1/14. Angio-immunoblastic lymphadenopathy. diagnosis and clinical course.The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/14. Splenic lymphoma arising in a patient with gaucher disease. A case report and review of the literature.patients with gaucher disease have an increased risk of malignancies, especially the lymphoreticular type. To our knowledge, this is the first reported case of gaucher disease diagnosed during the workup and diagnosis of a splenic marginal-zone lymphoma with progression to diffuse large B-cell lymphoma. There are several theories as to how gaucher disease leads to malignancies. The accumulated glucocerebroside in the reticuloendothelial organs, histologically visible as Gaucher cells, is thought to provide chronic antigenic stimulus to the immune system. Polyclonal hypergammaglobulinemia develops, and monoclonal populations of lymphocytes and plasma cells may arise from this premalignant proliferative state. How gaucher disease is related to nonlymphoid malignancies remains unclear.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/14. immunoblastic lymphadenopathy-like T-cell lymphoma with multiple cutaneous and visceral involvements.Morphologic and immunological findings found in a patient with immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma (IBL-T) are presented. Though the initial clinical features were suggestive of IBL, multiple cutaneous and visceral tumors appeared later in his course. The cutaneous lesion is considered to be unique, because the neoplastic T cells with suppressor/cytotoxic (S/C) phenotype showed focal epidermotropism, resulting in necrosis and ulceration of the overlying epidermis. An interesting feature in IBL-T is the frequent association of polyclonal hypergammaglobulinemia, yet the neoplastic T cells show S/C phenotype. Since Ia-like antigen was expressed on the neoplastic T cells, it is stressed that antigen-presenting and contrasuppressor cells should also be included in the cell populations which have a possibility to be a normal counterpart of IBL-T.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/14. Mesenteric hyaline plasma cell lymph node hyperplasia with amyloid deposits.Lymph node hyperplasia (mixed hyaline vascular and plasma cell type) of mesenteric localization in a young woman was accompanied by noticeable systemic manifestations--fever, highly increased sedimentation rate, anemia, and hypergammaglobulinemia--that disappeared after the tumor was removed. Perivascular deposits of amyloid material were found within the tumor and in the spleen. To our knowledge, this finding has not been previously reported. On the basis of earlier studies in the literature and other considerations, an immunologic disorder is proposed as the cause of both the general symptoms and the amyloid deposits.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/14. mycosis fungoides/Sezary's syndrome progressing to immunoblastic sarcoma. A T-cell lymphoproliferation with both helper and suppressor phenotypes.Multiparameter studies of an unusual patient exhibiting cutaneous manifestations of both mycosis fungoides and Sezary's syndrome are presented. The neoplastic cells of dermal and nodal infiltrates and peripheral blood expressed both helper and suppressor immunologic phenotypes. Cytofluorographic analysis of cells isolated from lymph node and peripheral blood showed a population of neoplastic cells that were stained with the monoclonal antibodies OKT 3, 4, 8, and 11. Immunoperoxidase staining of frozen sections with monoclonal antibodies Leu 1, 2, and 3 provided a topographical identification of an identically marking population of cells in dermis and lymph node. In light of current models depicting normal T-cell lineage, the authors suggest that the neoplastic population in this patient, expressing both helper and suppressor phenotypes, reflected a phenotypic stage of immunologic maturation (OKT 6-, OKT 10-, OKT 3 , OKT 11 , OKT 4 , OKT 8 ) in which the neoplastic cells had not yet segregated into distinctive T-cell subsets. While excess helper activity was suggested by serum hypergammaglobulinemia, in vitro helper and suppressor function was not determined. The range of studies employed illustrates the wide variety of technics required to adequately characterize complex clinico-immunopathologic disorders, as represented by this case, and the wealth of information that can be gleaned.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/14. Helper T-cell lymphoma with marked plasmacytosis and polyclonal hypergammaglobulinemia. A case report.A case of malignant lymphoma with a helper activity of neoplastic cells is reported. On admission, a significant number of plasma cells of polyclonal nature were seen in the peripheral blood, and polyclonal hypergammaglobulinemia was seen. The biopsied lymph node showed poorly differentiated lymphocytic lymphoma with marked proliferation of plasma cells. At the terminal stage, the patient became leukemic in contrast with the disappearance of plasma cells from the peripheral blood. Although the leukemic cells failed to form sheep erythrocyte rosettes, they were considered to be of T-cell origin morphologically. Cytochemically, they had a "dot"-like pattern of alpha-naphthyl acetate esterase and acid phosphatase activity. Ultrastructurally, they had highly convoluted nuclei, and cytoplasmic clustered dense bodies. They showed marked helper activity on pokeweed mitogen-induced B-cell differentiation in vitro. This case may provide a novel view concerning the cause of hypergammaglobulinemia induced by lymphoproliferative disorders.- - - - - - - - - - ranking = 6keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/14. A proposal for smoldering adult T-cell leukemia--diversity in clinical pictures of adult T-cell leukemia--.We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly but flared up after several years. skin lesions appeared in the form of erythema, papules or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was from 0% to 2%. The serum lactic dehydrogenase value was within normal range, and was not associated with hypercalcemia, lymphadenopathy, or hepatosplenomegaly, and bone marrow infiltration was very slight. In most cases, hypergammaglobulinemia was seen, and in one case monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their sera were positive for anti-ATL-associated antigen antibodies. None of them had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness, and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness, and died or cryptococcus meningitis. These cases were clinically and pathologically different from typical ATL cases already reported, and we feel it necessary to make distinctions from the viewpoints of prognosis and treatment. In discussing these cases, we compared smoldering ATL with typical ATL, and deliberated upon the causes of both.- - - - - - - - - - ranking = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/14. Immunologic characterization of a helper T-cell lymphoma.The lymphocytes of a patient with a T-cell non-Hodgkin's lymphoma with peripheral blood involvement and polyclonal hypergammaglobulinemia were characterized in terms of surface markers and immunologic functions. Using the fluorescence-activated cell sorter and employing various monoclonal antibodies against T-cell surface antigens, it was shown that almost all of the patient's peripheral blood lymphocytes were positive for OKT4 and 9.3, antibodies that recognize helper T-cell subset. The circulating lymphoma cells had typical characteristics for T cells; they formed spontaneous rosettes with sheep erythrocytes and stained with the pan-T-cell antibodies 9.6 and 10.2, but did not react with other anti-T-cell monoclonal reagents such as OKT3, UCHT-1, and 3A1. The cells appeared to be mature by the fact that they did not stain with OKT6, and terminal deoxynucleotidyl transferase was undetectable. Functionally, they were able to provide "help" for antibody production, and they could be stimulated to produce moderate amounts of interleukin-2, while unable to proliferate in response to mitogens. Morphologically, some of the lymphocytes showed a deeply cleaved nucleus.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/14. Monoclonal evolution of angioimmunoblastic lymphadenopathy.A patient with angioimmunoblastic lymphadenopathy diagnosed on initial lymph node biopsy was found on rebiopsy two months later to have immunoblastic lymphoma as well. At presentation she had polyclonal hypergammaglobulinemia with polyclonal (kappa and lambda light chains) immunoblasts demonstrated by the immunoperoxidase stain. During the course of her illness, a monoclonal IgM kappa gammaglobulinemia developed, accompanied by monoclonal (kappa light chains only) immunoblasts demonstrated by the immunoperoxidase method. These findings were unique in that lymphomatous transformation and clinical deterioration are shown to be accompanied by a monoclonal evolution of immunoblasts. This constitutes further evidence for the heterogeneous nature of patients who have angioimmunoblastic lymphadenopathy and the malignant potential of this disease.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/14. Multicentric giant lymph node hyperplasia. A hyperimmune syndrome with a rapidly progressive course.A patient who had diffuse lymph node enlargement, fever, skin rashes, anemia and polyclonal hypergammaglobulinemia is described. Histologic examination of lymph nodes taken from different sites (cervical, axillary and inguinal) revealed the presence of giant lymph node hyperplasia. The liver and bone marrow showed a moderate lymphocytic and plasma cell infiltration. The clinical presentation of a multicentric variety of giant lymph node hyperplasia in the reported case is similar to the clinical features usually associated with angio-immunoblastic lymphadenopathy with dysproteinemia, indicating that these two disorders may be related and may affect the same organs and systems. Alternatively, this histologic reactive giant lymph node hyperplasia progressing with a rapid declivitous course can be considered distinctive of a separate entity.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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