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  • Polyclonal lymphocyte and plasma cell disorders can be classified into two major groups: primary disorders and acquired disorders. See Table 48–1.

    — Primary disorders result from defects intrinsic to B lymphocytes (eg, X-linked agammaglobulinemia), T lymphocytes (eg, congenital thymic aplasia), and/or natural killer cells, the latter usually coupled with a B- or T-cell deficiency (eg, interleukin-7 receptor α-chain deficiency) (see Chap. 50).

    — Acquired disorders result from physiologic or pathophysiologic responses to extrinsic factors, usually infectious agents (eg, Epstein-Barr virus or human immunodeficiency virus infection) (see Chaps. 51 and 52).

  • Monoclonal (neoplastic) lymphocyte and plasma cell disorders are discussed in Part VIII and are classified in Chap. 53. Specific neoplastic disorders are discussed in Chaps. 54 to 71.

  • Lymphocyte disorders can have clinical manifestations that are not restricted to cells of the immune system (eg, leprosy or systemic lupus erythematosus).

  • In some cases, classification is influenced by disease manifestations:

    — Diseases caused by production of pathologic autoantibodies (eg, autoimmune hemolytic disease [see Chap. 2224] and autoimmune thrombocytopenia [see Chap. 73]).

    — Diseases caused by excess production of lymphocyte cytokines (eg, chronic inflammatory disorders).


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