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)

MRL/MpJ-lpr/lpr mice spontaneously develop a lupus-like autoimmune disorder characterized by massive proliferation of T cells and rapidly fatal immune complex glomerulonephritis. We evaluated the therapeutic effect of 5-azacytidine (5AC), a cytidine analogue known as an inhibitor of DNA methylation, in MRL/MpJ-lpr/lpr mice. Intraperitoneal injection of 5AC (50 micrograms, twice a week) starting from 6 weeks of age retarded the development of lymphadenopathy and autoimmune syndrome. Its beneficial effects included: (a) increased life-span, (b) diminution of lymphadenopathy and splenomegaly, (c) reduction in circulating levels of autoantibodies such as anti-DNA and rheumatoid factors, and (d) suppression of lupus glomerulonephritis. However, similar treatment in BALB/c mice did not affect the development of IgG anti-human IgG antibody responses. These results suggest that the protective effect of 5AC is related to the inhibition of the lpr gene-induced T cell proliferation, thereby suppressing the autoimmunity-accelerating effect mediated by the lpr gene.
Eur J Immunol 1990 Sep
PMID:5-Azacytidine inhibits the lpr gene-induced lymphadenopathy and acceleration of lupus-like syndrome in MRL/MpJ-lpr/lpr mice. 169 37

Conventional light and electron microscopic studies, together with cytochemical and immunocytochemical staining procedures, were carried out to ascertain whether the lymphomata of four elderly female patients living within 10 kilometers of each other, who presented within a short space of time with massive splenomegaly and varying cytopenia, belonged to any particular subgroup of lymphoma. In each case the lymphoma had a diffuse pattern and mature B cell phenotype. The malignant cells were of uniform cell type, slightly larger than admixed polymorphonuclear leucocytes, and showed minimal nuclear irregularity and positivity for tartrate resistant acid phosphatase (TRAP) staining. Their clinical and morphological features were compared with those of other lymphoproliferative disorders, but while sharing some features in common with each condition, this small group of patients seemed to have a unique combination of findings. The cytopenias of all four responded well after removal of the spleen and their disease has not been aggressive. It is concluded that these patients have a distinct subgroup of lymphoma, which it is important to recognise so that inappropriate use of aggressive cytotoxic drugs can be avoided.
J Clin Pathol 1990 Sep
PMID:Tartrate resistant acid phosphatase positive splenic lymphoma: a relatively benign condition occurring in a time-space cluster? 169 23

Haematological, clinical and some molecular genetic features of homozygous sickle cell (SS) disease in Saudi Arabia have been compared in 33 patients from the Eastern Province (Eastern) and 30 from the South Western Province (Western). Eastern patients all had the Asian beta globin haplotype whereas Western patients were more variable but predominantly of the Benin haplotype. Eastern patients had more deletional alpha thalassaemia, higher total haemoglobin and fetal haemoglobin levels, and lower HbA2, mean cell volume, reticulocytes, and platelet counts. Clinically, Eastern patients had a greater persistence of splenomegaly, a more normal body build and greater subscapular skin fold thickness, and Western patients had more dactylitis and acute chest syndrome. Painful crises and avascular necrosis of the femoral head were common and occurred equally in both groups. The disease in the Eastern province has many mild features consistent with the higher HbF levels and more frequent alpha thalassaemia but bone pathology (painful crises, avascular necrosis of the femoral head, osteomyelitis) remains common. The disease in the West is more severe consistent with the Benin haplotype suggesting an African origin.
Br J Haematol 1991 Sep
PMID:Two different forms of homozygous sickle cell disease occur in Saudi Arabia. 171 63

A 52-year-old woman presented slight fever, diffuse papular skin rash and painful cervical lymph node swelling. Her lymph node swelling generally up to 3 cm in diameter, with petechiae on the lower legs and hepato-splenomegaly within a few weeks. ESR was 45 mm/h, Hb 10.0 g/dl, RBC 345 x 10(4)/microliter, WBC 22,600/microliter (atypical lymphocyte 47%), PLT 1.0 x 10(4)/microliter, GPT 91 U/L, gamma-globulin 34.3%, EBV-VCA x 2,560, EBNA x 20, and anti-rubella antibody x 512. The biopsied cervical lymph node showed histologic features of effacement of nodal architecture by an exuberant vascular proliferation accompanied with infiltration of the immunoblasts, and was diagnosed as immunoblastic lymphadenopathy (IBL)-type lymphadenopathy. The pulse therapy of methylprednisolone and high dose of gamma-globulin improved lymphadenopathy, thrombocytopenia and anemia. IBL-type lymphadenopathy after infection of rubella virus may be different from true IBL, but is important to discuss the pathogenesis of IBL.
Rinsho Ketsueki 1991 Sep
PMID:[IBL-type lymphadenopathy after infection of rubella virus]. 171 58

We report our experience with high dose intravenous immunoglobulin (IVIg) in 3 thalassemic patients who had evidence of possible immune hemolysis. In 2 patients who had serious sepsis, their responses to IVIg were only partial and transient. The other patient who had marked splenomegaly had no evidence of response to IVIg. Both serious infections and large spleen may hamper the effect of IVIg and should be considered before IVIg is to be used in thalassemia.
Southeast Asian J Trop Med Public Health 1991 Sep
PMID:High-dose intravenous immunoglobulin in the management of immune hemolysis in patients with thalassemic disease: factors which determine refractoriness. 172 38

After the intraperitoneal injection of corpuscles of C. burnetii antigen (Ag), phospholipid (PL), and sediment obtained after the extraction of PL from Ag with chloroform-methanol (CM) slight leukocytic reaction developed in the peritoneum on day 1, and on day 2 it could be observed in the liver and in the spleen. Ag induced the most pronounced morphological changes. In the spleen they were manifested by the activation of T- and B-dependent zones of white pulp from day 2 and by the pronounced hyperplasia of reticular cells and macrophages, leading to splenomegaly, by days 7-14. Simultaneously lymphoid-macrophagal granulomas and hepatomegaly developed in the liver. By days 7-14 the foci of necrosis in the liver were caused by the thrombosis of portal veins and were not registered after the injection of PL and CM (and earlier also in experiments with Ag in doses of 0.1-0.3 mg).
Zh Mikrobiol Epidemiol Immunobiol 1991 Sep
PMID:[The host reaction to the administration of different components of Coxiella burnetii]. 175 30

Nine consecutive patients with HCL seen over a period of five years were reviewed. Male: Female ratio was 8:1. Median age at diagnosis was 49 years. Weakness and fatigue (66%) were the commonest presenting symptoms and splenomegaly (66%) was the commonest physical findings. Varying degrees of pancytopenia was the consistent feature in majority of cases. Diagnosis was made on the basis of bone marrow biopsy and characteristic EM picture. Forty-four percent of cases developed serious infection during their clinical course. Gram negative bacilli and fungi were the most frequently isolated organisms. Major sites of infections were pneumonia and septicemia. Splenectomy was carried out in four cases. Rapid recovery of haematological parameters without any significant complication was observed in all these cases. Two patients were treated with alfa-interferon. In both the cases recovery of haematological parameters was slow compared to those under going splenectomy. One patient treated with alfa-interferon died due to infection related complications while the other went into remission.
Indian J Cancer 1991 Sep
PMID:Hairy cell leukaemia. A review of nine cases. 178 82

Adult-onset Still's disease is characterized by high spiking fever, evanescent maculopapular rash and arthritis. Several recent reports have associated its development with a variety of infectious agents. We describe the case of a 25-year old woman presenting high fever, maculopapular rash and seronegative polyarthritis associated with lymphoadenopathy, splenomegaly and neutrophil leucocytosis together with acute acquired toxoplasmosis. Other causes of systemic illness were excluded by appropriate laboratory, radiological and histological investigations. Clinical, radiological and laboratory findings as well as possible etiopathogenetic correlations among both pathological conditions are discussed. Toxoplasma gondii should be considered as a further possible triggering agent associated with the development of adult-onset Still's disease.
Clin Rheumatol 1991 Sep
PMID:Adult-onset Still's disease associated to toxoplasma gondii infection. 179 Jun 46

Treating MRL/1pr mice, which spontaneously develop systemic lupus erythematosus and rheumatoid arthritis, with 15-DOS resulted in a decrease in the amount of autoantibodies and inhibited proteinuria of the developing glomerulonephritis with an improved survival rate of these autoimmune mice. 15-DOS treatment also lowered the percentage of animals with swollen lymph nodes and inhibited the development of splenomegaly. In the established disease 15-DOS returned urine-protein values and renal function (serum urea and creatinine) to normal levels. Circulating rheumatoid factor and autoantibodies to double-stranded DNA were reduced and the increase in paw volume (signs of a polyarthritis) was inhibited.
Agents Actions 1991 Sep
PMID:15-Deoxyspergualin (15-DOS) has a curative effect on the development of SLE-like autoimmune disease in MRL/1 mice. 179 21

Polyarthritis can be induced in rats using a synthetic adjuvant, N,N-dioctyldecyl-N', N-bis(2-hydroxyethyl) propanediamine (LA) suspended in oil. The disease is morphologically indistinguishable from the classic adjuvant arthritis induced by Freund's complete adjuvant (FCA). LA injection (7.5 mg/animal) consistently induced paw swelling, splenomegaly and fibrinogen level increases at certain time points. Studies evaluating various protocols and parameters determined that a 15 day assay where agents administered from days 9 through 13, would differentiate immunomodulatory and anti-inflammatory compounds. Parameters utilized were body weight, paw volumes, spleen weights, and fibrinogen levels. Immunomodulatory agents reduce paw swelling, splenomegaly and in some cases fibrinogen levels. NSAIDS reduce paw swelling, increase splenomegaly and have no effect on fibrinogen levels. These results indicate that compounds active in the traditional FCA assay can be detected and differentiated with respect to anti-inflammatory vs. immunomodulatory activity in a rapid screen.
Agents Actions 1991 Sep
PMID:Development of a rapid screen for detecting and differentiating immunomodulatory vs. anti-inflammatory compounds in rats. 179 40


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