1/7. Paraneoplastic syndrome and intrathoracic Castleman disease.We report two cases of intrathoracic Castleman disease presenting with paraneoplastic syndrome. Patient 1 was a 10-year-old girl with short stature. She was found to have delayed bone age, slow growth velocity, and iron-deficiency anemia, which was refractory to treatment. thrombocytosis and hypergammaglobulinemia were later detected. Chest X-ray revealed a hilar mass. Patient 2 was a 14-year-old boy who had severe cough, progressive mucocutaneous erosion, and dermatitis. Chest X-ray showed a mediastinal mass. Sections of skin biopsy showed findings consistent with pemphigus disease. In each case, the histological diagnosis of Castleman disease was made.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/7. Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy.Marked polyclonal immunoglobulin (Ig)G4 hypergammaglobulinemia has exceptionally been reported. Here we report on two Algerian patients who presented a syndrome characterized by anemia, plasmacytic lymphadenopathy, renal manifestations, and a marked polyclonal IgG4 hypergammaglobulinemia leading to a hyperviscosity syndrome in one case. The IgG4-expressing cell percentage was significantly increased in the peripheral blood lymphocytes collected from the two patients upon diagnosis. Moreover, in contrast with normal sera, both patients' sera significantly increased the percentage of IgG4-expressing cells when incubated with CD40-stimulated normal B lymphocytes. Similar effects were obtained with the culture supernatants of the patients' activated T cells. Anti-interleukin (IL) 4 and/or anti-IL-13 antibodies were unable to antagonize the IgG4 production. IL-4 and IL-13 serum concentrations were found to be normal in the two patients. The increased IgG4 production was found to be mediated by soluble factor(s), most probably secreted by activated T cells, which did not require the signal transducer and activator of transcription 6 signaling pathway.- - - - - - - - - - ranking = 6keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/7. immunoblastic lymphadenopathy with purpura and cryoglobulinemia.immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/7. Ribosome-lamella complex of the lymphocytes in a case of plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia.A case of lymphoid hyperplasia with prominent plasmacytic proliferation histologically and with polyclonal hypergammaglobulinemia and anemia clinically was reported. This condition in a 54-year-old Japanese male was probably a type of plasma cell dyscrasia similar to a plasma cell variant of Castleman's disease. About 19% of apparently nonatypical lymphocytes in the biopsy material of this case were found to have ribosome-lamella complexes (RLC) similar to those previously described in patients with hairy cell leukemia and some other hematologic disorders. An intimate association with the rough endoplasmic reticulum of these structures suggested aberrant protein synthesis by b-lymphocytes.- - - - - - - - - - ranking = 5keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/7. Angiomatous lymphoid hamartoma: inhibitory effects on erythropoiesis, growth, and primary hemostasis.An 11-year-old boy was noted to have microcytic anemia, growth retardation, polyclonal hypergammaglobulinemia, and abnormal platelet function. An angiomatous lymphoid hamartoma was removed from the retroperitoneal space. Postoperatively the child exhibited a dramatic growth spurt and complete resolution of the abnormal laboratory measurements. Studies were performed before and after tumor removal to investigate the nature of the associated anemia, growth retardation, and altered hemostasis. There was no evidence of iron deficiency, thalassemia, or an antierythropoietin factor. Prolonged bleeding time and impaired ristocetin-induced platelet aggregation normalized following tumor resection. serum obtained before surgery inhibited lymphocyte proliferation in mixed lymphocyte culture as well as fibroblast growth in vitro. Detailed study of growth regulatory hormones failed to reveal significant alterations except for significantly reduced somatomedin which normalized after surgery. The factor(s) which inhibit in vitro cellular growth and lower in vivo plasma somatomedin concentration remain unknown.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/7. 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) |
7/7. Mediastinal lymph node hyperplasia, hypergammaglobulinemia, and anemia.An adolescent girl with retarded development, delayed puberty, and hypergammaglobulinemia had anemia unresponsive to iron therapy that was cured when a mass of mediastinal giant lymph node hyperplasia was removed surgically. Rapid growth and development of secondary sexual characteristics occurred after operation. follow-up studies made 20 years after operation showed compensated increased erythrocyte hemolysis and increased serum IgM. When mediastinal giant lymph node hyperplasia with abundant plasma cells is seen in childhood, there may be refractory anemia and immune disturbances. Similar giant lymph node hyperplasia without plasma cells may be found in an asymptomatic child or adult. The pertinent features of the few symptomatic cases reported are reviewed.- - - - - - - - - - ranking = 5keywords = hypergammaglobulinemia (Clic here for more details about this article) |