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)

Histomorphometry was performed on representative trephine biopsies of the bone marrow on admission of 50 patients (21 male, 29 female - age 67 years) with so-called primary osteomyelofibrosis/-sclerosis (OMF) not preceded by any other subtype of chronic myeloproliferative disorders. This study was firstly aimed at testing correlations between histological features (amount of haematopoiesis, cytological aspects of megakaryocytes, density of reticulin and collagen fibres and degree of osteosclerosis) and laboratory data, as well as spleen size and duration of relevant prediagnostic symptoms. Secondly, we concentrated on a discrimination of OMF patients into two subgroups according to bone marrow morphology and clinical variables. Statistical evaluation of histomorphometric variables and haematological findings disclosed that there was a progressive fibro-osteosclerotic process in the evolution of disease features. Increase in medullary fibrosis was significantly paralleled by an abnormal or pleomorphic megakaryopoiesis in the bone marrow: there was an increase in irregularity of perimeters for megakaryocytes and naked nuclei combined with smaller sizes of these elements including the nuclei. Additionally, there was a greater number of pycnotic bare nuclei. A number of morphometric features (density of fibres, degree of osteosclerosis, amount of haematopoiesis) were associated with corresponding clinical data (spleen size, length of preclinical history). By consideration of a set of basic histomorphometric variables our cohort of 50 patients could be divided into an early hyperplastic subtype with no or minimal medullary reticulin and another group with conspicuous fibrotic and osteosclerotic alterations of the bone marrow. It was noticeable that we found no significant correlation between amount of haematopoiesis or marrow cellularity with splenomegaly. This result suggests that splenic haematopoiesis (myeloid metaplasia) may represent an autonomous or neoplastic process and not only compensation for a failing fibro-osteosclerotic bone marrow.
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PMID:Histomorphometry of bone marrow biopsies in primary osteomyelofibrosis/-sclerosis (agnogenic myeloid metaplasia)--correlations between clinical and morphological features. 250 23

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
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PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

A 68-year-old male who suffered from thrombocytopenia and mild splenomegaly for 18 years was found to present agranular gray platelets on peripheral blood smear. Bone biopsy revealed a mild, diffuse, reticular fibrosis with no collagen, and electron microscopy of the platelets showed an absence of almost all the alpha-granules. Platelet thrombospondin and fibronectin analysed by SDS-polyacrylamide gel electrophoresis and Rocket immunoelectrophoresis were absent. Follow-up of 4 years showed the same parameters with no evidence of active myeloproliferative or dysmyelopoietic disorders. Hemorrhagic diathesis was limited to ecchymoses and postprostatectomy bleeding, necessitating platelet transfusion. This led us to conclude that our patient probably had a constitutional primary alpha-granule deficiency or gray platelet syndrome. This extremely rare defect has been described in less than 10 patients, all of them very young. Our observation shows that these patients may have a long, uneventful survival.
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PMID:Gray platelet syndrome in the elderly. 341 74

In an adult man the spleen serves two physiologic functions. It is an organ of the immune system, the second function is the sequestration and removal of normal and abnormal blood cells. Most diseases of spleen are associated with enlargement of the organ. Causes of splenomegaly are liver diseases with portal hypertension, infections, malignant leukemias or lymphomas often combined with lymph node enlargement, collagen vascular diseases or Felty's syndrome, chronic hemolytic syndromes and infiltrative diseases such as Gaucher's disease. Some diseases associated with splenic enlargement respond to splenectomy.
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PMID:[Internal medicine diagnosis of splenic diseases]. 357 92

The anatomy of the splenic ligaments was examined in twenty cadavars. Histologic sections were taken from ten. In one of these twenty cases the entire ligamentous structure was studied by serial sections using a polarizing light microscope. There are four principal ligaments of the spleen: the gastrosplenic ligament, the colicosplenic ligament, the phrenocolic ligament and the phrenosplenic (splenorenal) ligament. There are two portions to the gastrosplenic ligament. In the upper portion where the spleen is very close to the stomach, the collagen fibers appear more dense. The term splenorenal ligament is vague. This is because there are three structures between the spleen and the kidney besides the peritoneal layer: collagenous tissue with fat (pararenal fat), the prerenal fascia and a fat capsule (perirenal fat). The vascular anatomy is of interest. Running through the gastrosplenic ligament are small branches from the short gastric and left gastroomental vessels. The colicosplenic ligament receives little branches from the left gastroomentalic vessels and the lower pole vessels. The other ligaments have mainly venous vascularisation which can develop into a collateral network within splenomegaly. The direction of the collagen fibers in the splenic ligaments is important. In all the ligaments most of the fibers come from two directions: laterocranial to mediocaudal and mediocranial to laterocaudal. Splenic capsular tears are a recognized complication of upper abdominal surgery. This is because traction in the mediocaudal direction during gastric and colon surgery and in the mediocranial direction during gastric surgery is oriented in the same direction as the collagen fibers in the splenic ligaments and thus tears the splenic capsule.
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PMID:[The ligament system of the spleen and its significance for surgical interventions]. 368 35

Hypersplenism induced by methylcellulose given to rats i.p. was characterized by splenomegaly, anaemia, leucocytopenia and thrombocytopenia. Hb, Hct and WBC count were normalized, and platelet count rose to supernormal values by splenectomy. At a standardized liver resection, the hypersplenic rats had an increased blood loss and prolonged bleeding time in comparison with control rats. Removal of the large spleen normalized these abnormalities. Hypersplenism also shortened APT time and impaired ADP and collagen-induced platelet aggregation, findings not normalized by splenectomy.
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PMID:Hypersplenism--effect on haemostasis. An experimental study in the rat. 396 Dec 74

This paper documents a case of vitamin A toxicity presenting with splenomegaly and ascites. The light microscopic, electron microscopic, and fluorescent findings are described in detail. The principal histopathologic finding was marked perisinusoidal fibrosis. The role of Ito cells in the storage of lipid-soluble vitamins and their subsequent transformation to fibroblasts producing collagen are discussed.
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PMID:Hepatic pathology in vitamin A toxicity. 646 34

The human spleen, an organ of unique anatomic and functional importance, is the largest component of the reticuloendothelial system, with direct interposition between systemic and portal circulation, and yet the morphologic correlates of its various functions remain somewhat mysterious. The contributions of transmission and scanning electron microscopy to the understanding of splenic structure have been considerable. They have helped clarify the three fundamental sites of structural alteration and specialization that are defined and discussed: 1) the white pulp with its two variable components--the lymphoid follicle and periarteriolar sheath--which, with the marginal zone of the red pulp, is the primary site of lymphoproliferative activity; 2) the cords of the red pulp, the functionally slow component of the splenic circulation, which sequester senescent or structurally altered red cells and effect their removal by means of scavenging macrophages (and which may be secondarily involved by the accumulation of platelets or certain types of leukemic cells, resulting in chronic cordal distention, or by the accumulation of collagen in fibrocongestive splenomegaly); and 3) the splenic sinuses, the unique structure of which determines that only healthy red cells with normally plastic and flexible membranes pass through to the venous circulation. Abnormal transiting cells such as sickle cells frequently clog the apertures to these sinuses. Direct arteriocapillary sinus terminations provide the anatomic basis for a fast component of the red pulp circulation, the existence of which was questioned for many years and the extent of which is still unknown in pathologic states.
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PMID:The spleen: a correlative overview of normal and pathologic anatomy. 707 17

We consistently observed small "reflective channels" (RCs) in the splenic parenchyma in patients with portal hypertension (PHT). The purpose of this study was to investigate the frequency of this sign in PHT and in splenic disorders unrelated to PHT compared to normal controls. The significance of this sign in the diagnosis of PHT and in differentiating PHT splenomegalies from others was also assessed. A total of 337 patients underwent sonographic examination of the spleen: 147 normal, 75 non-PHT splenic pathologies, and 115 with PHT. The RCs were scored from grade 0 to 3 by counting the number in an area of 12 mm2 in the splenic parenchyma. Of 222 normal and non-PHT spleens, 174 (78%) showed grade 0, 44 (20%) grade 1, four grade 2 (1%), and none showed grade 3 RCs. Of 115 PHT cases, 17 showed grade 0, 25 grade 1, 44 grade 2, and 29 showed grade 3 RCs. The sensitivity of this sign was 0.85 with a specificity of 0.77 and an accuracy of 0.80 in detecting PHT (p < 0.001). The interobserver and intraobserver variation for grading was insignificant (p > 0.1). The RCs could be explained by periarterial fibrosis and dilatation of venous sinuses with increased collagen in their walls, which is known to occur in PHT. The vascular nature on ultrasound (US) was confirmed by the presence of flow on color Doppler. This sign is readily differentiated from the calcifications of tuberculosis, histoplasmosis, sickle cell infarcts, and phleboliths; it serves as a useful aid in diagnosing and differentiating PHT splenomegaly from non-PHT splenomegaly.
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PMID:Multiple reflective channels in the spleen: a sonographic sign of portal hypertension. 795 Aug 27

Among the synthetic peptides derived from the 28-kDa Schistosoma mansoni glutathione S-transferase (Sm28GST), immunization with the C-terminal peptide comprising amino acid residues 190-211 induced a reduction in splenomegaly, in the number of hepatic eggs and in hepatic fibrosis in mice infected by Schistosoma mansoni. The absence of antibodies specific for the Sm28GST or for the 190-211 peptide observed in our conditions of immunization with this peptide argued in favour of the involvement of cellular-dependent mechanisms in the reduction in hepatic pathology. This was confirmed by the passive transfer of 190-211 peptide-specific T-cell enriched spleen cells which reproduced the protective effect conferred by immunization with the 190-211 peptide. These 190-211 peptide-specific cells produced little IL4 and high levels of IFN-gamma, a potent inhibitor of collagen synthesis. Furthermore, the use of a lipopeptidic form of the 190-211 peptide significantly improved the reduction in hepatic pathology obtained with the uncoupled peptide and induced a durable protective response. These results provide encouraging information for the possible use of synthetic peptides in the immunoprophylaxis of Schistosomiasis.
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PMID:Evaluation of the effect of Sm28GST-derived peptides in murine hepatosplenic schistosomiasis: interest of the lipopeptidic form of the C-terminal peptide. 796 86


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