Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

600 children born to HIV-infected mothers by June 15, 1990, in ten European centres were followed to study the natural history of HIV infection and the vertical transmission rate. They were seen at birth, every 3 months up to 18 months of age, and every 6 months thereafter. At last follow-up, 64 children were judged to be HIV infected and 343 had lost antibody and were presumed uninfected. The initial clinical feature in infected children was usually a combination of persistent lymphadenopathy, splenomegaly, and hepatomegaly, though 30% of children presented with AIDS, or with oral candidosis followed rapidly by AIDS. An estimated 83% of infected children show laboratory or clinical features of HIV infection by 6 months of age. By 12 months, 26% have AIDS and 17% die of HIV-related disease. Subsequently, the disease progresses more slowly and most children remain stable or even improve during the second year. The vertical transmission rate, based on results in 372 children born at least 18 months before the analysis, was 12.9% (95% Cl 9.5-16.3%). Virus has been repeatedly isolated in an additional small proportion of children (2.5%, 95% Cl 0.7-6.3%) who lost maternal antibody and have remained clinically and immunologically normal. Without a definitive virological diagnosis, the monitoring of immunoglobulins, CD4/CD8 ratio, and clinical signs could identify HIV infection in 48% of infected children by 6 months, with a specificity of more than 99%.
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PMID:Children born to women with HIV-1 infection: natural history and risk of transmission. European Collaborative Study. 167 Nov 9

Two hundred fifty-three children with newly diagnosed T-cell acute lymphoblastic leukemia (ALL), who were treated uniformly with modified LSA2L2 therapy, were evaluated using univariate and recursive partition analyses to define clinical or biologic features associated with risk of treatment failure. Overall event-free survival (EFS) at 4 years was 43% (SE = 4%). Factors examined included white blood cell (WBC) level, age, gender, race (black v other), presence of a mediastinal mass, hepatomegaly, splenomegaly, marked lymphadenopathy, hemoglobin level, platelet count, blast cell expression of antigens such as the common acute lymphoblastic leukemia antigen (CALLA, CD10), HLA-DR, and T-cell-associated antigens (CD3, CD4, CD8, CD7, CD5, and THY). Univariate analysis showed that age less than or equal to 5 or less than or equal to 7 years, WBC level less than 10, less than 25, less than 50 or less than 100 x 10(3)/microL, and blast cell expression of CD4, CD8, or CALLA were associated with significantly better EFS, while hepatomegaly and splenomegaly were associated with worse EFS. Recursive partitioning analysis showed that the most important single favorable prognostic factor was a WBC level less than 50 x 10(3)/microL and, for patients with WBC counts below this level, the most important predictor of EFS was blast cell expression of the pan-T antigen defined by the monoclonal antibody (MoAb), L17F12 (CD5). For patients with higher WBC levels, the most important predictor of EFS was blast cell expression of THY antigen. The recursive partitioning analysis defined three groups of patients with widely varied prognoses identified as follows: (1) those with a WBC count less than 50 x 10(3)/microL who lacked massive splenomegaly and had blasts expressing CD5 had the best prognosis (66%, SE = 7%, EFS 4 years, n = 84); (2) those with (b1) WBC counts less than 50 x 10(3)/microL with either massive splenomegaly or who had blasts lacking CD5 expression, or (b2) WBC counts greater than 50 x 10(3)/microL with expression of the THY antigen had an intermediate prognosis (39%, SE = 7% EFS at 4 years, n = 94); (3) those with WBC counts greater than 50 x 10(3)/microL and whose blasts lacked expression of THY antigen had the poorest outcome (EFS = 19% at 4 years, SE = 8%, n = 63). A three-way comparison of EFS according to these groupings showed significant differences among the three patient groups (P less than .001). The recursive partitioning was able to classify 241 (95%) of the patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Prognostic factors in childhood T-cell acute lymphoblastic leukemia: a Pediatric Oncology Group study. 168 95

We describe the clinical and laboratory findings of 78 adult patients with T-prolymphocytic leukemia (T-PLL) studied over the last 12 years. The main disease features were splenomegaly (73%), lymphadenopathy (53%), hepatomegaly (40%), skin lesions (27%), and a high leukocyte count (greater than 100 x 10(9)/L in 75%) with nucleolated prolymphocytes. A variant form with small, less typical cells was recognized in 19%. Membrane markers defined a postthymic phenotype TdT-, CD2+, CD3+, CD5+, CD7+; in 65%, the cells were CD4+ CD8-, in 21%, they coexpressed CD4 and CD8, and, in 13%, they were CD4- CD8+. Serology for human T-cell leukemia/lymphoma virus Type-I (HTLV-I) was negative in the 27 cases investigated. Cytogenetic analysis in 30 cases showed a consistent abnormality of chromosome 14, usually inv (14), with breakpoints at q11 and q32 in 76% of cases. Trisomy 8, including iso8q, was shown in 53%; t (11;14)(q13;q32) was documented in one case; and one had a normal karyotype. The clinical course was progressive with a median survival of 7.5 months. Thirty-one patients were treated with 2' deoxycoformycin and 15 responded (3 complete remissions and 12 partial remissions); the response rate (48%) increased to 58% in patients with a CD4+ CD8- phenotype. The median survival of responders was 16 months and of nonresponders 10 months; other treatments were less effective. T-PLL is a distinct clinico-pathologic entity with aggressive course and characteristic chromosome abnormalities. A subgroup of patients may benefit from deoxycoformycin.
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PMID:Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. 174 86

Twenty-eight Zairean patients with Schistosoma mansoni infection were investigated and treated with praziquantel. Of these, 22 were re-examined 18 months later and 13 were found to be re-infected. Eighteen uninfected Zaireans were monitored concurrently to control for variations unrelated to schistosomiasis. Pathophysiological changes related to liver fibrosis were assessed by the determination of serum cholylglycine and procollagen-III-peptide. Circulating T-cell subsets were quantitated, and shedded T-cell antigens were measured in sera. In patients initially presenting with hepatomegaly, the biochemical indicators for egg-induced immunopathology became normal after therapy and remained normal even after re-infection, when the parasite load attained about 50% of the pretreatment level. Among T-cell phenotypes, CD4+ cells transiently increased by three months after treatment, but after 18 months the CD4/CD8 ratios both in patients then re-infected and in those not re-infected had reverted to the respective balances which had been observed at the start of the investigation. Both soluble CD8 antigen and interleukin 2 receptor in patients' sera were significantly elevated throughout the study period. The results indicate a dissociation of factors regulating fibrogenesis and immunomodulation after treatment and re-infected.
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PMID:Re-infection in human schistosomiasis mansoni: a prospective field study 18 months after praziquantel therapy. 212 97

An 18 years old female was admitted to hospital due to pancytopenia on May 25, 1987 and found to have petechiae, mild hepatomegaly and severe splenomegaly. The bone marrow was found to contain 12% of blast cells. Splenectomy was performed followed by CHOP therapy. In September, 1987 the peripheral blood was found to contain tumor cells, which turned out to be resistant to various combination chemotherapies. The patient died on August 21, 1988. The phenotype of tumor cells in this case was CD2+ CD7+ CD3+ CD4- CD8- WT31-. Genetic analysis detected rearrangement of the beta and gamma chain of TcR but not transcription or translation of the beta chain of TcR, while the antibodies of delta TCS 1 and TcR delta 1 to the delta chain of TcR were positive. From this fact, the present case was considered to be the malignant counterpart of normal CD3+ WT31- double negative T cells. The reactivity of this tumor cells to IL-2 and IL-1 beta suggested the association of the IL-2R beta chain.
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PMID:[T gamma lymphoma with CD3+ CD4- CD8- WT31- and TcR gamma delta]. 213 75

A case of granular lymphocyte proliferative disorder with squamous cell carcinoma of penis is described. A 77-year-old Japanese male was admitted to our hospital in March, 1989 because of ulceration of penis and lymphocytosis. He had hepatomegaly and lymphadenopathy but not splenomegaly on physical examination. The WBC count was 10800/microliters with 83% of granular lymphocytes. Thrombocytopenia (32000/microliters) and mild anemia were also demonstrated. Bone marrow aspirate showed hypercellular marrow with 84% of granular lymphocytes. Proliferative granular lymphocytes were CD2+ CD3+ CD4- CD8- CDw29+ and exhibited ADCC activity but not NK activity. On March 15, amputation of penis was performed. But his lymphocytosis didn't change and thrombocytopenia increased. Thus we considered his granular lymphocytosis was neoplastic rather than reactive. Our case seemed to be rare and was compared with previous reports.
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PMID:[Granular lymphocyte proliferative disorder with penis cancer]. 217

Peripheral blood T cell phenotypes, CD3-induced mitogenesis and soluble IL 2 receptor and CD8 in sera were studied in intestinal and hepatosplenic Schistosomiasis mansoni before and three to six months after therapy with praziquantel. Fifteen pairs matched for intensity of infection were analyzed and compared with local, non-infected age-matched controls. CD3+ cell counts were lower in untreated hepatosplenic schistosomiasis (median 1040 cells/microliters; 95% confidence interval 608-1269) compared to controls (1534; 1264-1620). This difference was largely accounted for by immature CD1+/CD3-cells circulating in these patients (median 388/microliters, 252-474). The frequency of CD1+ T cells in circulation decreased drastically after chemotherapy. Similar, but less marked, alterations were seen in intestinal schistosomiasis. Lymphocyte proliferation initiated by agonistic anti-CD3 monoclonal antibody was severely impaired in hepatosplenic patients, who had suffered haemorrhagic complications, but not in the cases of incipient hepatomegaly. Soluble CD8 antigen circulated in increased amounts in hepatosplenic schistosomiasis. Remarkably, a negative correlation between CD3-induced mitogenesis and circulating levels of CD8 was noted in these patients. Whereas CD3-induced mitogenesis in hepatosplenic schistosomiasis normalized after therapy, circulating IL 2R and CD8 antigen in hepatosplenic patients still exceeded control levels. The results demonstrate disturbances of CD3 and CD8 expression and/or T cell maturation in hepatosplenic schistosomiasis. Imbalanced CD4/CD8 ratios and an increased IL 2R/CD8 turnover may reflect an inhibitory circuit within the T cell compartment.
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PMID:T cell phenotype alterations in hepatosplenic schistosomiasis mansoni normalize after chemotherapy. 251 52

Spontaneous improvement of active juvenile rheumatoid arthritis (JRA) occurred after T lymphocytosis in an 8-year-old boy. He had prominent lymphocytosis, the count reaching 59,000/mm3, followed by spontaneous disappearance of fever, arthralgia, lymphadenopathy, hepatomegaly, and C-reactive protein. The serum immunoglobulin levels were gradually decreased. The surface marker analysis, using two color flow cytometry, showed that the lymphocytes were activated suppressor T lymphocytes, expressing CD3, CD8, HLA-DR, and CD8 plus CD11. When studied in vitro with pokeweed mitogen stimulation, the T lymphocytes significantly suppressed the immunoglobulin production by autologous B lymphocytes as compared with the T lymphocytes at remission (p less than 0.01). Based on the widely believed notion that depression of suppressor T lymphocyte functions is one of the important mechanisms underlying systemic JRA, the activated T lymphocytosis with the suppressor phenotype and suppressive function on the immunoglobulin production may have been related to the improvement of active JRA in the patient.
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PMID:Spontaneous improvement of juvenile rheumatoid arthritis after T lymphocytosis with suppressor phenotype and function. 297 87

We report a case of an erythrophagocytic T-cell lymphoma with autoimmune hemolytic anemia, osteolytic lesions, hypercalcemia, hepatomegaly, and marrow invasion without evident lymph node, skin or peripheral blood involvement. The malignant cells were large immunoblastic cells with pleomorphic nuclei and occasional phagocytic vacuoles containing erythrocytes. Immunologic studies showed the tumor cells to have the phenotype of activated T-cells of the cytotoxic/suppressor subclass (HLA- Dr-positive, CD8 positive) with surface receptors for IgG Fc but lacking functional activity in assays of natural killer activity and antibody-dependent cytotoxicity. The tumor cells appeared to express receptors for T-cell growth factor (Tac). Cytogenetic study of marrow mononuclear cells revealed complex but non-random karyotypic abnormalities (45 XX, +11, -12, -16, 17 p+). The clinical and laboratory features indicate that erythrophagocytic T-cell lymphoma represents a true clonal malignancy of the CD8-positive subclass of T-lymphocytes.
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PMID:Erythrophagocytic T-cell lymphoma: immunologic and cytogenetic evidence for a clonal malignancy of the CD8-positive T-cell subclass. 313 49

A 31-year-old renal transplant recipient developed an unusual T-cell lymphoproliferative disorder 3 years after transplantation. The neoplasm involved the spleen, without concomitant hepatomegaly, lymphadenopathy, or obvious bone marrow involvement. Peripheral blood involvement developed after splenectomy. Immunophenotypically, the neoplastic cells expressed CD2, CD3, CD7, CD16, CD45, CD56, and the gamma/delta T-cell receptor on the surface membrane. The neoplastic cells were negative for surface membrane CD4, CD5, and CD8. Serologic and/or DNA analyses for viruses, including Epstein-Barr virus, human T-cell lymphotropic virus-1, human immunodeficiency virus, and human herpesvirus-6, were negative. Cytogenetic findings included a translocation breakpoint at chromosome 7p15, consistent with involvement of the T-cell receptor gamma-chain locus. Although gamma/delta T-cell lymphomas have been reported to have a predilection for hepatosplenic localization, this is the first well-documented case to be described in the setting of posttransplantation immunosuppression.
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PMID:Gamma/delta T-cell posttransplantation lymphoproliferative disorder primarily in the spleen. 808 54


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