Classification and Clinical Manifestations of Polyclonal Lymphocyte and Plasma Cell Disorders | Williams Manual of Hematology, 9e | AccessHemOnc

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 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

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

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CLASSIFICATION

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