Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ CLASSIFICATION ++ 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. 22–24] and autoimmune thrombocytopenia [see Chap. 73]). — Diseases caused by excess production of lymphocyte cytokines (eg, chronic inflammatory disorders). ++Table Graphic Jump LocationTABLE 48–1CLASSIFICATION OF NONCLONAL DISORDERS OF LYMPHOCYTES AND PLASMA CELLSView Table|Favorite Table|Download (.pdf) TABLE 48–1 CLASSIFICATION OF NONCLONAL DISORDERS OF LYMPHOCYTES AND PLASMA CELLS Primary disorders B-lymphocyte deficiency or dysfunction Agammaglobulinemia Acquired agammaglobulinemia Associated with plasma cell myeloma, heavy chain disease, light chain amyloid, Waldenström macroglobulinemia, or chronic lymphocytic leukemia Associated with celiac disease X-linked agammaglobulinemia of Bruton Autosomal recessive agammaglobulinemia Common variable immunodeficiency Transient hypogammaglobulinemia of infancy Bloom syndrome Comel-Netherton syndrome Selective agammaglobulinemia Immunoglobulin (Ig) M deficiency (1) Selective IgM deficiency (2) Wiskott-Aldrich syndrome Selective IgG deficiency IgA deficiency (1) Isolated asymptomatic (2) Steatorrheic IgA and IgM deficiency IgA and IgG deficiency (1) CD40/CD40L deficiency (2) Activation-induced cytidine deaminase (AID) (uracil-DNA glycosylate [UNG], hyper-IgM) (3) PMS2 deficiency Hyper-IgA Hyper-IgD Hyper-IgE Hyper-IgE associated with HIV infection Hyper-IgM immunodeficiency X-linked lymphoproliferative disease T-lymphocyte deficiency or dysfunction Cartilage-hair hypoplasia Lymphocyte function antigen-1 deficiency Thymic aplasia (DiGeorge syndrome) Thymic dysplasia (Nezelof syndrome) Thymic hypoplasia CD8 deficiency CD3γ deficiency Winged helix deficiency (Nude) Interleukin-2 receptor α chain (CD25) deficiency Signal transducer and activator of transcription 5b (STAT 5b) deficiency Schimke syndrome Janus kinase 3(JAK3) deficiency γc Deficiency Wiskott-Aldrich syndrome Zeta-associated protein of 70kDa (ZAP-70) deficiency Purine nucleoside phosphorylase deficiency Interleukin-7 receptor deficiency Major histocompatibility complex class I or II deficiency Coronin-1A deficiency IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome caused by mutations in FoxP3 that cause a deficiency of CD4+ regulatory T cells (Tregs) APECED (autoimmune polyglandular, candidiasis, and ectodermal dystrophy) syndrome caused by mutations in the autoimmune regulator gene (AIRE) gene Autoimmune lymphoproliferative syndrome Combined T- and B-cell deficiency or dysfunction Ataxia-telangiectasia Combined immunodeficiency syndrome Adenosine deaminase deficiency Thymic alymphoplasia CD45 deficiency X-linked severe combined immunodeficiency syndrome Major histocompatibility complex class II deficiency—bare lymphocyte syndrome IgG and IgA deficiency ... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription