Variability in expression of a lymphopoietic progenitor cell disorder may result in the spectrum of lymphocytic diseases, such as a B-lymphocyte or T-lymphocyte lymphoma, and different types of diseases, such as hairy cell leukemia (see Chap. 95), prolymphocytic leukemia (see Chap. 94), natural killer cell large granular lymphocytic leukemia (see Chap. 96),1 myeloma, and plasmacytoma (see Chap. 109). Hodgkin lymphoma also is derived from a neoplastic B cell that has highly mutated immunoglobulin genes that are no longer expressed into protein (see Chap. 99).

Table 92–1. Classification of Lymphoma and Lymphoid Leukemia by World Health Organization

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