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CHAPTER 86 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF LYMPHOCYTE AND PLASMA CELL DISORDERS

CHAPTER 86 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF LYMPHOCYTE AND PLASMA CELL DISORDERS
Williams Hematology

CHAPTER 86 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF LYMPHOCYTE AND PLASMA CELL DISORDERS

THOMAS J. KIPPS

Classification
Clinical Manifestations

B-Lymphocyte Disorders

T-Lymphocyte Disorders
Chapter References

This chapter outlines the major categories of lymphocyte and plasma cell disorders. Such disorders can be sorted into three main groups. The first is comprised of diseases caused by defects that are intrinsic to lymphoid cells. The second is caused by disorders that result from factors extrinsic to lymphoid cells. The third is comprised of disorders caused by neoplastic or preneoplastic lymphoid cells and is outlined in Chap. 96. While the clinical manifestations of diseases in any one of these three groups may be difficult to distinguish, this grouping can provide a framework with which to proceed in evaluating patients with known or suspected lymphocyte disorders. This chapter introduces this framework and presents a road map to the chapters in the text that discuss each of these disorders in greater detail.

CLASSIFICATION
Lymphocyte and plasma cell disorders can be classified into three major groups. The first group is comprised of lymphocyte disorders that are due to intrinsic defects in lymphoid cells that result in functional abnormalities of marrow-derived (B) lymphocytes, thymic-derived (T) lymphocytes, or both (impaired humoral and cellular immunity) (Table 86-1). These disorders primarily are due to inborn errors in lymphocyte metabolism (see Chap. 81 and Chap. 88) and/or receptor/ligand expression (see Chap. 15 and Chap. 88). These are grouped together as “primary disorders” in Table 86-1. Next are disorders that are caused by factors extrinsic to lymphocytes resulting in immune dysfunction. These conditions most commonly are the result of infection with viruses or other cellular pathogens (see Chap. 87, Chap. 89, and Chap. 90) but also may be caused by drugs or systemic disease of nonlymphoid cells. These disorders are listed as “acquired disorders” in Table 86-1. The third group of diseases are comprised of preneoplastic and neoplastic lymphocyte disorders (see Chap. 96).

TABLE 86-1 CLASSIFICATION OF DISORDERS OF LYMPHOCYTES AND PLASMA CELLS

Different categories of lymphocyte and plasma cell disorders may be difficult to distinguish clinically. For one, lymphocyte disorders can have many clinical manifestations that are not restricted to cells of the immune system. Also, disparate disorders can have similar clinical manifestations, and any one disorder may be associated with a diverse array of clinical pathologies.
In some cases, however, the classification of lymphocyte disorders is influenced by the manifestations of the disease. For example, autoimmune hemolytic disease (see Chap. 55, Chap. 56 and Chap. 57) and autoimmune thrombocytopenia (see Chap. 117) are caused by the inappropriate secretion of autoantibodies by lymphocytes. The blood cell that is coated with autoantibody is presumably normal, yet we classify the disease that can result from hemolytic autoantibodies as an acquired hemolytic anemia. This is because that aspect of the disease is more visible and better understood than is the inappropriate synthesis of antierythrocyte antibody by the disturbed lymphocyte population(s). These disorders are not considered here.
In addition, many diseases, especially infection (e.g., tuberculous adenitis), inflammatory states (e.g., rheumatoid arthritis), autoimmune disease (e.g., systemic lupus erythematosus), or metastatic carcinoma can involve lymph nodes or the spleen as a secondary alteration. These disorders also may be associated with abnormal production of antibodies, such as those resulting in the lupus anticoagulant (see Chap. 128). These disorders also are not considered here because the primary disease is not generally considered a lymphocyte disorder per se.
CLINICAL MANIFESTATIONS
B-LYMPHOCYTE DISORDERS
IMMUNOGLOBULIN DEFICIENCY
The clinical manifestations of B-lymphocyte disorders include the consequences of B-lymphocyte deficiency, dysfunction, or malignant transformation and may consist of a specific deficiency of one of the immunoglobulin isotypes or of several or all normal Ig molecules (panhypogammaglobulinemia) (see Chap. 83). Inability to synthesize or secrete antibodies impairs the clearance of pathogens due to the inability to opsonize microorganisms for phagocytosis, resulting in immune deficiency (see Chap. 88).
ABNORMAL IMMUNOGLOBULIN PRODUCTION
Excess production of immunoglobulin by a clone of B cells can result in essential monoclonal gammopathy (see Chap. 105). This could result from a primary defect in the B-cell clone or expansion of a clone in response to chronic antigen stimulation. Essential monoclonal gammopathy could be a harbinger for development of B-cell neoplastic disease, such as plasma cell myeloma (see Chap. 106) or Waldenström macroglobulinemia (see Chap. 108). Production of abnormal immunoglobulin molecules or immunoglobulin fragments also can be seen associated with chronic infection, leading to development of immunoglobulin heavy-chain disease (see Chap. 109). Deposition of immunoglobulin or immunoglobulin fragments can contribute to formation of amyloid (see Chap. 107). Reactivity of the immunoglobulin with self-antigen(s), such as those found on the red cell membrane (see Chap. 55 and Chap. 56), can result in systemic autoimmune disease.
T-LYMPHOCYTE DISORDERS
IMPAIRED IMMUNOREGULATION
The clinical manifestations of deficiencies or excesses of T lymphocytes depend on the subset of T lymphocytes involved. For example, delayed hypersensitivity normally is mediated by CD4-positive helper T cells (TH cells) and more specifically TH1-type cells (see Chap. 84). A deficit or functional disturbance in these T cells can impair the cellular immune response to mycobacteria, listeria, brucella, fungi, or other intracellular organisms associated with the formation of immune granulomas. TH2-type CD4-positive helper T cells, on the other hand, appear better suited to induce B-cell responses to antigen and direct the immune response against parasitic infestations (see Chap. 84). Depletion of CD4 T cells in patients infected with human immunodeficiency virus accounts in large part to the acquired immune deficiency that develops in patients infected with this virus (see Chap. 89).
T lymphocytes within a marrow allograft are responsible for initiation of the graft-versus-host reaction (see Chap. 18). The acute form of the reaction can lead to severe dermatitis, gastroenteritis, and hepatitis. The chronic syndrome simulates a collage of vascular diseases, such as scleroderma, xerophthalmia, xerostomia, and pulmonary insufficiency. Eosinophilia, hypergammaglobulinemia, development of autoantibodies, and plasmacytosis also can occur. Infection with classical or opportunistic pathogens is a common complication of both acute and chronic graft-versus-host disease. A similar qualitative reaction, albeit more limited, is seen in mononucleosis that results from Epstein-Barr virus infection (see Chap. 90).
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Books@Ovid
Copyright © 2001 McGraw-Hill
Ernest Beutler, Marshall A. Lichtman, Barry S. Coller, Thomas J. Kipps, and Uri Seligsohn
Williams Hematology

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