Immunoblastic Lymphadenopathy with Mixed Cryoglobulinemia

Abstract

The premise that chronic antigenic stimulation may be involved in lymphoproliferative disorders was considered in a patient with immunoblastic lymphadenopathy who had received liver extract by injection and by mouth for many years. The salient features were lymphadenopathy and hepatosplenomegaly, a predominance of lymphocytes and plasmacytoid cells with mitotic figures in lymph-node imprints, a cryoglobulin containing IgG, IgA, IgM and bound complement components, depressed serum complement levels, and Coombs-test-positive erythrocytes. Immunoglobulin concentrations per 100 ml of serum were IgG, 5900 mg, IgA, 1480 mg, and IgM, 5640 mg, with normal ranges of 710 to 1540, 60 to 490, and 37 to 204 mg, respectively. Serum precipitins to an antigen (or antigens) in the liver extract resided in the IgA and IgM classes. Complete remission followed one course of cyclophosphamide, vincristine, and prednisone. We propose that the syndrome was caused by chronic antigenic stimulation with liver extract.

A New Case of Gamma-Heavy Chain Disease

“…If we take into account the possibility of transition from an immuno blastic lymphadenopathy to an immunoblastic sarcoma [23], it becomes significant to weigh the possibility of a chronological relationship between the two groups mentioned. In this way it is interesting to comment that in various cases the diagnosis was carried out in patients who had previously been subject to so-called autoimmune disease: rheumatoid arthritis [14,35], Sjogren's syndrome [13], myastenia gravis [21], hemolytic anemia [34], lupus erythematosus [30], This could be considered somewhat more than sheer chance, especially if one takes into account the clinical observations which would make one suggest a possible relationship between a prolonged antigenic stimulation and the development of a nonneoplastic lymphadenopathy and the transi tion from this to a lymphoplasma-reticulum proliferative malignant dis ease [23,27], A third group would consist of those cases in which a predominant in filtration of plasma cells were found, lacking in atypia or even without it, but with malignant features due to effacing of the normal lymph node structure [24,26]. It should be noted that 2 patients died from plasma cell leukemia [30,34], As regards the immunochemical diagnosis of the abnormal protein, it must be stressed that in spite of its high concentration, the electrophoresis of the serum did not disclose any M-component, because the abnormal y-protein had an a2-globulin mobility and was confused with the usual as pect of this fraction, although it was increased.…”

Rapidly fatal respiratory failure and angioimmunoblastic lymphadenopathy: possible contributions of immunoblastic leukaemia, chemotherapy, and multiple antibodies directed against mature blood cells.

“…Similarly, any rise in lymphocytotoxic antibodies after chemotherapy, as postulated for the antihaemocytic antibodies above, would exert a minimal cytotoxic effect. Hypocomplementaemia has been described in other cases of AIL,5 [24][25][26] but the findings in this case suggest that lymphocytotoxic antibodies may directly reduce the blood lymphocyte concentration and consume complement in the process so that the resultant low complement concentrations would abrogate further lymphocytotoxic effects. This emphasises the need for serial monitoring in such cases, not only of the total peripheral blood lymphocyte concentrations, but also of the concentrations of different lymphocyte subpopulations, complement levels, and the presence or absence of immune complexes.…”

“…Accepted for publication 9 October 1980 monary infiltration by immunoblasts,2 5 1319-23 and additional immunological aberrations such as the presence of multiple autoantibodies,10 hypocomplementaemia,5 [24][25][26] and cutaneous anergy all well reviewed by Cullen et al 8 We report on a case of AIL exhibiting the special features of IBL, pulmonary infiltration, and some previously undescribed serum antibodies directed against neutrophils, lymphocytes, and platelets. The possible interrelations of these features are discussed in detail, and particularly the immunological changes induced by chemotherapy since these may have contributed significantly to the morbidity and death of the patient.…”

Immunoblastic lymphadenopathy—report of a case

“…Lukes and Tindle (1975) consider it as a hyperimmune proliferation *of the B-cell system which may be triggered off as a result of hypersensitivity reaction to therapeutic agents. Schultz and Yunis (1975) reported a case of immunoblastic lymphadenopathy associated with a prolonged administration of liver extract, suggesting that the syndrome may be caused by chronic antigenic stimulation. Occurrence of malignant lymphoma is well known in association with disorders of the immune system such as systemic lupus erythematosus, rheumatoid arthritis, Sj6gren's syndrome and alpha chain disease (as cited by Rappaport and Moran, 1975).…”