1/16. Autoimmune pancreatitis presenting as a mass in the head of the pancreas: a diagnosis to differentiate from cancer.We report a case of autoimmune pancreatitis presenting as a mass in the head of the pancreas that was successfully diagnosed without pancreaticoduodenectomy. The patient was a 64-year-old man who had no complaint. A routine physical checkup unexpectedly revealed mild diabetes and a low-echoic mass in the pancreatic head. The diagnosis was made by noting irregular narrowing of the main pancreatic duct, hypergammaglobulinemia, and increased immunoglobulin g levels. An open wedge biopsy of the mass was performed; this showed a marked fibrosis with lymphocyte- or macrophage-predominant inflammatory infiltrates. Immunohistochemical study revealed that the remnant acinar cells expressed Fas (CD95) ligand and not Fas. We review some of the literature and discuss various features and diagnostic clues of autoimmune pancreatitis. awareness of this pathologic condition may prevent confusion with pancreatic malignancy and unnecessary surgery.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/16. Autoimmune pancreatitis with effective steroid therapy.Autoimmune pancreatitis has recently been described as a clinical entity that causes chronic pancreatitis. This unique form of chronic pancreatitis is characterized by minimal attacks of abdominal pain, irregular narrowing of the pancreatic duct, and a diffuse enlargement of the pancreas. Autoimmune pancreatitis is associated with hypergammaglobulinemia. In addition, there is histological evidence of lymphoplasmacytic inflammation, the occasional coexistence of other autoimmune diseases, and has a favorable response to glucocorticoid treatment. Recently autoimmune pancreatitis has been increasingly reported particularly in japan. We report two patients with autoimmune pancreatitis who were treated successfully with corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/16. Acquired chronic pure red cell aplasia successfully treated with intravenous pulse methylprednisolone therapy.A 65-year-old male patient developed acquired chronic pure red cell aplasia (PRCA) associated with hypergammaglobulinemia and positive Coombs' test during the treatment of eosinophilic pneumonia with prednisolone (PSL). His PRCA was treated with oral PSL at a dose of 60 mg/day for 3 weeks, but anemia further progressed. Immediately after high-dose intravenous pulse methylprednisolone therapy (1 g/day for 3 days) however, reticulocyte crisis occurred and his anemia rapidly improved. He has been in complete remission under a maintenance dose of PSL for more than 2 years. This patient indicates that high-dose intravenous methylprednisolone therapy is one of the useful treatments, not only for constitutional PRCA, but also for acquired chronic PRCA.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/16. Peripheral CD8 /CD25 lymphocytes may be implicated in hepatocellular injuries in patients with acute-onset autoimmune hepatitis.BACKGROUND: The mechanism of liver injuries in autoimmune hepatitis (AIH) is not fully understood, especially because the onset is insidious and the clinical courses fluctuate with spontaneous exacerbation and improvement even without immunosuppressive therapies. methods: Eleven patients with acute-onset AIH, some of whom were without hypergammaglobulinemia or anti-nuclear antibodies, and 41 patients with chronic AIH were compared serologically, biochemically, and histologically to determine differences in liver injuries between patients with acute-onset and chronic AIH. All patients fulfilled the diagnostic criteria according to a scoring system proposed in 1999. RESULTS: lymphocytes with CD8 /CD25 markers in pretreatment blood were significantly more prevalent in acute-onset than chronic AIH patients (24% vs 14%; P < 0.05). After treatment, however, CD8 /CD25 lymphocytes were fewer in patients with acute-onset than in those with chronic AIH at 1 and 2 weeks ( P = 0.0001). No other differences were noted in clinical characteristics or immunological parameters between patients with acute-onset and those with chronic AIH. In a patient with typical acute-onset AIH, CD8 /CD25 lymphocytes increased and decreased in parallel with the activity of liver disease. CONCLUSIONS: Activated CD8 T lymphocytes with CD25 markers may be implicated in the development of acute-onset AIH.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/16. Autoimmune pancreatitis--an uncommon type of chronic pancreatitis.Autoimmune pancreatitis is a recently recognized clinical entity characterized by narrow strictured main pancreatic duct on ERCP, diffusely enlarged sausage-shaped pancreas on CT scan and MRI, seropositivity for antinuclear antibodies, hypergammaglobulinemia, and excellent response to steroids. We report a 25-year-old man and a 53-year-old man with this condition.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/16. The disappearance of germinal centers in chronic lymphadeno-hepato-splenomegaly syndrome in childhood: report of three cases.Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/16. Chronic idiopathic neutropenia with hypergammaglobulinemia.Chronic idiopathic neutropenia can occur in spite of the normocellular bone marrow in myeloid series with or without anti-neutrophilic antibody. We report a patient with chronic neutropenia and severe polyclonal hypergammaglobulinemia. The patient demonstrated a positive anti-neutrophil antibody by fluorocytometry, although granulocyte-specific anti-nuclear factor and anti-stem cell (CFU-GM) antibody were negative. Thus, neutropenia of this patient appeared to be due to the antibody-mediated destruction of neutrophils. Both neutropenia and hypergammaglobulinemia were normalized by the administration of prednisone.- - - - - - - - - - ranking = 6keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/16. Acute liver biopsy lesions in early autoimmune ("lupoid") chronic active hepatitis.We studied two female patients with autoimmune ("lupoid") chronic active hepatitis whose liver biopsies at initial presentation showed the unusual features of an acute hepatitis. Centrilobular hepatocyte swelling and multinucleation, acidophilic degeneration, cholestasis, mild fatty change and bile duct damage in one case resembled lesions of non-A, non-B hepatitis. Lobular and portal infiltrates of plasma cells with piecemeal necrosis suggested transition to chronicity as well as an autoimmune component. This was additionally supported by the presence of hypergammaglobulinemia and auto-antibodies in both patients. We conclude that liver biopsy features in the acute presentation of lupoid hepatitis may be difficult to distinguish from those seen in acute hepatitis due to virus or drugs.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/16. Long lasting lymphadenopathy in childhood as an expression of a severe hyperimmune B lymphocyte disorder.On the basis of the 6 cases reported here and scattered published cases, the existence of a childhood chronic immunoblastic lymphadenopathy syndrome is proposed. It is characterized by the following features: A systemic immunoblastic proliferation with varying degrees of maturation resembling the B lymphocyte hyperimmune disorder observed in angioimmunoblastic lymphadenopathy but with no deposits of interstitial amorphous material or vascular proliferation. Disseminated superficial and deep lymphadenopathy. Chronic, pronounced splenomegaly. A constant thrombocytopenia. A polyclonal hypergammaglobulinemia, with markedly elevated antibody titers to various agents. An early onset and a course of several years (up to 20), interrupted in half the cases by the occurrence of a virus-associated (EBV, Papova) neoplastic process or a fatal viral infection. Low natural killer (NK) cell activity in 2 cases.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/16. Chronic liver disease manifesting as Waldenstrom's macroglobulinemia.A 59-year-old woman was initially seen with anemia, purpura, gastrointestinal tract and vaginal bleeding, pronounced hyperglobulinemia, and an increased serum viscosity, all suggestive of Waldenstrom's macroglobulinemia. Subsequent investigation, however, indicated that these abnormalities were more likely due to chronic active hepatitis with cirrhosis. The striking similarity of this patient's symptoms to those of Waldenstrom's macroglobulinemia and the pathophysiologic characteristics of polyclonal hypergammaglobulinemia in chronic liver disease are discussed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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