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)

This report concerns a 6-year-old child with severe dyseythropoietic anaemia and splenomegaly, apparently present since the first months of life. Striking anisopolikilocytosis was observed in the blood smear. The bone marrow showed marked erythroblastic hyperplasia with dyserythropoiesis. Ineffective erythropoiesis was demonstrated by ferrokinetic studies. Ultrastructurally erythroblasts appeared grossly abnormal. The clinical course was progressively worsening, necessitating repeated transfusions. The patient's father, also affected by a chronic anaemia of moderate degree since childhood, had a peripheral picture of anisopoikilocytosis, a shortened life span of the erythrocyte and in his bone marrow an erythroblastic hyperplasia with many atypical erythroblasts. His condition deteriorated because of persistent jaundice, biliary cholelithiasis, fibrosis and haemosiderosis of the liver. The clinical course, the pattern of the genetical transmission, the peculiar features of the erythroblasts disclosed by light and by electron microscope studies suggest that these cases represent a new type of congenital dyserythropoietic anaemia.
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PMID:A new type of congenital dyserythropoietic anaemia. 69 27

Eighty-eight patients with Hemoglobin (Hb) H diagnosed in our hospital in the past decade were reviewed. Among them, 37 were males and 51 were females, and their age ranged from 10 to 77 years. In physical examination, 43.3% of them showed to have jaundice, 47% had splenomegaly, 14% had hepatomegaly and 19.3% had gall stone. Hb electrophoresis revealed the presence of Hb H (1.4-40%), Hb F (23/88, range: 0.5-7.5%) and decreased Hb A2 (mean = 1.23 +/- 1.2%). The study of iron status showed increased serum ferritin concentration (mean = 421.4 +/- 343.7 ug/dl) and transferrin saturation ratio (53.9% +/- 20.5%). Hemosiderosis was found in three out of four patients received liver biopsy. Although most patients did not receive blood transfusion frequently, iron overload was not uncommon in the patients with Hb H disease. Further study would be needed to explore the true relationship between iron overload and Hb H disease.
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PMID:Hemoglobin H disease--ten years' experience. 216 37

Pathologic changes produced after 4 weeks of infection by Babesia microti in Syrian hamsters are described and compared to babesiosis of humans. Following intraperitoneal inoculation, both intravascular and extravascular hemolysis developed. Up to 70% of red blood cells were parasitized. The principal morphologic abnormalities were an increase in extramedullary hematopoiesis and hyperplasia of the mononuclear phagocytic cells of the red pulp manifested grossly as splenomegaly, marked renal tubular hemosiderosis and hypertrophy of Kupffer cells. The disease was not fatal to any hamsters during the 4 week study. The clinical signs and lesions were less severe than fatal babesiosis of asplenic humans and similar to severe, but nonfatal disease in spleen intact humans. The hamster may serve as an animal model for the studying the pathophysiology of human babesiosis and for studying potential chemotherapeutic agents.
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PMID:Pathology of experimental Babesia microti infection in the Syrian hamster. 369 1

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

One splenectomized and 6 intact coyotes (Canis latrans), and 2 coydogs were experimentally inoculated with a recent isolate of Babesia gibsoni. The disease was mild in intact animals, was fatal in the splenectomized coyote, and was characterized by a regenerative hemolytic anemia with the PCV decreasing to 16% in intact animals and to 6% in the splenectomized coyote. Peak parasitemia ranged from 3% to 21% of erythrocytes infected and was inversely correlated to PCV. Serum lactate dehydrogenase, bilirubin, and globulin concentrations were increased in all infected animals. Three weeks after inoculation, specific antibody titers increased to 1:65,536 and remained elevated in the chronically infected animals. The splenectomized coyote had progressive weakness until death, 24 days after inoculation. Intact animals had splenomegaly and anorexia at the height of infection. The splenectomized coyote had generalized edema, omental petechiae, renal and hepatic degeneration, membrano-proliferative glomerulonephritis and congestion, extramedullary hematopoiesis, lymphoid hyperplasia, and severe hemosiderosis in an accessory spleen. The only consistent change in the intact animals was splenomegaly.
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PMID:Experimental babesiosis in coyotes and coydogs. 397 Apr 35

Enlarged spleen, fever, increased susceptibility to infections, and thrombocytosis, are manifestations of iron deficiency which are relatively specific of pediatric patients. Iron deficiency anemia is part of everyday pediatrics. Patients are referred to the hematologist in the following situations: 1) Therapy is ineffective for one of the following reasons: the hypochromic anemia is not caused by iron deficiency (hemoglobinopathies); iron is less efficiently used because of transferrin deficiency or infectious, inflammatory or cancerous disease; iron therapy is inadequate either because of insufficient dosage or of suboptimal duration. 2) A relapse occurs in spite of adequate therapy. Before investigating the digestive tract, abnormal hemostasis. Osler-Weber-Rendu syndrome and pulmonary hemosiderosis should be considered. 3) Iron deficiency anemia is less common in adolescents. This condition, known as chlorosis, results mainly from increased needs, unbalanced diet, and onset of menses. In some cases no explanation is found but iron therapy leads to recovery. 4) Difficult problems arise in patients with complex anemias: iron deficiency with folic acid or vitamin B12 deficiency; hyposideremia complicating one of the hemoglobinopathies.
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PMID:[Iron-deficiency anemia. Hematologist's viewpoint]. 629 49

In experimental infection of chickens with a virulent strain of Borrelia anserina, peak spirochaetaemia was recorded from 72 to 96 hours. Progressive enlargement of the spleen with mottling was the predominant gross finding. Spleen, liver and small intestine showed extensive erythrophagocytosis, which continued even after the disappearance of spirochaetes from blood and tissues. While haemosiderosis was mild in the lungs, it was absent from the heart, kidney and brain. Spirochaetes were demonstrable in the spleen, liver, intestine, kidney and to a lesser extent in the lungs, but absent from the heart and brain. Widespread erythrophagocytosis and extravascular haemolysis suggest involvement of an immune mechanism in the pathogenesis of splenomegaly.
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PMID:Observations on the pathology of experimental avian spirochaetosis. 663 39

A 43-year-old man with lifelong anaemia showed features which indicate him to have a previously undescribed variant of congenital dyserythropoietic anaemia (CDA), type II. The main clinical features--of which the first two are unique or very unusual in CDA--have been severe tophaceous gout, massive splenomegaly, gall stones mecessitating cholecystectomy and haemosiderosis affecting the liver and probably the heart. At age 41 he sustained a spontaneous retinal detachment. In the peripheral blood there were large numbers of nucleated red blood cells and marked macrocytosis; otherwise the picture was typical of CDA type II. The bone marrow contained many bi- and multi-nucleated erythrocyte precursors. There were increased levels of a number of red cell enzymes and a slightly raised level of HbF. Uncharacteristically, the red cells failed to lyse with acidified normal serum. The cells were strongly agglutinated by anti-i and were of the rare Kpb-negative phenotype. Plasma lipid analysis showed very low levels of cholesterol and vitamin E. Lipid peroxidation was markedly increased. Ultrastructural studies showed reduplication of the erythrocyte, granulocyte, and platelet cell membranes.
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PMID:Congenital dyserythropoietic anaemia (CDA) with severe gout, rare Kell phenotype and erythrocyte, granulocyte and platelet membrane reduplication: a new variant of CDA type II. 737 99

A case report of a 58-year-old patient with hereditary spherocytosis and large paravertebral masses in the thorax and abdomen is presented MRI detects the origin of the masses with typical signal intensities of the masses and the bone marrow in T1 and T2 weighted sequences as extramedullary hematopoiesis. In this special case there is bleeding into these masses and, as often, liver hemosiderosis and splenomegaly.
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PMID:[Nuclear spin tomographic findings in compensated chronic hemolysis. A case report of a hereditary spherocytosis]. 836 56

Fatal disseminated toxoplasmosis was diagnosed in a Risso's dolphin (Grampus griseus) dam and its fetus on the basis of pathologic findings, immunohistochemistry, and structure of the parasite. The dolphin was stranded alive on the Spanish Mediterranean coast and died a few hours later. At necropsy the dam was in good condition. From the standpoint of pathology, however, it had generalized lymphadenomegaly and splenomegaly, enlargement of and multifocal hemorrhage in the adrenal glands, diffuse mucosal hemorrhage of the glandular and pyloric stomach, ulcerative glossitis and stomatitis, focal erosions and reddening of the laryngeal appendix, and severe paraotic sinusitis with intralesional nematodes Crassicauda grampicola. The dolphin was pregnant, most probably in the first gestational trimester. The most prominent microscopic lesions were multifocal granulomatous encephalomyelitis, diffuse subacute interstitial pneumonia, mild multifocal necrotizing hepatitis and nonsuppurative cholangiohepatitis, gastritis and adrenalitis, mild lymphoid depletion, medullary sinus and follicular histyocitosis, and systemic hemosiderosis. The fetus had foci of coagulative and lytic necrosis in the kidneys, the lung, and the heart. Most lesions were associated with tachyzoites and tissue cysts of Toxoplasma gondii. The diagnosis was confirmed immunohistochemically. This is the first report on toxoplasmosis in a Risso's dolphin (G. griseus) and on transplacental transmission to an early-stage fetus in any cetaceans.
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PMID:Disseminated toxoplasmosis in a Mediterranean pregnant Risso's dolphin (Grampus griseus) with transplacental fetal infection. 1243 53


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