Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

By the mid 1990s the number of newly diagnosed children with HIV infections in the US may rival the 6,500 children diagnosed with cancer each year. But recent developments in therapy for the child with AIDS offer some hope. A pediatric trial at the NCI used a continuous infusion of azidothymidine in order to achieve the desired blood and cerebrospinal fluid levels. Objective and subjective evidence of response to therapy was noted in all patients who presented with neurodevelopmental deficit. Increases in appetite and weight gain and reductions in lymphadenopathy and hepatosplenomegaly, and increases in CD4 count similar to those observed in adults were apparent. Promising preliminary results have also been seen in trials of dideoxycytidine, dideoxyinosine, and recombinant CD4.
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PMID:Pediatric AIDS: a perspective for the oncologist. 214 35

A 56-year-old man was admitted to Sapporo Kitano Hospital on May 30, 1987 because of fever, retention of ascites and pleural effusion, generalized lymphnode swelling and hepatosplenomegaly. Laboratory findings showed Coombs' positive hemolytic anemia, leukocytosis and polyclonal hypergammaglobulinemia. Serological test included positive RA factor, anti-DNA 16 U/ml, thyroglobulin Ab 1600 x and microsome Ab 3200 x. A cervical lymphnode specimen exhibited typical histologic picture of IBL like T cell lymphoma. The surface markers of lymphoma cells were CD2(+), CD8(+) and CD4(-). Chromosomal analysis of these cells revealed polyploidy abnormality for all chromosomes except for No. 14's which are disomy. All lymphoma cells have XXY sex chromosome and mar1, mar2, mar3. Gene rearrangement for beta-chain of T cell receptor was proved in these lymphoma cells. He was treated with prednisolone, vincristine, cyclophosphamide, adriamycin etc, but died of respiratory failure 171 days after admission. We reported a rare case of IBL like T cell lymphoma with polyploidy chromosomal abnormality accompanied with hemolytic anemia.
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PMID:[IBL like T cell lymphoma associated with hemolytic anemia and polyploidy chromosomal abnormality--a case report]. 215 94

Omenn's syndrome is a rare autosomal recessive disease characterized by the onset, from the first weeks of life, of an exsudative skin rash, alopecia, hepatosplenomegaly, diffuse lymph-nodes, diarrhea and increased susceptibility to infections with hypereosinophilia. The associated severe combined immunodeficiency (SCID) differs from the other SCID by the existence of lymphocytosis. The number of T lymphocytes is normal or elevated; they are sometimes immature; the distribution of their subsets (CD4/CD8) is variable. The B lymphocytes are quantitatively and functionally deficient. Pathological investigations reveal major lymphoid depletion with severe thymic hypoplasia associated with a proliferation of cells which have the whole immunohistochemical characteristics of Langerhans' cells but do not contain their specific granulations (Birbeck's granulations) on the ultrastructural examination. These cells are also found in the skin, lungs and liver. The outcome is usually fatal before 1 year of age. The pathogenesis of this disease is still discussed: it might be the result, in an immunodeficient child, either of a graft-versus-host reaction after materno-fetal transfusion of immunocompetent cells, or of an abnormal immunologic reaction after antigenic stimulation, or a deficit of molecules in lymphocyte ecotaxy.
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PMID:[Omenn syndrome]. 216 40

An important disease entity distinct from cutaneous T-cell lymphoma (CTCL) in Japan is adult T-cell leukemia/lymphoma (ATL), which usually shows the same phenotype as CTCL, i.e., a helper/inducer T-cell phenotype (CD4+CD8-), and usually involves the skin. Clinically, both CTCL and ATL are heterogeneous in nature. In this study, we demonstrated differences between CTCL and ATL in terms of clinical and immunopathologic cell surface features. In patients with ATL, the predominant clinical findings were peripheral lymph node involvement, skin lesions, hepatosplenomegaly, leukemic manifestations, and an aggressive course. In patients with CTCL, by contrast, only skin lesions predominated at the onset of the disease and a relatively good prognosis was demonstrated. Phenotypic heterogeneity of ATL in the skin, i.e., CD4-CD8-, CD4+CD8-, and CD4-CD8+, was demonstrated. Expression of Leu8, CD7 (Leu9), and CD45RA (2H4) was high in both the skin-infiltrating ATL cells and peripheral blood and lymph node ATL cells compared with that in the skin-infiltrating CTCL cells. Expression of CD25 (IL-2R), CD71 (OKT9), HLA-DR, and HLA-DQ was higher in the skin-infiltrating ATL cells than in CTCL cells. Expression of CD29 (4B4) was high, and that of CD45RA (2H4) was low in both the skin-infiltrating ATL and CTCL cells compared with the peripheral blood and lymph node ATL cells. Expression of CD45RO (UCHL-1) was not significantly high in the skin-infiltrating CTCL cells compared with that in ATL cells. The most significant phenotypic difference between ATL cells and CTCL cells was the expression of Leu8 (lymph node homing receptor), CD7 and CD25 antigens on the cell surface, and the main phenotypic difference between skin-infiltrating ATL and CTCL cells and peripheral blood and lymph node ATL cells was the expression of CD29 and CD45RA. These findings confirm that the difference in antigen expression on the cell surface might reflect the clinical features of ATL and CTCL, and suggest that the predominant phenotype of peripheral blood and lymph node ATL cells is that of naive, relatively immature or activated T-cells, and that CTCL cells are previously activated (memory) T-cells. In other words, CTCL cells do not share the same origin as ATL cells. These observations support the concept that ATL is a disease distinct from CTCL.
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PMID:Comparative study of cutaneous T-cell lymphoma and adult T-cell leukemia/lymphoma. Clinical, histopathologic, and immunohistochemical analyses. 224 93

A 28-year-old male was admitted to our hospital because of hepatosplenomegaly and granular lymphocytosis. His peripheral leukocyte count was 3,000/microliters with 43% of granular lymphocytes (GL). These GLs were immunologically phenotyped as CD2+CD3-CD4-CD8-CD16+CD56+HLA-DR+ and were found that TcR genes coding beta and gamma chains were not rearranged. Chromosomal analysis of his GLs stimulated with IL-2 showed 47 XY, +8. This patient was diagnosed as a granular lymphocyte leukemia of natural killer cell type. Blood chemistry showed elevation of serum GOT, GPT and LDH values. The fever persisted until administration of prednisolone was initiated. But 40 days after, high fever appeared again and the liver and spleen were extremely enlarged. Combined chemotherapy was then started but resulted in no effects. He died of hepatic failure on the 77th day from admission. 47 XY, +8, that has been reported in acute non-lymphocytic leukemia and myelodysplastic syndrome, may be related to the pathogenesis in some cases of granular lymphocyte leukemia.
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PMID:[Granular lymphocyte leukemia of natural killer cell type; association with 47 XY, +8 by interleukin 2 (IL-2)-stimulated chromosomal analysis]. 225 62

A case of large granular lymphocyte (LGL) leukemia with ascites and CNS involvement was reported. A 39-year-old Japanese female was admitted to our hospital in March, 1987 because of high fever. Her clinical and hematological features were characterized by generalized lymphadenopathy, marked hepatosplenomegaly, high serum LDH level (3,257 mU/ml), marked leukocytosis (71,000/microliters) with 74% LGLs and bone marrow infiltration with 57% LGLs. Despite of chemotherapy, ascites, retroperitoneal mass and CNS involvement developed and she died of sepsis after three months. LGLs from the patient's blood, marrow and ascites, stained positively for acid phosphatase. These LGLs were E rossete+ and Fc (IgG) receptor+ and were positive for CD2, OKM1, HLA-DR and Leu11, but were negative for CD1, CD3, CD4, CD8 and Leu7 as well as for terminal deoxynucleotidyl transferase activity. The natural killer activity against K562 target cells was high and was significantly augmented after stimulation by recombinant human interleukin 2. These LGLs also demonstrated normal antibody-dependent cytotoxicity activity. Cytogenetic study on bone marrow cells and ascitic cells revealed clonal chromosomal abnormalities. These clinical, hematological, immunological and cytogenetic findings suggest that this patient had a neoplastic proliferation of natural killer cells.
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PMID:[CD3-, OKM1+, Leu7-, Leu11+ large granular lymphocyte leukemia with ascites and CNS involvement]. 247 53

A 53-year-old woman born in Kamo village of Shizuoka prefecture was admitted to Juntendo Izunagaoka hospital complaining cough and appetite loss. On physical examinations, general lymphadenopathy and hepatosplenomegaly were recognized. And also dry rales and wet rales were heard in the bilateral lungs. On hematological examinations, leukocytes has counted 74,900/microliters, of which 61% atypical lymphocytes, and there were HTLV-I infection and positive anti-ATLA antibody. By the above results, she was diagnosed as adult T cell leukemia (ATL). T cell subset analysis was (CD4-, CD8-, CD3-), which is rare in ATL. Three months after the admission, she was died of disturbances of respiratory function in spite of VEPA therapy. Surface marker changed from CD3- to CD3+ in the course. To illuminate this mechanism will be a key step for the future study.
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PMID:[Adult T cell leukemia with CD4- and CD8-]. 252 5

The clinical, morphologic, immunologic, functional, serologic and cytogenetic features of 4 cases of chronic granular T cell lymphocytosis with neutropenia were studied. The records of these patients were reviewed and an infectious event preceding the development of the disorder could be documented in 3 cases, suggesting a cause and effect relationship. A benign monoclonal gammopathy was detected in the remaining patient. The clinical picture was characterized by moderate blood and bone marrow lymphocytosis, neutropenia, hepatosplenomegaly, absence of lymphadenopathy, and a stable course, observed over a period of up to 11 years. Surface marker analysis in all the patients showed the common membrane phenotype of granular T cell lymphocytosis (CD3+, CD4-, CD8+, Leu7+). One patient treated with steroid therapy had reversal of lymphocytosis and severe neutropenia, which both recurred after steroids were withdrawn. The disorder again resolved after pulse steroid treatment. From these findings and a review of the literature, we suggest that granular T cell lymphocytosis with the immunologic phenotype exemplified by our cases is a distinct reactive or immunoregulatory disorder. In view of the similarities in character to benign monoclonal B cell lymphocytosis and idiopathic paraproteinaemia, we suggest that this disorder be termed chronic granular T cell lymphocytosis of undetermined significance.
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PMID:[Chronic granular T lymphocytosis of undetermined significance]. 266 61

An unusual case of an aggressive leukemia of natural killer (NK) cells occurred in a 65-year-old male. Clinical characteristics of this case included hepatosplenomegaly, ascites, marrow infiltrate with leukemic cells, and a WBC up to 82.8 X 10(9) before therapy. One year before his presentation he had been noted to have a WBC of 12.1 X 10(9) with 78% lymphocytes, and 6 months before had noted intermittent fever and weight loss. He and his brother had well documented hereditary cold urticaria. The patient was treated with a modification of ProMACE CYTABOM regimen and had prompt regression of the leukemia with associated acute tumor lysis. Renal, hepatic, and marrow failure predominated during a terminal course that ended 22 days after therapy was commenced, and at autopsy there was no evidence for leukemic cell infiltrate in the liver, spleen or marrow. The leukemic cells were large granular lymphocytes by light and electron microscopic criteria, and had the following immunophenotype: CD2+, DR+, Leu7+, NKH1+, CD11+, CD3-, CD5-, CD4-, CD8-, CD16-. The cells displayed high antibody-dependent cell-mediated cytotoxicity (ADCC) and NK activity, and had a high rate of spontaneous proliferation in vitro that was not augmented by phytohemagglutinin (PHA), concanavalin A (Con A), or pokeweed mitogen (PWM). Southern analysis of DNA from leukemic cells revealed normal germline arrangements for the beta and gamma chains of the T cell antigen receptor and immunoglobulin heavy chain genes. The majority of metaphases were clonally abnormal revealing consistent rearrangements involving extra material attached to the long arms of chromosomes 5 and 11.
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PMID:Leukemia of non-T lineage natural killer cells. 284 89

The leukemic cells of adult T-cell leukemia (ATL) usually express the helper/inducer associated antigen reactive with anti-CD4 antibodies but not with anti-CD8. We present a 63-yr-old woman with ATL characterized by circulating leukemic cells with CD4+/CD8- phenotype, hepatosplenomegaly with no lymphadenopathy, and the presence of proviral DNA of human T-cell leukemia virus I in the leukemic cells. She was successfully treated with interferon beta and the remission lasted for 12 months. She then relapsed in the lymph nodes with minimal peripheral blood involvement. The neoplastic cells of the lymph node now co-expressed CD4 and CD8 antigens indicating that the change in clinical manifestation was accompanied by a phenotypic change of the leukemic cells.
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PMID:A case of CD4+/CD8- adult T-cell leukemia with good response to interferon-beta terminating as a CD4+/CD8+ adult T-cell lymphoma. 288 3


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