This chapter delineates the major categories of lymphocyte and plasma cell disorders*. The disorders are classified into three main groups. The first is composed of diseases caused by defects intrinsic to lymphoid cells. The second is caused by disorders that result from factors extrinsic to lymphoid cells. The third is composed of disorders caused by neoplastic or preneoplastic lymphoid cells and are further delineated in Chapter 89 using the World Health Organization classification of tumors of lymphoid tissues. The clinical manifestations of diseases in any one of the three groups may be difficult to distinguish, but this grouping can provide a framework with which to proceed in evaluating patients with known or suspected lymphocyte and plasma cell disorders. This chapter introduces the framework and presents a roadmap to other chapters in this book that discuss each of the disorders in greater detail.
Acronyms and Abbreviations
GVHD, graft-versus-host disease; Ig, immunoglobulin; NK, natural killer; SCID, severe combined immune deficiency; Th, T helper; TREG, CD4+ regulatory T cell.
Lymphocyte and plasma cell disorders can be classified into three major groups (Table 77–1). The first group, listed under “primary disorders,” is composed of lymphocyte disorders caused by intrinsic defects in lymphoid cells that result in functional abnormalities of marrow-derived (B) lymphocytes, thymic-derived (T) lymphocytes, combined T and B cells (impaired humoral and cellular immunity), or natural killer (NK) cells. These disorders primarily result from inherited disorders in lymphocyte metabolism (Chaps. 20, 73, and 79) and/or receptor–ligand expression (Chaps. 16 and 79). The second group listed under “acquired disorders,” consists of disorders caused by factors extrinsic to lymphocytes, resulting in immune dysfunction. These conditions most commonly result from an infection with viruses, bacteria, or other pathogens (Chaps. 79, 80, and 81), but they also may be caused by drugs or systemic disease of nonlymphoid cells. The third group of diseases is composed of neoplastic lymphocyte disorders and is discussed in detail in Chapter 89.
TABLE 77–1.Classification of Disorders of Lymphocytes and Plasma Cells ||Download (.pdf) TABLE 77–1. Classification of Disorders of Lymphocytes and Plasma Cells
B-lymphocyte deficiency or dysfunction (Chap. 79)1,2
Associated with plasma cell myeloma, heavy-chain disease, light-chain amyloid, Waldenström macroglobulinemia, or chronic lymphocytic leukemia (Chaps. 91 and 106–109)4,5
Associated with celiac disease6
Autosomal recessive agammoglobilinemia9
Autosomal dominant agammaglobulinemia2,10–13
Common variable immunodeficiency (CVID)14
Transient hypogammaglobulinemia of infancy15
Selective agammaglobulinemia (Chap. 79...