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Harrison's Principles of Internal Medicine, 18e
| Part 7. Oncology and Hematology > Section 2. Hematopoietic Disorders > |
Chapter 111. Plasma Cell Disorders
Sections: Plasma Cell Disorders: Introduction, Multiple Myeloma, Waldenström's Macroglobulinemia, Poems Syndrome, Heavy Chain Diseases, Further Readings.
Topics Discussed: pathophysiology of disorders of the immune system; pathophysiology of neoplasia; the genetics of cancer.
Excerpt:"The plasma cell disorders are monoclonal neoplasms related to each other by virtue of their development from common progenitors in the B lymphocyte lineage. Multiple myeloma, Waldenström's macroglobulinemia, primary amyloidosis (Chap. 112), and the heavy chain diseases comprise this group and may be designated by a variety of synonyms such as monoclonal gammopathies, paraproteinemias, plasma cell dyscrasias, and dysproteinemias. Mature B lymphocytes destined to produce IgG bear surface immunoglobulin molecules of both M and G heavy chain isotypes with both isotypes having identical idiotypes (variable regions). Under normal circumstances, maturation to antibody-secreting plasma cells and their proliferation is stimulated by exposure to the antigen for which the surface immunoglobulin is specific; however, in the plasma cell disorders the control over this process is lost. The clinical manifestations of all the plasma cell disorders relate to the expansion of the neoplastic cells, to the secretion of cell products (immunoglobulin molecules or subunits, lymphokines), and to some extent to the host's response to the tumor. Normal development of B lymphocytes is discussed in Chap. 314...."
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