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)

We report a series of seven patients with reactive hemophagocytic syndrome, which was quite characteristic of its etiological spectrum. Infections were the leading cause, among them a case associated with HIV and another one with Salmonella enteritidis (a hitherto unreported association). The clinical findings consisted of fever, hepatomegaly, splenomegaly, lymphadenopathy, rash and pancytopenia. The diagnosis was carried out by bone marrow aspiration-biopsy except in two patients who were diagnosed at autopsy. The difficulty of the differentiation from malignant histiocytosis is discussed: one case of hemophagocytic syndrome due to diphenylhydantoin toxicity (the second reported one in the literature) was histologically undistinguishable from it. We think that, in any etiology, hemophagocytic syndrome is a reactive syndrome with variable intensity. The need for extensive microbiological investigation even in cases of histiocytosis of neoplastic appearance is emphasized.
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PMID:[Reactive hemophagocytic syndrome: analysis of a series of 7 cases]. 232 64

We prospectively studied 339 patients diagnosed of brucellosis over a six year period in order to evaluate the clinical and serological characteristics of brucellosis in the elderly. 319 patients (94.1%) were under 65 years of age (group A), and 20 patients (5.9%) were older than 65 (group B). No patient in group B developed splenomegaly as opposed to 69 (21.6%) in group A (p less than 0.05). The percentage of positive blood cultures (Brucella melitensis isolated in all cases) was 72.1% in group A and 60% in group B. The mean titer of IgM antibodies measured by indirect immunofluorescence test was significantly lower in the elderly patients, with no other differences in serologic response between the two groups. 103 patients (32.2%) in group A and seven patients (35%) in group B developed some complications; spondylitis was more common and severe among group B patients. There was no therapeutic failure or relapse among patients over 65. We conclude that clinical, bacteriological and serological characteristics and the outcome of brucellosis in the elderly are similar to those seen in younger patients.
Infection
PMID:Serology, clinical manifestations and treatment of brucellosis in different age groups. 236 66

We have analysed 139 consecutive cases (71 males and 68 females) of nutritional megaloblastic anaemia over a period of four and a half years. The majority of these patients belonged to the low socio-economic class and many of them were strict vegetarians. Sixty one percent were in the second and third decades of life. At the time of presentation, 46% had mild hepatomegaly, 42% fever, 34% mild splenomegaly and 20% bleeding manifestations. Of 102 cases in whom the biochemical parameters were available, vitamin B12 deficiency was detected in 76%, folate deficiency in 6.8%, combined B12 and folate deficiency in 8.8%; the remaining 7.8% had normal vitamin levels at presentation. All 139 patients had severe anaemia, 80.5% had thrombocytopenia and 43.8% had neutropenia as well as thrombocytopenia. It appears that during progression (in terms of duration) of megaloblastosis, anaemia is followed by thrombocytopenia and then neutropenia. Infection and bleeding in these patients may be aggravated by impaired functions of neutrophils and platelets, respectively.
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PMID:Pancytopenia in nutritional megaloblastic anaemia. A study from north-west India. 263 48

Since 1985 organ donors are routinely tested for the presence of HIV-antibodies, but prior to that time several patients acquired HIV-infection from grafts. In May 1984 a 65-year-old woman on hemodialysis received a cadaver kidney graft from a young iv drug addict. The transplant functioned perfectly with cyclosporin A immunosuppression. Retrospectively, 22 days after surgery HIV antigen was detected. At this time only a faint band of anti-p24 antibodies was found in the Western blot. Two years after surgery splenomegaly was found in the apparently healthy patient. During the third year thrombocytes fell and she developed lymphadenopathy and constitutional symptoms. Up to this time the immunological parameters were in the range of 10 healthy renal transplant patients with cyclosporin A treatment. In the 4th year T-lymphocytes dropped to values below 200 and the patient developed Pneumocystis carinii pneumonia. A few months later a pulmonary node, which later proved to be a B-cell lymphoma, appeared. Slightly less than 5 years after transplantation the patient died from clinically diagnosed pulmonary embolism. The progression of the HIV-Infection in this patient and in one of 18 patients in published reports show that the incubation period is several years shorter in renal transplant patients than in those who acquire HIV from blood products.
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PMID:[HIV infection caused by kidney transplant: case report and review of 18 published cases]. 267 39

The hematopoietic disregulation in adult mice induced by the malignant histiocytosis sarcoma virus (MHSV) and the Harvey murine sarcoma virus (Ha-MuSV), which both possess c-Ha-ras-related oncogenic sequences, was investigated. Spleen focus formation induced by MHSV and Ha-MuSV was not restricted by the Fv-2 resistance locus in congenic DDD and C57BL/6 mice, unlike leukemogenesis induced by Friend virus, Rauscher virus, and the myeloproliferative sarcoma virus (MPSV). C57BL/6 mice were much more resistant to MHSV and Ha-MuSV-induced spleen focus formation than DDD mice regardless of their Fv-2 state. Infection of DDD mice with MHSV caused a systemic histiocytic neoplasia, best described as murine malignant histiocytosis. Transformed histiocytic cells proliferated excessively in the bone marrow, spleen, and lymph nodes and, in the final stages of the disease, in all major parenchymal organs. The Ha-MuSV caused a strikingly different benign histiocytic tumor in DDD mice and, unlike MHSV, did not induce a rapid, progressive splenomegaly in C57BL/6 mice. Infection of DDD mice with MHSV induced a rapid and synchronized depletion of early and late erythroid precursor cell pools. In MHSV-infected C57BL/6 mice comparable changes were observed with dissimilar kinetics. Macrophage colony-forming cells of MHSV-infected mice were increased in number and proliferated independently of stimulating growth factors. The disease induced by MHSV in mice can thus serve as a model for malignant histiocytosis in humans.
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PMID:Murine retrovirus-induced malignant histiocytosis, an experimental model for the disease in humans. 282 12

Infection of 10-day-old chickens with an avian osteopetrosis virus resulted in a severe regenerative aplastic crisis. Hematopoietic and lymphopoietic tissues of chickens infected with myeloblastosis-associated virus (of subgroup B, inducing osteopetrosis, MAV-2(O] were analyzed for integrated and unintegrated viral DNA sequences, cell population shifts, weight changes, and morphological alterations. By 6 days postinfection (p.i.), DNA from bone marrow cells and peripheral blood leukocytes (PBL) contained between 0.50 and 0.70 copies of viral DNA per haploid genome. Erythrocytes and splenic leukocytes contained less than 0.10 copies/haploid genome. Granulocytes and precursor mesomyelocytes were absent from bone marrow, but numbers of erythrocytes, erythroblasts, and reticulocytes were normal. By 9 days p.i., bone marrow was severely hypoplastic and both granulopoietic and erythropoietic colonies were depleted. By 12 days p.i., erythrocytes and granulocytes were maximally depressed in peripheral blood and the amount of integrated virus in bone marrow and PBL decreased to less than 0.20 copies/haploid genome. In contrast, erythrocytes contained integrated viral DNA of up to 0.30 copies/haploid genome, indicating infection of erythrocyte precursors. At 18 days p.i., viral DNA was detected only in erythrocytes. Unintegrated viral DNA was not detected in any organs. Anemia was accompanied by splenomegaly and erythrophagocytosis. Viral DNA was never detected in thymus or bursa. Differential counting and flow cytometry of cells from bursa, thymus, and spleen, and of blood lymphocytes did not detect significant population shifts. These results suggest that MAV-2(O) infection of immunocompetent chickens occurs primarily in myelopoietic tissues, and tissues are selectively infected.
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PMID:Analysis of hematopoietic and lymphopoietic tissue during a regenerative aplastic crisis induced by avian retrovirus MAV-2(O). 283 18

Early reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 +/- 17,976 (S.D.) versus 1,250 +/- 1,500 factor units/year, (p less than 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.
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PMID:The spectrum of human immunodeficiency virus infection in patients with factor IX deficiency (Christmas disease) 303 83

Liver biopsy specimens previously taken from 16 haemophilic patients with chronic non-A, non-B hepatitis were reviewed. The degree of fibrosis correlated with serum procollagen III peptide (sPIIIP) concentrations, measured both at the time of biopsy and 4.25 years later. Two patients with extremely high sPIIIP concentrations had collateral veins on computed tomography, suggesting portal hypertension. Twenty eight of 47 patients (60%) had splenomegaly on computed tomography, and of 28 patients in whom intravenous contrast medium was used, seven (25%) had collateral oesophageal veins. Serum procollagen III peptide estimations and computed tomography, both non-invasive investigations, indicated that hepatic fibrosis and portal hypertension had developed in a proportion of haemophilic patients with non-A, non-B hepatitis. Infection with the human immunodeficiency virus (HIV) may modify the course of this presumably cytopathic virus infection of the liver.
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PMID:Non-invasive investigation of liver disease in haemophilic patients. 314 33

Cellular changes in the spleens of mice infected with Sarcocystis muris have been studied. Immunofluorescent staining for B and T cells and alpha-naphthyl acetate esterase (ANAE) staining for macrophages combined with histological studies revealed marked changes in the populations and distributions of all three cell types. Infection was accompanied by a marked splenomegaly, attributable mainly to widespread hyperplasia of the white pulp. Following infection there was an increase in the relative proportions of B cells (i.e. surface immunoglobulin+) and ANAE+ cells and a decrease in the proportion of T cells (i.e. Thy 1.2+). There was also a progressive accumulation of immunoglobulin-containing cells in the periarteriolar lymphocytic sheaths. Splenomegaly was most pronounced 20 days after infection. At this time there were 9.3 times as many B cells, 3.7 times as many T cells and 16.6 times as many ANAE+ cells as in uninfected mice.
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PMID:Cellular changes in the spleens of mice infected with Sarcocystis muris. 314 39

Microbiological features, diagnostic investigations, treatment, and complication rate in 53 cases of infective endocarditis were reviewed in this study. Infection occurred both on prosthetic (47%) and native valves (38%), while in 15% of the cases no prior valvular disease was known. Streptococcal (38%) and staphylococcal (30%) infections were predominant. In 17% of the cases apparent negative blood cultures were obtained. The most frequent portal of entry was dental infection or manipulation (45%), however in 28% of the patients etiology remained obscure. Major clinical signs and symptoms included heart murmurs (96%), fever (91%), dyspnoea (32%), and splenomegaly (30%). Echocardiography revealed vegetations in 78%, aortic and mitral valve being nearly equally affected. All patients were medically treated and 53% received antibiotics prior to blood cultures. Associations of ampicillin or penicillin with an aminoglycoside (43%) and penicillinase-resistant antibiotics (30%) were most frequently administered. In 28% of the patients, it was necessary to insert a prosthetic (aortic or mitral) valve. During follow-up, heart failure (28%), embolization (11%), and infections (11%) were the major complications.
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PMID:A six years review on 53 cases of infective endocarditis: clinical, microbiological and therapeutical features. 325 78


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