Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A typical case of congenital erythropoietic porphyria in a 8 month old girl is reported (photosensitive blistering syndrome, hypertrichosis; erythrodontia; fluorescence of urine, teeth, erythrocytes and myeloblasts; splenomegaly; anaemia). Porphyrins, isomers I, were present in urine and blood. In faeces a porphyrin-X-like substance migrating as PX was detected; this porphyrin was finally identified as CP I unusually difficult to extract because of the presence of a banding and fluorescence quenching agent probably of exogenous origin. The usual methods are not always able to extract and to identify the faecal porphyrins.
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PMID:[Gunther's congenital erythropoietic porphyria in an 8-month old girl (author's transl)]. 84 24

Weanling male rats were fed a Torula yeast diet supplemented with selenium, vitamin E, or both for 3 months. Of rats fed each diet, one group received 250 ppm lead in the drinking water and another group did not. In rats not poisoned with lead, neither vitamin E nor selenium deficiency affected spleen weight, hematocrit value, or erythrocyte mechanical fragility. Vitamin E deficiency increased the splenomegaly, anemia, and mechanical fragility of red cells of lead-poisoned rats, whereas selenium deficiency did not. Addition of 0.5 ppm selenium to the vitamin E-supplemented diet increased slightly the splenomegaly and anemia in lead-poisoned rats. Excess levels of selenium (2.5 and 5 ppm) in the vitamin E-deficient diet had little or no effect on spleen size or hematocrit of rats not receiving lead, but partially prevented the splenomegaly and anemia of red cells from either non-poisoned or lead-oisoned vitamin E-deficient rats, but not as effectively as vitamin E. These results show that vitamin E status of rats is more important that selenium status in determining response to toxic levels of lead. Excess dietary selenium did protect partially against lead poisoning in vitamin E-deficient rats, but the levels of selenium used were toxic in themselves.
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PMID:Comparative effects of selenium and vitamin E in lead-poisoned rats. 84 75

A 69-year-old woman was referred to our department because of moderate anemia and thrombocytopenia. On admission the spleen was slightly enlarged. On the basis of histological examination of biopsy specimens from spinal processes the diagnosis of myelofibrosis was made. The subsequent clinical course was progressively downhill. Although splenomegaly was of only moderate degree, severe anemia and thrombocytopenia supervened. Platelet mean life span was dramatically shortened (1.8 hours) and platelet production rate considerably increased (about 18xnormal). Neither corticosteroid therapy nor splenectomy alleviated the thrombocytopenia. Extremely large platelets, with diameters of up to 10 mum, were seen in the peripheral blood. The mean platelet diameter and percentage of megathrombocytes reached peak values about 2 weeks after splenectomy. It is suggested that the immunologic background of the rapid thrombocytolysis is similar to that which governs platelet destruction in idiopathic thrombocytopenic purpura.
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PMID:Myelofibrosis and rapid thrombocytolysis. A case report. 84 65

The splenic red cell volume has been measured directly by an isotope method with quantitative scanning in 10 patients with leukaemic reticuloendotheliosis (hairy cell leukaemia). The volume ranged between 211 and 726 ml (mean 410 ml, SD 158) and this constituted 15--48% (mean 28.1%, SD 9.5) of the total circulating red cell volume. This is an exceptionally large pool when compared with that found in myeloproliferative and lymphoproliferative disorders with the same degree of splenomegaly. It is consistent with the histological features which show marked red cell accumulation in the splenic cord areas. The red cell pooling in the spleen thus appears to be a significant factor in the anaemia and there was fairly good correlation between the percentage of improvement in the anaemia and the percentage of red cell volume contained in the spleen. By direct measurement of the splenic red cell pool, it is possible to predict the extent to which splenectomy will benefit the anaemia and this may also provide an indirect measure of the extent of bone marrow dysfunction in the causation of the anaemia.
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PMID:Splenic red cell pooling in hairy cell leukaemia. 87 2

Sixteen subjects, with sickle-cell anaemia, all Iranians (ages 3 to 56 years), with very mild symptomatology are reported. Some of the subjects had been totally asymptomatic. Splenomegaly was noted in 9 cases. There was an increase in the mean level of fetal haemoglobin (18%); this is the probable explanation for the mild phenotype. In 29 subjects with sickle-cell trait, the level of HbF was also significantly raised as compared with normal (1-6% vs. 0-6%). The mechanism of increased synthesis of HbF is unknown. The findings are similar to those reported in the Shiite Moslems of Saudi Arabia suggesting that in these populations there is a genetically-determined ability to produce high levels of Hb F in the presence of the sickle-cell gene.
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PMID:Mild sickle-cell anaemia in Iran associated with high levels of fetal haemoglobin. 88 5

A screening survey for abnormal hemoglobins at a hospital in Mizunami city, Gifu prefecture, Japan detected a fast-moving variant of hemoglobin in a family of Japanese origin. The abnormal hemoglobin constitutes about 45 percent of the total hemoglobin from the propositus and another carrier in the family, but neither of these persons had anemia, jaundice, cyanosis or splenomegaly. Structural analysis of this hemoglobin revealed that the amino acid substitution was at residue 83 in the beta chain, where a glycine was replaced by an aspartic acid. This hemoglobin variant has been previously reported in a Greek child (hemoglobin Pyrgos) (1). Oxygen affinity of hemoglobin Pyrgos was found to be normal.
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PMID:Hemoglobin Pyrgos (beta83 Gly replaced by Asp) in a Japanese family. 89 27

A four-year old boy with familial erythrophagocytic lymphohistiocytosis is reported. The clinical picture was that of prolonged fever, hepato and splenomegaly, adenopathies and, in the terminal state, bleeding tendency and sligth jaundice. The laboratory data showed anemia, leucopenia and abnormal coagulation studies compatibles with disseminated intravascular clotting. The differential diagnosis with malignant histiocytosis is attempted from a histological point of view.
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PMID:[Familial erythrophagocytic lymphohistiocytosis (author's transl)]. 90 35

1. Equine infectious anemia (EIA) is an immunologically-medicated disease. Immune complexes formed in blood and tissues are responsible for most symptoms and lesions (anemia, splenomegaly, lymphadenopathy, glomerulonephritis, etc.). In addition, a state of cellular hypersensitivity of the delayed type is involved in the pathogenesis. 2. Periodical attacks of pyrexia and clinical illness in the presence of immunity are caused by antigenically-modified variants of virus. By means of immunosuppressive treatments similar relapses of fever associated with the appearance of new virus variants can be also provoked during longlasting asymptomatic periods. 3. The mechanisms responsible for the lifelong persistence of virus are not fully elucidated. Obviously of prime importance is the viral antigenic drift allowing the virus to escape from humoral and cellular immune reaction. Finally, however, a state of cell-mediated immunity ensuring protection against homologous and heterologous virus strains may be reached. 4. Pathogenetic analogies and differences existing between EIA and other chronic viral infections of animals are recorded.
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PMID:[Pathogenesis of equine infectious anemia (with reference to similar chronic viral infection)]. 91 50

Evaluation of the histologic changes of the bone marrow and clinical data of twelve male and two female patients with hairy cell leukamia before treatment. Mean age of the patients was 46 years, time from the onset of symptoms 5.5 months. 13 of the patients were anaemic, showing splenomegaly, 12 suffered from thrombopenia, 9 from granulocytopenia, and 8 from hepatomegaly. In all of the cases, the relative numbers of lymphocytes in the blood had been increased together with various amounts of characteristic hairy cells. All of the cases had a typical histologic picture of lymphocytic bone marrow infiltration, mostly of the diffuse type. In 50% of the cases not only the well known rod-like intracellular inclusions could be seen, but also ring-shaped figures whose significance is discussed. Decrease of the granulopoiesis, disintergration of the marrow sinusoids, and osteoporosis are the most important additional signs. The progression of the disease is marked by increasing bone marrow infiltration, by splenohepatomegaly, anaemia, thrombopenia, and increasing numbers of typical lymphocytes in the blood. The bone marrow being considered to be the origin of the disease for good reasons, the histobiopsy of this organ ranks among the diagnostic and prognostic measures to be taken at first sight.
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PMID:[Bone marrow diagnostic in hairy cell leukaemia (author's transl)]. 92 7

Eperythrozoon coccoides and Haemobartonella muris produced in mice and rats respectively, essentially the same disease, characterized by anaemia, splenomegaly and in severe cases, haemoglobinuria with death. In both infections anaemia was associated with phagocytosis of erythrocytes by monocytes of the spleen, and with the presence of cold-active haema-glutinin for trypsinized red cells (CAH). An antigen similar to the serum antigen (SA) associated with acute malaria and babesiosis was also found in the blood of the anaemic animals. One or two days later antibody to SA (ABSA) was detected and for several days thereafter, both SA and ABSA could be detected in plasma samples. Anaemia crisis with haemoglobinuria was better correlated with the appearance of ABSA than with the presence of CAH. It is suggested that CAH, and complexes of SA and ABSA could have acted as anaemia factors and were in part causal in the sequestration or haemolysis of erythrocytes during acute infection.
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PMID:Autoimmune factors associated with anaemia in acute Haemobartonella and Eperythrozoon infections of rodents. 94 46


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