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Chapter 48: Classification and Clinical Manifestations of Polyclonal Lymphocyte and Plasma Cell Disorders

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    AMA Citation
    Classification and Clinical Manifestations of Polyclonal Lymphocyte and Plasma Cell Disorders. In: Lichtman MA, Kaushansky K, Prchal JT, Levi MM, Burns LJ, Armitage JO. Lichtman M.A., Kaushansky K, Prchal J.T., Levi M.M., Burns L.J., Armitage J.O. Eds. Marshall A. Lichtman, et al.eds. Williams Manual of Hematology, 9e New York, NY: McGraw-Hill; . http://hemonc.mhmedical.com/content.aspx?bookid=1889&sectionid=137389449. Accessed April 25, 2018.
    MLA Citation
    . "Classification and Clinical Manifestations of Polyclonal Lymphocyte and Plasma Cell Disorders." Williams Manual of Hematology, 9e Lichtman MA, Kaushansky K, Prchal JT, Levi MM, Burns LJ, Armitage JO. Lichtman M.A., Kaushansky K, Prchal J.T., Levi M.M., Burns L.J., Armitage J.O. Eds. Marshall A. Lichtman, et al. New York, NY: McGraw-Hill, , http://hemonc.mhmedical.com/content.aspx?bookid=1889&sectionid=137389449.

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CLASSIFICATION

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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. 22–24] and autoimmune thrombocytopenia [see Chap. 73]).

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

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Table Graphic Jump Location
TABLE 48–1CLASSIFICATION OF NONCLONAL DISORDERS OF LYMPHOCYTES AND PLASMA CELLS
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TABLE 48–1 CLASSIFICATION OF NONCLONAL DISORDERS OF LYMPHOCYTES AND PLASMA CELLS

  1. Primary disorders

    1. B-lymphocyte deficiency or dysfunction

      1. Agammaglobulinemia

        1. Acquired agammaglobulinemia

        2. Associated with plasma cell myeloma, heavy chain disease, light chain amyloid, Waldenström macroglobulinemia, or chronic lymphocytic leukemia

        3. Associated with celiac disease

        4. X-linked agammaglobulinemia of Bruton

        5. Autosomal recessive agammaglobulinemia

        6. Common variable immunodeficiency

        7. Transient hypogammaglobulinemia of infancy

        8. Bloom syndrome

        9. Comel-Netherton syndrome

      2. Selective agammaglobulinemia

        1. Immunoglobulin (Ig) M deficiency

          • (1) Selective IgM deficiency

          • (2) Wiskott-Aldrich syndrome

        2. Selective IgG deficiency

        3. IgA deficiency

          • (1) Isolated asymptomatic

          • (2) Steatorrheic

        4. IgA and IgM deficiency

        5. IgA and IgG deficiency

          • (1) CD40/CD40L deficiency

          • (2) Activation-induced cytidine deaminase (AID) (uracil-DNA glycosylate [UNG], hyper-IgM)

          • (3) PMS2 deficiency

      3. Hyper-IgA

      4. Hyper-IgD

      5. Hyper-IgE

      6. Hyper-IgE associated with HIV infection

      7. Hyper-IgM immunodeficiency

      8. X-linked lymphoproliferative disease

    2. T-lymphocyte deficiency or dysfunction

      1. Cartilage-hair hypoplasia

      2. Lymphocyte function antigen-1 deficiency

      3. Thymic aplasia (DiGeorge syndrome)

      4. Thymic dysplasia (Nezelof syndrome)

      5. Thymic hypoplasia

      6. CD8 deficiency

      7. CD3γ deficiency

      8. Winged helix deficiency (Nude)

      9. Interleukin-2 receptor α chain (CD25) deficiency

      10. Signal transducer and activator of transcription 5b (STAT 5b) deficiency

      11. Schimke syndrome

      12. Janus kinase 3(JAK3) deficiency

      13. γc Deficiency

      14. Wiskott-Aldrich syndrome

      15. Zeta-associated protein of 70kDa (ZAP-70) deficiency

      16. Purine nucleoside phosphorylase deficiency

      17. Interleukin-7 receptor deficiency

      18. Major histocompatibility complex class I or II deficiency

      19. Coronin-1A deficiency

      20. IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome caused by mutations in FoxP3 that cause a deficiency of CD4+ regulatory T cells (Tregs)

      21. APECED (autoimmune polyglandular, candidiasis, and ectodermal dystrophy) syndrome caused by mutations in the autoimmune regulator gene (AIRE) gene

      22. Autoimmune lymphoproliferative syndrome

    3. Combined T- and B-cell deficiency or dysfunction

      1. Ataxia-telangiectasia

      2. Combined immunodeficiency syndrome

        1. Adenosine deaminase deficiency

        2. Thymic alymphoplasia

        3. CD45 deficiency

        4. X-linked severe combined immunodeficiency syndrome

      3. Major histocompatibility complex class II deficiency—bare lymphocyte syndrome

      4. IgG and IgA deficiency ...

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