Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis.

Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease. The nonamyloid forms were found in 13 patients who had evidence of plasmacytic dyscrasia by the immunohistochemical detection of immunoglobulin light chains of kappa or lambda class (with or without staining for a single heavy chain isotype) and by the absence of amyloid P component in tissue sections that did not show the birefringence characteristic of amyloid after Congo Red staining. All but two of the patients presented with proteinuria with or without azotemia. Clinical syndromes involving other organ systems were less common but occasionally severe. Four patients had overt multiple myeloma. Three others had hypercalcemia and mild bone marrow plasmacytosis but no lytic lesions. Analyses of immunoglobulin synthesis in bone marrow cells from seven patients showed excess light chains in all and incomplete light chains or heavy chain fragments in six, regardless of whether an intact monoclonal protein or related subunit was in the serum or urine. The fibrillar (amyloidotic) and nonfibrillar forms of monoclonal immunoglobulin deposition occur either in overt multiple myeloma or in the course of less neoplastically aggressive plasmacytic dyscrasias. Bone marrow cells from patients with either type produce immunoglobulin fragments that are related to those deposited in the affected tissues.

Lymphomas associated with the acquired immune deficiency syndrome (AIDS): a study of 35 cases.

An increased incidence of lymphoid neoplasias is associated with the states of immune deficiency both congenital and acquired. Thirty-five cases of lymphoma in males at high risk for AIDS were diagnosed in one community hospital in New York City within the past 2 years. The mean age of these patients was 39.6 years; 34 were homosexual, and one was an intravenous drug abuser. There were four Hodgkin and 31 non-Hodgkin lymphomas of various histologic types but almost all of high-grade categories. The proportion of extranodal lymphomas, the involvement of the gastrointestinal tract, central nervous system, bone marrow, and myocardium were significantly higher than in the lymphomas of the general population. The phenotypes were B-cell and non-B-non-T-cell types without any T-cell lymphomas. All patients had reversed helper-suppressor T-cell ratios, and all those tested had circulating HTLV-III and antilymphocyte antibodies. Nine patients have had previous lymph node biopsies showing the lesions of AIDS-related lymphadenopathies that were often directly associated with lymphoma. A variety of severe opportunistic infections and Kaposi sarcoma affected these patients. All lymphomas associated with immune deficiency were highly aggressive, involved multiple organs, and responded poorly to treatment resulting in early deaths.

Lymphomas in men at high risk for acquired immune deficiency syndrome (AIDS). A study of 21 cases.

An increased incidence of lymphoid neoplasias is associated with the states of immune deficiency, both congenital and acquired. Twenty-one cases of lymphoma in men at high risk for Acquired Immune Deficiency Syndrome (AIDS) were diagnosed in one community hospital in New York City within the last 2 years. The mean age of these patients was 39.6 years, 20 were homosexual, and 1 was an intravenous drug abuser. There were 3 Hodgkin's and 18 non-Hodgkin's lymphomas of various histologic types, but almost all of high-grade categories. The proportion of extranodal lymphomas, the involvement of the gastrointestinal tract, central nervous system, bone marrow, and myocardium were significantly higher than in the lymphomas of the general population. The phenotypes were B-cell and non-B-non-T-cell types without any T-cell lymphomas. All patients had reversed helper-suppressor T-cell ratios and all those tested had circulating HTLV-III antibodies. Seven patients have had previous lymph node biopsies performed, showing the lesions of AIDS-related lymphadenopathies that often were directly associated with lymphoma. A variety of severe opportunistic infections and Kaposi's sarcoma affected these patients. All lymphomas in this group were highly aggressive, involved multiple organs, and responded poorly to treatment, resulting in early deaths.