Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prevention of a secondary response to factor IX by cyclophosphamide was attempted in an 11 year old patient with severe Christmas disease. An antibody to factor IX had been present for 4 years before immunosuppressive therapy was tried. Despite profound lymphopenia, synthesis of factor IX antibody was not depressed. The difficulties of modifying the anamnestic response to factor IX by chemical immunosuppression may be as real as has been reported for factor VIII in classical haemophilia.
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PMID:Failure of immunosuppression in a severe haemophilia B patient with specific antibody. 103 30

With the advent of standard flow cytometric methods using two-colour fluorescence on samples of whole blood, it is possible to establish the ranges of CD3, CD4 and CD8 T lymphocyte subsets in the routine laboratory, and also to assist the definition of HIV-1-related deviations from these normal values. In 676 HIV-1-seronegative individuals the lymphocyte subset percentages and absolute counts were determined. The samples taken mostly in the morning. The groups included heterosexual controls, people with various clotting disorders but without lymphocyte abnormalities as well as seronegative homosexual men as the appropriate controls for the HIV-1-infected groups. The stability of CD4% and CD8% values was demonstrated throughout life, and in children CD4 values less than 25% could be regarded as abnormal. The absolute counts of all T cell subsets decreased from birth until the age of 10 years. In adolescents and adults the absolute numbers (mean +/- s.d.) of lymphocytes, CD3, CD4 and CD8 cells were 1.90 +/- 0.55, 1.45 +/- 0.46, 0.83 +/- 0.29 and 0.56 +/- 0.23 x 10(9)/l, respectively. In patients with haemophilia A and B the mean values did not differ significantly. In homosexual men higher CD8 levels were seen compared with heterosexual men and 27% had an inverted CD4/CD8 ratio but mostly without CD4 lymphopenia (CD4 less than 0.4 x 10(9)/l). However, some healthy uninfected people were 'physiologically' lymphopenic without having inverted CD4/CD8 ratios. When the variations 'within persons' were studied longitudinally over a 5-year period, the absolute CD4 counts tended to be fixed at different levels. As a marked contrast, over 60% of asymptomatic HIV-1+ patients exhibited low CD4 counts less than 0.4 x 10(9)/l together with inverted CD4/CD8 ratios. Such combined changes among the heterosexual and HIV-1-seronegative homosexual groups were as rare as 1.4% and 3%, respectively. For this reason, when the lymphocyte tests show less than 0.4 x 10(9)/l CD4 count and a CD4/CD8 ratio of less than unity, the individuals need to be investigated further for chronicity of this disorder, the signs of viral infections such as HIV-1 and other causes of immunodeficiency.
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PMID:Laboratory control values for CD4 and CD8 T lymphocytes. Implications for HIV-1 diagnosis. 134 72

CD4+ lymphocyte counts of 91 HIV+ hemophilia patients were monitored for a mean of 4 years (range: 15-69 months). CD4+ lymphocytes decreased in 55 but increased in 36 patients over time. The CD4+ cell increases were persistent in 5 patients, whereas they fluctuated in 31. Of the 36 patients with increasing CD4+ counts 3 developed AIDS and 1 LAS. The other 32 patients were clinically asymptomatic (CDC II), but had immunological abnormalities, such as increased serum neopterin (N = 18) and impaired in vitro T cell responses to pooled allogenic stimulator cells (N = 15) or mitogens (N = 18). In contrast, of the 55 patients whose CD4+ cells decreased, 24 developed AIDS and 5 ARC (P less than 0.0005). Only 2 of these 55 patients had normal mitogen stimulation in vitro and normal serum neopterin levels.
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PMID:Improving CD4+ lymphocyte counts in HIV-infected hemophilia patients. A favorable prognostic indicator? 168 52

This study was established to examine the longitudinal consequences of F VIII therapy on immune function in 24 patients with haemophilia A. Antibodies to the human immunodeficiency virus (HIV) were found in 15 of 16 patients with severe haemophilia and in 2 of 8 patients with mild disease. The principal clinical and immunological abnormalities were restricted to the HIV antibody-positive patients: T helper cell lymphopenia (less than 0.55 x 10(9)/l) in 10 patients, persistent glandular lymphadenopathy in 4 patients and depressed response to skin recall antigens in 7 of 9 HIV-positive patients tested. Although no extension of these immunological and clinical abnormalities developed in the 18-month period of monitoring, T helper cell counts and platelet counts were significantly lower in a group of patients with established long duration HIV seropositivity (since 1982/1983) in comparison with the remaining seropositive patients. This suggests that a progressive pathological process is associated with infection by this virus, but the factors which determine the long-term sequelae are still unknown.
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PMID:Relationship of clinical and immunological abnormalities in haemophilia A to F VIII therapy and HIV exposure: a longitudinal study. 296 54

Five of 22 hemophiliacs who were seropositive for human T cell leukemia virus III (HTLV III) and manifested severe impairment of immune parameters (both in vivo and in vitro) similar to those observed in patients with clinical symptoms of acquired immune deficiency syndrome (AIDS) were chosen for this study. Profound lymphopenia was observed in four of five patients with decreased and qualitatively impaired helper/inducer (T4) cells and increased T suppressor/cytotoxic (T8) cells. Observed in all patients was impaired endogenous production of interleukin-2 (IL-2), expression of the IL-2 receptor combined with diminished responses to mitogens, mixed leukocytes reaction (MLR), and natural killer (NK) reactivity. In vitro supplement of exogenous IL-2 markedly augmented T and NK cell functions, as well as the expression of activation antigens on both T4 and T8 cell in four of five patients. Our findings suggest that a substantial proportion of this cell-mediated immunologic defect in hemophiliacs stems from their inability to produce adequate amounts of IL-2. Interleukin-2 may therefore have the potential for therapy as an immune response modifier in patients with hemophilia by providing beneficial preventive therapy for patients at risk.
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PMID:In vitro restoration by interleukin-2 (IL-2) of the impaired natural killer cell activities, IL-2 receptor expression, and T cell proliferation in hemophilia. 309 Feb 9

Unresponsiveness to skin testing with PPD and tetanus toxoid was commonly seen in patients with haemophilia A but not infected with human immunodeficiency virus but was uncommon in controls. Vaccination history indicated that the unresponsive patients had not been immunised in childhood. Other tests of immune competence (skin tests with other antigens, lymphocyte stimulation with mitogens and antigens, and viral serology) showed that the haemophilia A patients had an adequate response to pathogens to which they had been exposed. Five of 12 such patients had a mild T4 lymphopenia, and this may have been related to parenteral administration of large quantities of protein.
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PMID:Unresponsiveness to skin testing with bacterial antigens in patients with haemophilia A not apparently infected with human immunodeficiency virus (HIV). 349 42

We report here nine children with AIDS. The risk factors of these patients were hemophilia in one, blood transfusions in four, maternal intravenous drug use in three and paternal AIDS in one. One baby was also of Haitian parentage. The major clinical symptoms included failure to thrive, hepatomegaly, lymphadenopathy, interstitial pneumonia, recurrent bacterial and viral infections and persistent oral thrush. Three infants had chronic recurrent parotitis. Five infants developed opportunistic infections primarily Pneumocystis carinii pneumonia and all five died of bacterial sepsis. None of the infants were lymphopenic but all had reversed T4/T8 ratios and poor in vitro lymphocyte responses to pokeweed mitogens. Although many of the clinical and laboratory features of pediatric and adult AIDS are similar, there are some unique features for pediatric AIDS such as the absence of lymphopenia and the high prevalence of recurrent bacterial infections and sepsis.
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PMID:Acquired immunodeficiency syndrome (AIDS) in infants and children: report of nine cases. 383 Feb 64

The immunological status of two multiply transfused patients with severe haemophilia A and diffuse lymphadenopathy was evaluated. But patients appeared clinically well and denied homosexuality or intravenous drug abuse. Immunological studies revealed depressed cellular immune function with anergy, relative lymphopenia, decreased T helper cells, and increased T suppressor cells in both patients and an altered T-helper-cell/T-suppressor-cell ratio in one. Hyperresponsiveness of the humoral immune system was demonstrated by elevated IgG and IgM. Lymph-node biopsy revealed benign nonspecific hyperplasia in both patients. The lymphadenopathy and immunological features in these two haemophiliacs bear a striking resemblance to the acquired immunodeficiency syndrome (AIDS) of homosexuals, intravenous drug abusers, and Haitian immigrants. These findings may represent a prodromal phase or a forme fruste of AIDS. Transmission of an infectious agent in blood products seems likely.
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PMID:Acquired-immunodeficiency-like syndrome in two haemophiliacs. 613 Feb 47

Acquired immunodeficiency syndrome (AIDS) is a disease of unknown aetiology characterised by opportunistic infections, unusual malignancies, and aberrations in cellular immunity; lymphopenia and reversal of helper/suppressor T-cell ratios are the most common. In this study the immune function in 25 children and adolescents with haemophilia was assessed. Normal humoral immunity was observed in all patients evaluated. T and B cell numbers and stimulation were within normal limits. However, 5 of the patients had reversed helper/suppressor T-cell ratios similar to, but less pronounced than, the reversed ratio observed in patients with clinical signs of AIDS. These results suggest that patients with haemophilia may be at increased risk of AIDS and that regular investigation of cellular immunity is warranted in these patients.
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PMID:Altered distribution of T-lymphocyte subpopulations in children and adolescents with haemophilia. 613 Dec 13

Two patients with hemophilia A had generalized lymphadenopathy, lymphopenia, elevated IgG values, depressed T4 (helper) lymphocytes, elevated T8 (suppressor) lymphocytes, and abnormally low T4/T8 ratios. One of the patients, who also had hepatosplenomegaly, underwent cervical lymph node biopsy; the node contained 43% T8-lymphocytes, a marked elevation over the small fraction of T8 cells usually found in lymph nodes. These patients may have a form of the acquired immune deficiency syndrome described in male homosexuals, Haitians, intravenous drug abusers, and recently, in patients with hemophilia. We studied T cell phenotypes in 43 patients with hemophilia. Fourteen of 28 patients given commercial factor VIII concentrates had abnormal T4/T8 ratios; none of nine patients who used cryoprecipitate had abnormal values. T4 helper cells were significantly lower, T8 suppressor cells significantly elevated, and T4/T8 ratios significantly lower in the lyophilized concentrate users and in patients with hemophilia as a total group. The type of therapeutic factor VIII replacement may alter the risk of developing T4/T8 abnormalities or AIDS.
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PMID:Generalized lymphadenopathy and T cell abnormalities in hemophilia A. 622 33


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