M

Figure 2 Macroglobulinemia is a disease in which monoclonal plasma cells in the marrow, depicted in the lower two panels, secrete a monoclonal IgM protein, shown in the upper two panels. The plasma cells are monoclonal as shown with fluoresceinated antibody to kappa light chain (lower right panel). The secreted IgM monoclonal protein is shown to be kappa light chain restricted by immuno-fixation (upper right panel).

Table 3 Miscellaneous monoclonal gammopathies

Lichen myxedematosis (also called papular mucinosis or scleromyxedema) Light chain deposition disease Cryoglobulinemia (type I and type II) Adult acquired Fanconi syndrome Systemic capillary leak syndrome Acquired CI esterase inhibitor deficiency Cold agglutinin syndrome reflects deposition of amyloid. The kidneys and heart, nerves, liver, gastrointestinal tract, tongue and blood vessels are prone to develop amyloid deposits. The recognition of AL and being able to distinguish it from the nonimmunoglobulin forms of secondary, familial and senile amyloid depends on the recognition of monoclonal plasma cell proliferation by immunoelectrophoresis and immunofixation of the serum and urine, and by the detection of a monoclonal increase in plasma cells in the marrow using immunofluorescence or immunohistochemical stains for k and X light chain. If a monoclonal plasma cell process is not established it is necessary to specifically stain amyloid tissue using immunohistochemical techniques.

A monoclonal IgM in the serum often indicates the presence of a malignant lymphoproliferative disorder. The outcome reflects the histology of the underlying lymphoproliferation rather than the type or amount of IgM produced. Waldenstroms macroglobulinemia is the syndrome that develops when the IgM levels are high, usually greater than .3 g dl '. Serum hyperviscosity, anemia, and bleeding can result (Figure 2).

Miscellaneous monoclonal gammopathies (Table 3) include lichen myxedematosis, primarily affecting the skin; light chain deposition disease; types I and II cryoglobulinemia; adult acquired Fanconi syndrome, primarily affecting the kidney; systemic capillary leak syndrome, causing episodic hypotension and hemo-concentration; osteosclerotic myeloma causing bias-tic bone lesions, associated with neuropathy and endocrinopathy; acquired CI esterase inhibitor deficiency, causing episodic hives and angioedema and loss of C3 and C4; and cold agglutinin syndrome, causing hemolytic anemia. Heavy chain diseases are covered in another article in this work.

See also: Adhesion molecules; Amyloid; Autoimmune diseases; B lymphocyte differentiation; ß2-microglobulin; Bone marrow and hematopoiesis; CD45 (the leukocyte common antigen); Cold agglutinins; Complement deficiencies; C-reactive protein; Cryoglobulin; Cytokine receptors, soluble; Cytokines; Fas (CD95) and fas ligand; Gammaglobulin; Heavy chain diseases; Immunoelectrophoresis; Immunoglobulin, functions; Immunoglobulin structure; Interleukin 6; Interleukin 6 receptor; Sjögrens syndrome; Stromal cells; Varicella-Zoster virus, infection and immunity.

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