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)

Clonal gene rearrangements and aneuploidy are commonly recognized as characteristics of malignancy. We challenge this by an observation of a 3 1/2 year old boy with chronic benign lymphadenopathy, connatal splenomegaly and peripheral lymphocytosis. His brother and mother suffered from similar disease without progression. Cervical lymph node biopsy from our patient revealed a benign histology corresponding to the clinical course; however, clonal rearrangements of the JH and TCR gamma gene were demonstrated in lymph node cells and DNA aneuploidy in 11% of bone marrow cells. Immunohistology showed an inverse T-helper/T-suppressor cell ratio with polyclonal B cell increase and extensive lymphocyte activation. In situ hybridization demonstrated foci of lymphoid cells in the paracortical zone with expression of herpes virus type 6 genome. In addition, the patient, his brother and his mother showed serological evidence of active infection with the human herpes virus-6 (HHV-6). The association of HHV-6 infection, monoclonality and benign lymphoproliferation is discussed.
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PMID:Monoclonal B cell proliferation in lymphoproliferative disease associated with herpes virus type 6 infection. 256 Jun 19

A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.
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PMID:[Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus]. 259 83

A study was made of the effect of injection of cyclophosphamide (CP) immunomodulating to BALB/c mice, according to 3 schemes of injection with the whole dose being 200 mg/kg. In all, the state of peripheral blood and spleen, the cellular content of lymph nodes and the number of DNA-synthesizing cells in them were studied, in addition to the ability of lymph node lymphocytes to proliferative reaction on T and B cells mitogens in vivo. It was determined that a 2-fold injection of CP, in contrast to 10- and 5-fold injections, resulted in splenomegaly and leukocytosis in the experimental animals. By means of quantitative tests in vivo, in was demonstrated that the dynamics of restoration of proliferative responses to mitogens depends on the CP injection scheme. These tests proved to be more informative for registration of immunomodulating effect of CP, than the study of cellular content of lymph nodes and the number of DNA-synthesizing cells in them.
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PMID:[Immunomodulating effect of cyclophosphane under different modes of administration to mice]. 258 45

Early experiments had shown that splenopentin, the active part of the splenic hormone splenin, stimulates the differentiation of virgin B- and T-lymphocytes and significantly enhanced the reconstitution of immune reactions after immune suppression. The present study investigates the influence of splenopentin on the course of the graft-vs-host reaction (GVHR). The experimental model used were adult hybrid mice which received intravenously parental spleen cells. During the GVHR which has an chronical course in the strain combinations used a short stimulatory phase is followed by a long-lasting immunosuppression detected by antibody formation against sheep erythrocytes. Furthermore, the splenomegaly in the first weeks after spleen cell injection changed to a drastic decrease of the spleen weight up to strongly beyond normal values. Continuous treatment with splenopentin significantly prevented both symptoms of the GVHR: The suppression of the antibody formation was diminished widely, and no loss of spleen weight occurred. Furthermore, during the stimulatory phase anti-DNA-autoantibodies were produced in the untreated animals, while the splenopentin therapy prevented this reaction. During the further course of the experiment no increase of autoantibody production was detected later on.
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PMID:Prevention of graft-vs-host reaction induced immunodeficiency by treatment with splenopentin (DAc-SP5). 262 51

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

A colinear molecular clone of the Lilly-Steeves polycythemia strain of Friend spleen focus-forming virus (SFFV) was modified by inserting a 215-base-pair tag of simian virus 40 DNA into its nonfunctional pol gene region. The DNA was then transfected into psi-2 packaging cells, and helper-free tagged SFFV was recovered in the culture medium. Injection of this helper-free virus into NIH/Swiss mice caused transient mild splenomegaly and formation of spleen foci at 9 to 10 days. Although the vast majority of infected erythroblast clones then differentiated and died out, rare cell clones that were present in only 20 to 30% of the mice grew extensively by 26 to 33 days to form transplantable leukemias. The clonality of these leukemias was established by Southern blot analysis of their DNAs by using several restriction endonucleases and the simian virus 40 tag as a hybridization probe. All transplantable leukemias lacked helper virus contamination and contained a single tagged SFFV provirus that expressed the mitogenic env gene product gp55. The SFFV proviruses in these leukemias also appeared to be integrated into a few tightly clustered sites in the cellular genome. Although the tagged SFFV caused polycythemia during the polyclonal early stage of erythroblastosis, growth of the helper-free clonal erythroleukemias caused severe anemia. These results suggest that a single SFFV can cause mitosis of erythroblasts, and that cell immortalization also occurs when the provirus integrates into a critical site in the host genome. We propose that mice with clonal-stage leukemia become anemic because the immortalizing proviral integrations interfere with the cellular commitment to differentiate.
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PMID:A tagged helper-free Friend virus causes clonal erythroblast immortality by specific proviral integration in the cellular genome. 284 27

Nineteen children who presented with fever, hepato-splenomegaly, bone marrow and/or hepatic failure, and biopsy evidence of histiocytic proliferations were evaluated for lymphocyte dysfunction and evidence of prior viral infection. Seventeen of the children had erythrophagocytosis consistent with the previously described virus-associated hemophagocytosis syndrome (VAHS) or Familial erythrophagocytic lymphohistiocytosis (FEL). The other two had benign histiocytic proliferations in the central nervous system (CNS) with liver and bone marrow dysfunction. There were two sibling pairs and six patients with known disorders of immune deficiency. The remaining nine cases appeared to be sporadic and idiopathic. Epstein-Barr Virus (EBV) was identified in patients by serologic or DNA hybridization studies (15), EBV and cytomegalovirus (CMV) (1), adenovirus plus EBV and CMV (1), or adenovirus and EBV (1). Herpes zoster was associated with reactivation of symptoms in one patient. Immunologic impairment was evidenced by lymphopenia in 10 of 19 patients. More extensive evaluations could be done at diagnosis on only some of the children because the histiocytic proliferative syndrome was not recognized or because there were insufficient numbers of lymphocytes in samples obtained. For those who could be evaluated, the following immune deficiencies were found: decreased lymphocyte proliferation to mitogens (4 of 9), absent or markedly decreased natural killer function (5 of 5), and decreased cytotoxic lymphocyte reactivity to allogenic EBV-infected target cells (3 of 3). A new finding reported here is a higher than expected prevalence of HLA types A30, B8, and A1/B8 among the patients tested.
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PMID:Virus-associated histiocytic proliferations in children. Frequent association with Epstein-Barr virus and congenital or acquired immunodeficiencies. 284 31

Sixteen Italian patients with chronic T-cell lymphocytic leukaemia (T-CLL) and leukaemic T-helper phenotype lymphocytes (Thp-CLL) were investigated for serum antibodies against human T-cell leukaemia virus I (HTLV-I) or its integrated DNA sequences. Common features of this series of patients were an aggressive clinical course with poor response to treatment, high white blood-cell count, bone-marrow infiltration, splenomegaly, and chromosome abnormalities. Three patients had skin infiltration and one had hypercalcaemia. Immunological analysis showed a Thp (OKT4+) in all cases, and a heterogeneity, within the OKT4 population, of phenotypes and functional activities. Three patients had either HTLV-I integrated DNA sequences or anti-HTLV-I serum antibodies, or both. These patients had not received any blood transfusions, denied intimate contact with foreigners, and had always lived in small towns of central or southern Italy. Clinical and immunological findings in this series of patients suggest that both HTLV-I related and unrelated cases of Thp-CLL should be regarded as one disease arising from the same subpopulation of mature T-lymphocytes.
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PMID:T-helper phenotype chronic lymphocytic leukaemia and "adult T-cell leukaemia" in Italy. Endemic HTLV-I-related T-cell leukaemias in southern Europe. 286 33

2 cases of malignant T-lymphoproliferative disease are reported. The proliferating cell was a large blast expressing E and Fc gamma receptors but no helper or suppressor phenotypes and no SmIg. Skin infiltrates were the dominant clinical sign with conspicuous perivascular aggregations of T, E, Fc gamma lymphocytes, though both patients initially had disseminated disease with mild lymphadenopathy, splenomegaly and, in case 2, also hepatic infiltrations. Accordingly, DNA measurements on skin biopsies, taken early in the course, showed a dominating hypotetraploid clone (case 1) and a pronounced population in S-phase (case 2). The patients were alive for 6 and 2 yr, respectively, with a final fatal course of about 6 months duration involving a rather sudden progression of the skin infiltrates, increasing lymphadenopathy and splenomegaly, leukaemic transformation of the neoplastic T, E, Fc gamma lymphocyte and practically no response to cytostatic treatment.
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PMID:A transitional variant of a T-lymphoproliferative malignancy. Two cases of cutaneous T-cell lymphoma co-expressing E and Fc gamma receptors with final leukaemic transformation. 293 48

A case of T gamma lymphocytosis with neutropenia is presented. The patient showed mild lymphocytosis, splenomegaly, anemia, neutropenia and recurrent infections without progression for 15 years. The expanded lymphoid cells were morphologically large granular lymphocytes (LGL), had receptors for both sheep red blood cells and IgG-Fc portion and were positive for OKT3 and 8 antigens. They displayed ADCC activity, whereas they showed low responses to T-cell mitogens and deficient NK activity. They showed neither suppressor activity on antibody production by B-cells nor suppressor activity on CFU-C formation. The DNA isolated from the expanded cells of the patient showed T-cell beta-chain (T beta) gene rearrangement, indicating monoclonality of the proliferation. This finding supports that the proliferation of T8 lymphocytes in the present case is neoplastic rather than reactive, regardless of the benign clinical course.
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PMID:Immunological functions and T-cell receptor gene rearrangement of proliferating lymphocytes in a case of T gamma lymphocytosis with neutropenia. 295 88


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