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)

Reversal of myelofibrosis and splenomegaly is described in a 41 year old woman with metastatic breast cancer. After intensive chemotherapy and hormonal therapy, the tumor regressed, the splenomegaly receded, the hemogram showed no abnormalities, and the dense collagen and reticulin fibers in the marrow disappeared. The severe thrombocytopenia and leukoerythroblastosis noted before therapy were not obstacles to clinical management. In our report we document that myelofibrosis associated with breast cancer is not an ominous sign. Patients may benefit from an intensive, but well titrated, therapeutic program.
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PMID:Reversal of myelofibrosis in advanced breast cancer. 62 31

The second documented case of prolidase deficiency is presented. Clinical manifestations include chronic otitis media and sinusitis, dermatitis, and splenomegaly. Prolidase is undetectable in the white blood cells of the patient and near or less than the lower range of normal in each parent. The peptide chromatographic pattern of the urine is similar to that of the previously reported patient with prolidase deficiency. The quantity of amino acids excreted in urine per 24 hours is at least three times that of the upper range of normal (of these same amino acids) for the patients age group. More than 80% of the total amino acids excreted are in peptide form. The proline-to-hydroxyproline ratio suggests that the dipeptides are the catabolic products of other proteins in addition to collagen.
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PMID:Prolidase deficiency: report of a second case with quantitation of the excessively excreted amino acids. 87 81

In the course of the Trypanosoma equiperdum-infection of mice an increase of IgM antibodies against the autoantigens dsDNA, keratin and collagen as well as against a protozoan foreign antigen consisting of Sarcocystis gigantea-extract could be observed with a maximum level between 4th and 8th day p. i. The IgG-antibodies did not significantly change during the investigation time. A splenomegaly appeared after the infection. The weight of spleen was four times higher than normal. It was suggested that splenomegaly as well as induction of antibodies against several autoantigens and a foreign antigen were due to a polyclonal activation of lymphocytes.
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PMID:[Autoimmune phenomena during Trypanosoma equiperdum inoculation of the laboratory mouse]. 149 80

Sarcoidosis is a granulomatous multisystemic disorder, of unknown origin, that commonly affects young adults between 20-40 years of age. The disease usually manifests itself with changes in the chest which are radiologically visible in 90% of the patients in the form of bilateral hilar lymphadenopathy to interstitial infiltrates in the lungs and bronchi. Once the diagnosis of sarcoidosis has been established, the next step is to evaluate the activity and stadium of the disease. These activity markers include clinical, biochemical and immunological parameters. The clinical criteria include symptoms which indicate the clinical manifestation of sarcoidosis, as well as symptoms which are of prognostic importance for the further course of the disease: dry cough, dyspnea, erythema nodosum, posterior uveitis, polyarthralgia, myopathy, cardiac, renal or nervous system involvement, lymphadenopathy, skin lesions, splenomegaly, enlarged parotid and lacrimal glands, changes in chest x-ray and changes in pulmonary function tests. Biological criteria: biochemical markers in serum which are related to: macrophage and epithelioid cell activity, to lymphocyte activity, to granuloma activity and to collagen metabolism alterations; isotopic markers--67 gallium scan and cellular and soluble components in bronchoalveolar lavage fluid (BAL).
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PMID:[Sarcoidosis activity markers]. 180 91

Ultrasonography was used to evaluate spleen size in patients with various clinical conditions including those of the liver, blood, collagen, or autoimmune disease. To express spleen size, a spleen index (SI), the product of the transverse diameter and its perpendicular diameter measured on the maximum cross-sectional image of the spleen, was used. SI correlated well with the volumes of resected spleens. Splenomegaly was present in high percentages of patients with liver, blood, collagen, and autoimmune disease, even though a majority of these spleens were not large enough to palpate. By grading the SI, characteristic distributions of SI were obtained for patients with different types of diseases. Obtaining and grading the SI by the use of ultrasound appears to be a significant supplemental aid for evaluating spleen size, especially in patients whose spleens are not palpable.
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PMID:Sonographic assessment and grading of spleen size. 184 75

Hematologic abnormalities are common in association with collagen diseases, specially Systemic Lupus Erythematosus and include anemia, neutropenia, thrombocytopenia with alterations in lymphocyte subpopulations. On the other hand, patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis or primary myelofibrosis) have clinical and laboratory evidence of immunologic dysfunction. Clinical findings include the presence of arthritis, vasculitis and erythema nodosum. Laboratory abnormalities include the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs test, elevated latex fixation and a circulating lupus type anticoagulant. Total hemolytic complement markedly depressed has also been reported. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis. A review of the literature revealed that myelofibrosis occurring in the setting of collagen diseases is rare. However, a role for immunologic factors in the pathogenesis of myelofibrosis is also supported by the patients with coincident well defined collagen disease and myelofibrosis. In this report, we present two patients with such an association. Case 1 was a 58-year-old male with a two year duration history of rheumatic arthritis. He had bone erosions on hands, splenomegaly and myelofibrosis. Rheumatoid factor (latex) was positive: 1:2560. He had positive LE cells and hypocomplementemia: 37 CH50/ml (NV 70-150). The patient did not meet criteria for SLE. Case 2 was a 36-year-old female admitted because of dyspnea and fever. Diagnosis of myeloid metaplasia with myelofibrosis and progressive systemic sclerosis had been made four years before hand.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Coexistence of myelofibrosis and collagen diseases]. 213 Feb 12

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.
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PMID:A case of idiopathic portal hypertension after renal transplantation. 222 56

During a period of 19 months, 22 organ preserving splenic operations were performed. Eleven of these, were severe third degree traumatic bursting ruptures, which were treated using a resorbable compressive mesh. An intralienal pancreatic cyst, a case of splenomegaly suspicious for lymphoma, one traumatic and two accidental polar lesions were treated by partial splenic resection using stapler. In two patients with traumatic and four with accidental first and second degree lienal lesions, collagen tampon and fibrin adhesive were employed. None of these patients required a second laparotomy. Aside from an edematous tail pancreatitis, no complications arose. Two patients succumbed to their severe cranial injuries. In the same period, four posttraumatic splenectomies were performed. Thus, excluding incidental and technical splenectomies, the application of the described methods resulted in orthotopic splenic preservation in 78% of traumatic splenic ruptures.
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PMID:[Clinical use of new organ-saving technics in splenic surgery]. 231 69

Liver fibrogenesis involves the synthesis of collagen fibrils and proteoglycans by various types of liver cells, including Ito cells, transitional cells, myofibroblasts and hepatocytes. Synthesis of collagen fibrils follows a complex metabolic pathway with intermediate products such as type III procollagen (III-PC). Serum levels of III-PC may reflect the activity of the fibrogenetic process. We analysed the relationship between the serum levels of III-PC (N-terminal peptide) and diverse clinical, biochemical and histological parameters of 77 alcoholic patients (27 cirrhotics), comparing them with those of 15 age- and sex-matched controls. A highly significant difference was obtained between controls and patients (P less than 0.0001), but no differences were observed between cirrhotics and non-cirrhotics. Serum III-PC significantly correlated with clinical and biochemical data of liver function derangement (prothrombin activity, serum albumin, bilirubin, gynecomastia, ascites, encephalopathy, edema, splenomegaly); with the duration of ethanol addiction and with MCV. Sixty patients were followed up for a period ranging between 3 and 1056 days (mean = 356 days); 9 of them died. Patients with III-PC levels above 38 ng/ml had a significantly higher mortality (P = 0.006) than those with levels under 38 (log rank test). Thus, serum III-PC may be a useful tool in the clinical evaluation and prognostic assessment of patients with chronic alcoholic liver disease.
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PMID:Clinical and prognostic value of serum procollagen levels in chronic alcoholic liver disease. 232 14

Although collagen myelofibrosis indicates poor prognosis in late stages of chronic myelogenous leukemia, the significance of reticulin stain-measured fibrosis in newly diagnosed patients is unknown. One hundred and thirty-eight patients with untreated or minimally treated chronic phase Philadelphia chromosome-positive chronic myelogenous leukemia had reticulin stain studies made on their bone marrows at diagnosis. Reticulin fibrosis was graded on a scale of 1 to 4. Significant (Grade 3 or 4) fibrosis was noted in 65 patients (47%). Compared with patients with mild (Grade 1 to 2) reticulin fibrosis, those with significant fibrosis had a higher incidence of splenomegaly greater than or equal to 10 cm (29% versus 49%; P = 0.02), hemoglobin less than 10 g/dl (19% versus 49%; P less than 0.01), weight loss greater than or equal to 6.75 kg (10% versus 30%; P = 0.11), marrow blasts greater than or equal to 5% (7% versus 28%; P less than 0.01), peripheral blasts greater than or equal to 3% (30% versus 46%; P = 0.09), and additional karyotypic abnormalities (1% versus 17%; P less than 0.01). The incidence of thrombocytosis was similar in the two groups. Prognostically, median survival was significantly shorter for the 26 patients with Grade 4, compared with the 39 patients with Grade 3, and the 73 patients with Grade 1 or 2 reticulin fibrosis (32 versus 49 versus 57 months; P = 0.03). Reticulin fibrosis is a useful biologic and prognostic index in newly diagnosed patients with chronic phase chronic myelogenous leukemia.
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PMID:The relevance of reticulin stain-measured fibrosis at diagnosis in chronic myelogenous leukemia. 243 99


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