1/7. New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis b. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/7. Acquired hypolipoproteinemia.We present a six-year follow-up of a boy with a novel type of hypolipoproteinemia, with clinical and biochemical features distinct from classical hypoalphalipoproteinemias. There were abnormally low concentrations of total and high-density lipoprotein (HDL) cholesterol, apolipoprotein (apo) B, apo A-I, and apo A-II, and the phospholipids were decreased. The most striking abnormality was an extra fraction containing mainly phospholipids and apo A-I in the HDL3 subfraction. This fraction is reminiscent of concentric 20- to 50-nm-diameter lamellar phospholipid liposomes. Plasma lecithin:cholesterol acyltransferase activity was strongly decreased. We noted a persisting polyclonal hypergammaglobulinemia, hematological abnormalities (hemolytic anemia and thrombocytopenia), and a progressive splenomegaly. After the five-year follow-up, the patient had recurrent severe infections; moderate hematuria and proteinuria developed gradually. Treatment with corticosteroids and immunoglobulins improved thrombocytopenia and hypolipoproteinemia. These clinical and biochemical findings differ from those in the known primary and secondary hypo-alpha-lipoproteinemia syndromes. Although investigation of the relatives suggests a familial predisposition for hypo-alpha-lipoproteinemia, the subject's condition can be regarded as acquired.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/7. 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) |
4/7. The varied clinical and laboratory manifestations of type II Gaucher's disease.An infant of Arab extraction with the Type II form of Gaucher's disease is described. His clinical presentation was unusual because in addition to the extensive neurological involvement and marked hepatosplenomegaly a severe congestive cardiomyopathy and renal tubular dysfunction were present. In addition, marked hypergammaglobulinemia and raised serum angiotensin converting enzyme levels were found. It is suggested that these varied manifestations may be ascribed to the consequences of glucocerebroside deposition within the macrophages of the reticuloendothelial system.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/7. Idiopathic portal hypertension associated with progressive systemic sclerosis.Herein we describe the clinical course of a case of idiopathic portal hypertension accompanied by splenomegaly, portal hypertension, and pancytopenia in addition to progressive systemic sclerosis. Immunological studies revealed positive antinuclear antibodies, hypergammaglobulinemia with increases in IgG and IgA, a decrease in peripheral T lymphocytes, and a decrease in the rate of lymphocyte blast transformation induced with phytohemagglutinin. The spleen weighed 1070 g, and the portal pressure was 270 nm H2O. Esophageal varices were also noted. These findings suggest the possibility of association of some immunological abnormalities with the etiology of idiopathic portal hypertension.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/7. Helical hairs: a new hair anomaly in a patient with Netherton's syndrome.A new hair shaft defect, helical hair, is presented. This hair abnormality was found to accompany trichorrhexis invaginata and pili torti in an infant with Netherton's syndrome. The patient's main clinical features included erythroderma since birth, failure to thrive, recurrent infections, hepatosplenomegaly, lymphadenopathy, eosinophilia, hypergammaglobulinemia, and high serum IgE levels. A possible mechanism for the formation of helical hair is reviewed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/7. Idiopathic portal hypertension associated with high serum titer of autoantibodies and liver dysfunction.Idiopathic portal hypertension (IPH) is a condition marked by unexplained portal hypertension. Although a number of immunological abnormalities occur in patients with IPH, liver function is usually normal. We experienced an unusual case of IPH in a 49-year-old woman, who had pronounced splenomegaly. Laboratory data revealed pancytopenia, hypergammaglobulinemia, and liver dysfunction. Antinuclear antibodies were positive, with high titer at 1280 dilutions of sera. LE cell phenomena were also positive. Histological examination of biopsied liver showed only mild changes, but portal venous pressure was markedly elevated, at 38 cm H2O. This case was thus characterized by both a high serum titer of autoantibodies and liver dysfunction.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |