UMLS:C0021051 - FACTA Search

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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adult T cell leukaemia-lymphoma (ATL) was first discovered and reported in Japan, where it has a high incidence in the south-west region. The first human retrovirus HTLV-I (human T-cell lymphotropic virus type I) is considered to be related to its aetiology. In ATL endemic areas, HTLV-I carriers form a fairly high percentage of the population, even among healthy individuals. ATL shows diverse clinical features. It can be divided into four subtypes: acute, chronic, smouldering and lymphoma type. ATL cells originate from the CD4-positive subset of peripheral T cells; they show a characteristic notch in the nucleus and a tendency to lobulation. ATL resists chemotherapy, and patients with acute and lymphoma types have a fairly poor prognosis. A definite diagnosis of ATL is made by documenting the presence of HTLV-I proviral DNA in the DNA of tumour cells. HTLV-I infection is caused by transmission of live lymphocytes via three routes (from mother to child, from males to females, and by transfusion). Familial occurrence of ATL is frequently seen. HTLV-I infection is seen in other countries, but its incidence is highest in Japan. Infection with HTLV-I is a direct cause of ATL. Furthermore, infection with this virus can indirectly cause many other diseases via the induction of immunodeficiency, such as chronic lung disease, opportunistic lung infection, cancer of other organs, monoclonal gammopathy, chronic renal failure, strongyloidiasis, non-specific dermatomycosis, HTLV-I-associated lymphadenitis, HTLV-I uveitis and HTLV-I-associated myelopathy-tropical spastic paraparesis (HAM/TSP).
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PMID:Adult T cell leukaemia-lymphoma. 803 96

A case of spindle-cell pseudotumor of the spleen due to nontuberculous mycobacteria in a patient with acquired immunodeficiency syndrome (AIDS) is described. The patient was a 55-year-old, human immunodeficiency virus-positive Haitian man who died of acute neurologic complications while on treatment for central nervous system toxoplasmosis. At autopsy, an enlarged multinodular spleen was noted. Histologic examination revealed coarse nodules of splenic parenchyma replaced by a dense spindle cell proliferation, admixed with scattered inflammatory cells. Immunostains showed strong cytoplasmic positivity of the spindle cells with MAC 387, HAM 56, and alpha-1-antichymotrypsin antibodies and negative staining for actin, vimentin, and S-100 protein antibodies. Ziehl-Neelsen stains revealed numerous elongated acid-fast bacilli within the cytoplasm of the cells that were occasionally lying free within the interstitium. The organisms also had a strongly positive reaction with antibodies to desmin intermediate filaments. Mycobacterial spindle-cell pseudotumor should be included in the differential diagnosis of conditions affecting the spleen in patients with AIDS.
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PMID:Mycobacterial spindle-cell pseudotumor of the spleen. 816 Jun 49

Detailed neuropathologic and immunohistologic analysis of a case of serologically and polymerase chain reaction-confirmed human immunodeficiency virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is reported in a 73-year-old North American black woman. In addition to the usual neuropathologic features of HAM/TSP, including tractal degeneration of the spinal cord, leptomeningeal and perivascular fibrosis, perivascular demyelination and chronic inflammation, neuroaxonal spheroids were prominent in the spinal cord. Neuroaxonal dystrophy was characterized by neurofilamentous masses that were immunoreactive for phosphorylated neurofilament epitopes, but not ubiquitin. Neuroimmunologic analysis of the inflammatory reaction revealed a prevalence of CD8+ T cells and class I major histocompatibility molecules (MHC) (HLA-ABC and beta 2-microglobulin), but very few CD4+ T cells. Microglia were highly reactive for class II MHC (HLA-DR alpha) and this was attributed to activation, rather than CD4 interaction, since CD4 presence was minimal. Inflammatory cytokine immunoreactivity was also detected in glia. It is concluded that the cumulative effects of cytotoxic T cell (CD8) infiltration and the possible involvement of cytokines were responsible for the unusual degree of neuroaxonal dystrophy and vascular fibrosis, as well as the observed demyelination in this case.
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PMID:Neuroaxonal dystrophy in HTLV-1-associated myelopathy/tropical spastic paraparesis: neuropathologic and neuroimmunologic correlations. 821 80

Human T-cell lymphoma/leukemia virus type I (HTLV-I) causes adult T-cell leukemia/lymphoma and HTLV-I-associated myelopathy. Specific regions within the outer envelope proteins of other retroviruses, e.g., human immunodeficiency virus type 1, are highly immunogenic and, because of the selective pressure of the host immune system, quite variable. Mutations in the external envelope protein gene of murine retroviruses and human immunodeficiency virus type 1 influence cellular tropism and disease pathogenesis. By contrast, no disease-specific viral mutations have been identified in HTLV-I-infected patients. However, all isolates studied thus far have originated from leukemic cell lines, peripheral blood mononuclear cells, or cerebrospinal fluid lymphocytes from patients with HTLV-I-associated myelopathy and adult T-cell leukemia/lymphoma and, therefore, may not truly reflect tissue-associated variation. The midregion of the HTLV-I gp46 external envelope glycoprotein (amino acids 190-209) induces an antibody response in 90% of infected individuals, and a hexapeptide in this region (amino acids 191-196) elicits antibodies in rabbits which inhibit syncytia formation and infection of target lymphocytes. Because of the above, we expected the neutralizing domain of the gp46 env gene of HTLV-I to possess disease or organ-associated mutations selected by the infected host's immune system. Hence, we amplified, cloned, and sequenced HTLV-I DNA directly from in vivo central nervous system, spleen, and kidney specimens, and a leukemic cell line from a patient (M. J.) with both HTLV-I-associated myelopathy and adult T-cell leukemia/lymphoma to discern the possibility of tissue- and/or disease-specific variants. In addition, we sequenced several HTLV-I isolates from different regions of the world, including Papua New Guinea, Bellona, and Liberia, and compared them to other previously published HTLV-I and related retroviral sequences. The 239-base pair sequence corresponding to amino acids 178 to 256 in gp46 displayed minor tissue-specific variation in clones derived from central nervous system tissues from patient M. J., but overall was highly conserved at both the DNA and amino acid levels. Variation was observed in this region among the other HTLV-I, simian T-cell lymphoma virus type I, and HTLV-II isolates in a pattern that was consistent with their known phylogenetic relationship. No consistent disease-related changes were observed.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Sequence analysis of an immunogenic and neutralizing domain of the human T-cell lymphoma/leukemia virus type I gp46 surface membrane protein among various primate T-cell lymphoma/leukemia virus isolates including those from a patient with both HTLV-I-associated myelopathy and adult T-cell leukemia. 826 24

A seroprevalence study for human T lymphotropic virus type-1 (HTLV-1) and HTLV-2 was conducted in Sao Paulo, Brazil among 2,312 individuals that included following groups: 1,148 volunteer blood donors, 37 patients with tropical spastic paraparesis (TSP), 53 with lymphoproliferative disorders, 171 with a history of multiple blood transfusions, 268 human immunodeficiency virus type-1 (HIV-1) seropositive subjects, and 635 Amazonian Indians. Antibodies to HTLV-1/2 were screened by enzyme-linked immunosorbent assay (ELISA) and confirmed by Western blot and/or radioimmunoprecipitation. The differentiation of HTLV-1 and HTLV-2 was achieved using a synthetic recombinant peptide (rgp46) ELISA. We confirmed the presence of HTLV-1 infection in Brazil, both in blood donors (0.4%) and in patients exposed to blood transfusions (2.9%), as well as the occurrence of HTLV-1-associated TSP (11 patients, or 30% of all TSP cases) and adult T cell leukemia/lymphoma (two cases, or 3.5% of all hematologic malignancies). The HIV-1 infected individuals were shown to be coinfected (8.9%) with either HTLV-1 or HTLV-2. All HIV-1 and HTLV-2 coinfected individuals were intravenous drug abusers. In addition, we also demonstrated the presence of HTLV-2 (4.7%), and HTLV-1/2 (0.8%) in tribes of Amazonian Indians who lived in the eastern Amazon basin (southeastern State of Para). The selectivity of these retroviral infections in particular groups is emphasized, as well as the need for HTLV-1/2 screening of all blood donors in Brazil as a public health measure.
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PMID:Selectivity of human T lymphotropic virus type-1 (HTLV-1) and HTLV-2 infection among different populations in Brazil. 827 33

Individuals with HIV-1 infection show two major immunological effects--the early persistent stimulation of immune responses (e.g. of CD8 cells and antibody production) and later catastrophic immunodeficiency. Peripheral blood dendritic cells (DC) become infected with HIV-1 and infected cells can stimulate responses to virus. By contrast infected DC show a reduced capacity to stimulate either primary or secondary responses to other antigens. We have proposed that the block, particularly in primary responses, may be instrumental in the development of immunodeficiency. In HTLV-1 infected patients with tropical spastic paraparesis (TSP) a major feature of disease is 'spontaneous' T cell proliferation thought to underlie development of inflammatory neurological disease. We have now shown that some DC in addition to T cells are infected in TSP and that DC stimulate the persistent T cell activity. Here we demonstrate this using cells from an informative family where the daughter was normal, the father an HTLV-1 seronegative carrier and the mother had TSP. DC from all individuals stimulated normal allogeneic T cells in a mixed leukocyte reaction (MLR) and T cells responded well to normal allogeneic DC. T cells from the daughter showed little stimulation with autologous DC, those from the father showed significant but low stimulation, and T cells from the TSP patient gave a response to autologous DC which exceeded that to allogeneic DC. Taken together, the studies of DC and both HIV-1 and HTLV-1 indicate that infection of DC may play a central role in development of T cell abnormalities in human retrovirus-induced diseases.
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PMID:Dendritic cells in HIV-1 and HTLV-1 infection. 837 24

Bovine leukaemia virus (BLV) is the aetiological agent of Leukosis enzootica bovis [Viral Oncology (1980), G. Klein (Ed.) Raven Press, New York, pp. 231-238], a widely spread disease in cattle. BLV is reported as the animal model of human T-cell leukaemia virus (HLTV) which is the causative agent of adult T-cell leukaemia and tropical spastic paraparesis. Like the viruses themselves, the two retroviral proteinases (PR) are very closely related [Virology 142 (1985) 357-377]. BLV and HTLV-I PR are reported as putative proteins made of 126 [J. Virol. 57 (1986) 826-832] and 125 [FEBS Lett. 293 (1991) 106-110] amino acids, respectively (long sequences), belonging to the aspartyl proteinase family [Nature 329 (1987) 351-354], with the aid of molecular modelling, we show that BLV and HTLV-I proteinases made of only 116 and 115 amino acids, respectively (short sequences), display three-dimensional structures similar to that observed for other retroviral aspartyl proteinases. The models are based on three-dimensional structures of Rous sarcoma virus (RSV PR) and the human immunodeficiency virus (HIV-1 PR). We used solid phase peptide synthesis to produce the putative proteolytic enzyme of BLV (116 amino acids). In this study, we show that the folded synthetic protease accurately hydrolyzes a decapeptide corresponding to the sequence of the Matrice-Capside (MA/CA) cleavage site of the gag polyprotein. In addition, the proteolytic activity is inhibited by a statine ((4S,3S)-4-amino-3-hydroxyl-6-methylheptanoic acid) containing an analogous sequence.
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PMID:Modelling, synthesis and biological activity of a BLV proteinase, made of (only) 116 amino acids. 839

In serum, the enzyme adenosine deaminase (ADA) is known to be divided into two isoenzymes, ADA1 and ADA2, which have different molecular weights and kinetic properties. The present study investigated ADA isoenzyme levels in the sera of patients infected with retroviruses associated with adult T-cell leukemia (ATL), human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM), and AIDS, ADA isoenzyme activities were found to be significantly (P < 0.001) higher in the sera of patients with ATL, HAM, and AIDS than in the sera of healthy controls. In the case of the ADA subtypes in the sera of patients with ATL, ADA1 activity was significantly (P < 0.001) elevated in patients with the acute and lymphoma types of ATL compared with that in patients with the chronic and smoldering types of ATL. ADA2 activity was significantly elevated in the sera of patients with the acute, lymphoma, and chronic types of ATL (P < 0.001) compared with that in patients with smoldering ATL and HTLV-1 carriers. In the case of patients with human immunodeficiency virus type 1 (HIV-1) infection, ADA1 and ADA2 activities in the sera of patients with AIDS and HIV-1 antibody-positive individuals were significantly (P < 0.001) higher than those in the sera of HIV-1 antibody-negative individuals. A significant elevation in ADA2 activity was also seen in the sera of AIDS patients (P < 0.01) compared with that in the sera of HIV-1 antibody-positive individuals. These results suggest that the magnitude of elevation of ADA isoenzyme levels in serum correlates well with the clinical conditions of the patients with these diseases. Measurement of the activities of ADA isoenzymes may therefore provide an additional parameter for distinguishing the subtypes of ATL and may prove to be useful as prognostic and therapeutic monitors in diseases associated with HTLV-1 and HIV-1 infections.
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PMID:Adenosine deaminase isoenzyme levels in patients with human T-cell lymphotropic virus type 1 and human immunodeficiency virus type 1 infections. 854 45

Human T-lymphotropic virus type I (HTLV-1) is associated with tropical spastic paraparesis (TSP) and adult T-cell leukemia/lymphoma. HTLV-I infection is endemic in certain parts of the Natal/KwaZulu region of South Africa. No studies on the seroprevalence of HTLV-I infection in the Free State (FS) have been published. This study was designed to determine the prevalence of HTLV-I antibodies among different patient groups in the FS. Sera from 863 patients were analyzed. There were: 178 asymptomatic rural Blacks, 200 asymptomatic urban Blacks, 50 asymptomatic Whites, 60 patients with spastic myelopathy, 70 patients with other neurologic disorders, 12 patients with T-cell haematologic malignancies and 293 human immunodeficiency virus (HIV) seropositive patients. Sera were tested for the presence of HTLV-I/II antibodies using an enzyme linked immunosorbent assay (ELISA). Positive results were confirmed using a modified Western blot assay. None of the asymptomatic Whites were HTLV-I antibody positive (95 pc confidence interval (CI): 0 to 7 pc), while 2 pc (95 CI: 0.5 to 5 pc) of asymptomatic urban Blacks and 1.1 pc (95 pc CI: 0.14 to 4 pc) of asymptomatic rural Blacks had HTLV-I antibodies. Of the group of patients with spastic myelopathy 33.3 pc (95 pc CI: 21.7 to 46.7 pc had HTLV-I antibodies, while none of the patients with T-cell haematologic malignancies (95 pc CI: 0 to 26.5 pc) or other neurologic disorders (95 pc CI: 0 to 5 pc) had HTLV-I antibodies. Of the HIV seropositive patients 6.1 (95 pc CI: 4 to 9.5) were co-infected with HTLV-I. HTLV-I infection is present in the Free State. It is strongly associated with spastic myelopathy in this region. HIV seropositive patients have a high rate of HTLV-I co-infection.
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PMID:HTLV-I infection in the Free State region of South Africa: a sero-epidemiologic study. 865 70

Human T-cell lymphotropic virus type II (HTLV-II) is endemic in several ethnic tribes and among intravenous drug users in metropolitan areas. Despite the presence of HTLV-II in these various populations, the association of HTLV-II with disease is sparse and mainly limited to isolated case reports. This study is an extension of an earlier description of an HTLV-II-infected patient with neurologic disease and presents the clinical and immunologic findings of 4 patients with HTLV-II seropositivity and spastic paraparesis. The patients are of African-American origin with 3 of the patients being of Amerindian descent. All of the patients are seronegative for the human immunodeficiency virus (HIV). The patients progressed to a nonambulatory state in less than 5 years. Magnetic resonance imaging studies obtained from 3 of the patients demonstrated white matter disease in the cerebrum and spinal cord. The cerebrospinal fluid and serum contained antibodies to HTLV-II. The presence of proviral HTLV-II was confirmed by polymerase chain reaction analysis of peripheral blood lymphocytes (PBLs). A spinal cord biopsy from 1 patient demonstrated HTLV RNA within a lesion. Immunologic studies on 2 patients demonstrated that spontaneous lymphoproliferation of PBLs was present but decreased relative to HTLV-I-infected patients. The clinical and immunologic findings from these HTLV-II-infected patient resemble those found in HTLV-I-associated myelopathy/tropical spastic paraparesis.
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PMID:Human T-cell lymphotropic virus type II-associated myelopathy: clinical and immunologic profiles. 895 12

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