Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intravenous immunoglobulin (IVIG) may be considered first-line maintenance therapy for idiopathic thrombocytopenic purpura (ITP) because it has been proven to be the least toxic. In a study of 25 children with acute ITP, treatment with IVIG maintained platelet counts above 40,000/mm3 in all of the children. After 1 year, none of these patients required further therapy. In another study group of 25 pediatric patients with chronic ITP, treatment with IVIG circumvented splenectomy in 60% of the cases. The therapeutic regimens for adults and children are described, as is a strategy to overcome IVIG resistance. Experience with IVIG in hemolytic anemia and neutropenia are discussed. The mechanism of action is explored in some detail, specifically as it relates to reticuloendothelial system (RES) Fc receptor blockade and suppression of antiplatelet antibody synthesis.
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PMID:Therapy in cytopenia. 376 52

A case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome), associated with a microcystic adenoma of the pancreas is reported. Early recognition of this rare entity is important, as corticosteroids and splenectomy are usually ineffective, whereas removal of the tumor may be a cure for this condition. Possible mechanisms of the hemolytic anemia and thrombocytopenia are also discussed.
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PMID:Evans' syndrome associated with microcystic adenoma of the pancreas. 381 8

The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.
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PMID:Splenic growth rates in cirrhotic and other splenomegalic diseases of childhood. 384 62

The role of cytoplasmic microtubules in the phagocytosis of yeast cells by blood monocytes was studied by means of therapeutic concentrations of the Vinca alkaloids vincristine, vinblastine, and vindesine. Phagocytosis was measured in a monolayer of glass-adherent monocytes fed with fluorescein-labelled yeast cells. Phagocytosis was composed of two sequential processes, yeast cell adherence to the monocyte and yeast cell engulfment by the monocyte. Monocyte phagocytosis was significantly inhibited by the Vinca alkaloids, mainly due to inhibition of engulfment but probably also due to inhibition of adherence. Since the Vinca alkaloids are microtubule antagonists it is reasonable to assume that monocyte phagocytosis, both adherence and engulfment, are partially microtubule-dependent processes. It is suggested that Vinca alkaloid inhibition of monocyte phagocytosis is of value in the treatment of type II-III autoimmune disorders, such as idiopathic thrombocytopenic purpura and autoimmune haemolytic anaemia.
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PMID:The phagocytosis of yeast cells by blood monocytes. Effects of therapeutic concentrations of Vinca alkaloids. 391 91

The anatomy and pathology of the splenic red pulp was studied in three-dimensional reconstructions of methylmethacrylate embedded blocks of tissue obtained after splenectomy, as well as by morphometrical analysis of a large number of specimens. The sinuses of the spleen form a plexus of anastomosing vessels with remarkable buds. Capillaries end as sheathed capillaries in the cord tissue, the 'filtering' area, but a large proportion of the red pulp cords appear to be 'non-filtering'. These might form part of the lymphatic compartment, which is separate from the white pulp and its extension along the capillaries. This area has not yet been described in man. The change in the volume and structure of the various components of the red pulp were studied in 60 controls and in cases of traumatic rupture, idiopathic thrombocytopenic purpura, aplastic anaemia, autoimmune haemolytic anaemia, congenital spherocytosis, splenic congestion, and Hodgkin's disease. Significant differences were found in the volume of filtering and non-filtering areas, the size of the sinus compartment, and the degree of vascularization; these differences were only partially expected, for instance in disorders with excessive erythrocyte sequestration. A decrease of the 'non-filtering' area in Hodgkin's disease might indicate an unknown aspect of this disease. In agreement with our previous paper on the amount of white pulp, spleens removed because of traumatic rupture and those incidentally removed during abdominal surgery may not be combined as a single control group, because of significant and probably functional differences in the composition also of the red pulp.
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PMID:The splenic red pulp; a histomorphometrical study in splenectomy specimens embedded in methylmethacrylate. 400 86

In a series of 316 surgically removed spleens, a histological and supportive immunohistological study was performed on methylmethacrylate sections. The structure of the human white and red pulp differs from the rat spleen in many respects, e.g. the human lacks the marginal sinus and the architecture of the periarteriolar lymph sheath seen in the rat. In man, the lymphoid compartment is in both white and red pulps. In the white pulp separate periarteriolar T-cell areas contain a large lymph-vessel plexus, which was reconstructed in serial sections. The circulation in the red pulp is discussed. The area between the red and white pulp, the perifollicular zone, is not the equivalent of the marginal sinus in the rat. Its anatomy in man suggests that it is an area formed from red pulp during the expansion of new follicles. The micro-anatomy was analysed in 119 controls. In cases of traumatic rupture the white pulp showed evidence of stimulation. A pathognomonic histological picture was not found in idiopathic thrombocytopenic purpura. In haemolytic anaemia the pulp cords were engorged by erythrocytes accompanied by a decreased B/T cell ratio in autoimmune haemolytic anaemia and by an increased B/T cell ratio in congenital spherocytosis.
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PMID:The human spleen; a histological study in splenectomy specimens embedded in methylmethacrylate. 404 Aug 84

By light and electron microscopy, we observed foamy cells in the spleens from a patient with hemolytic anemia due to red cell adenosine deaminase (ADA) overproduction, a patient with rheumatoid arthritis (RA) treated with gold, and patients with idiopathic thrombocytopenic purpura (ITP). The foamy cells associated with red cell ADA overproduction were essentially similar to Gaucher-like cells described in patients with thalassemia, and it was suggested that the accelerated destruction of red cells was one of the factors responsible for the development of foamy cells. Foamy cells in ITP and RA were closely associated with an increased destruction of platelets in the spleen. Morphologic transitions between phagocytosed platelets and myelin-like materials were traced in these disorders. In RA, however, foamy cells were heterogeneous from an ultrastructural standpoint, with different cytoplasmic inclusions. In addition to myelin-like materials, dense bodies, vacuoles with flocculent materials, and gold were noted in most of foamy cells. As gold compounds are known to inhibit lysosomal enzymes, we surmise that an acquired disturbance in lysosomal digestion is partially responsible for the accumulation of intermediate metabolites. In the pathogenesis of foamy cells associated with blood cell dyscrasia, the accelerated destruction of blood cells and/or acquired disorders in catabolic pathways within the macrophages are suggested to be the underlying mechanism of an intralysosomal accumulation of incompletely degraded cellular debris.
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PMID:Three kinds of foamy cells in the spleen: comparative histochemical and ultrastructural studies. 404 43

Virus-like particles have been seen on electronmicroscopic examination of four spleens surgically removed for haematological disorders-three cases of idiopathic thrombocytopenic purpura and one case of haemolytic anaemia. The particles were approximately spherical and 50-60 nanometres in diameter.
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PMID:Unusual particles in splenic disorders. 516 11

The rapid rise in platelet count after immunoglobulin treatment in acute and chronic forms of idiopathic thrombocytopenic purpura (ITP), autoimmune neutropenia, and post-transfusion purpura is well documented. It is suggested that the rise in platelet count is due to competitive inhibition of the macrophage binding of platelets by preferential sequestration of immunoglobulin-coated red blood cells. Measurement of haptoglobin levels, a sensitive indicator of haemolysis, suggests that clinically inapparent haemolysis occurs during immunoglobulin therapy of ITP patients. In-vitro experiments confirm that there is immunoglobulin coating of red blood cells. The hypothesis is further supported by the findings that immunoglobulin treatment in autoimmune haemolytic anaemia is ineffective, and that platelet counts rise in some ITP patients after induction of a mild haemolytic syndrome by injection of anti-Rho (D).
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PMID:Effect of intravenous immunoglobulin in immune thrombocytopenia. 613 31

Idiopathic thrombocytopenic purpura developed in a 31-year-old man, and he underwent a splenectomy because of his failure to respond to steroid therapy. Subsequently, during a six-year follow-up, chronic active hepatitis, Coombs' positive hemolytic anemia, and pulmonary interstitial fibrosis developed. Since such a clustering of autoimmune manifestations in a single subject is unusual, possible contributing factors were sought. We suggest that both the splenectomy and the histocompatibility antigen HLA-B8, which the patient was found to carry, may have contributed to this rare clinical syndrome.
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PMID:Multiple autoimmune manifestations in a splenectomized subject with HLA-B8. 660 30


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