Pathology of dysproteinemia: light chain amyloidosis, non-amyloid immunoglobulin deposition disease, cryoglobulinemia syndromes, and macroglobulinemia of Waldenström.
This review has dealt with four syndromes associated with dysproteinemia, and has emphasized studies of the tissue deposits and forms of tissue injury which occur in such patients. However, similar tissue deposits and tissue damage occasionally occur in the absence of a serum or urine paraprotein, in which case other clinical data are necessary to suggest the need for examination of tissue for Ig heavy and light chain determinants in order to provide a correct diagnosis of dysproteinemia. In such cases, one may speculate that there is a low rate of paraprotein production and secretion, in addition to tissue tropism. Some paraproteins are antibodies, in which case they may circulate and/or deposit as immune complexes, or bind to tissue antigens with immune complex formation in situ. Some paraproteins are also cryoproteins, and clues to this property can also be found in the tissue, particularly at the ultrastructural level. Thus, a wide spectrum of clinical manifestations of a B cell proliferative disorder may be associated with any of a variety of circulating paraproteins and a variety of forms of tissue deposit and injury. Consequently, the best understanding of an individual patient requires correlation of the clinical features of the disorder, the immunochemical characterization of the circulating and excreted paraproteins, and an immunohistochemical analysis of the tissue deposits and associated morphologic abnormalities. This should be correlated with histologic and immunohistologic assessment of bone marrow, looking for overt B cell neoplasia, the more difficult to define "lymphoproliferative disorders," or alterations in kappa to lambda plasma cell ratios which may correlate with the deposited material. Studies of the Ig synthesized by cultured bone marrow plasma cells, and biochemical analyses of the deposited material, have demonstrated structural abnormalities of paraproteins which may be responsible for their tissue deposition.
D000361:Agammaglobulinemia / Q000276:immunology / Q000473:pathology; D000686:Amyloidosis / Q000276:immunology / Q000473:pathology; D003449:Cryoglobulinemia / Q000276:immunology / Q000473:pathology; D006801:Humans; D007147:Immunoglobulin Light Chains; D007136:Immunoglobulins / Q000032:analysis; D007150:Immunohistochemistry; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology*; D008258:Waldenstrom Macroglobulinemia / Q000276:immunology / Q000473:pathology
D007147:Immunoglobulin Light Chains; D007136:Immunoglobulins