Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with thalassemia who receive multiple blood transfusions are at risk for the acquired immunodeficiency syndrome. Peripheral blood lymphocyte subpopulations were studied in 22 multitransfused thalassemic patients; 10 patients were without splenectomy and 12 were studied after splenectomy. Both groups were negative for anti-HIV. Four additional patients who were found positive for anti-HIV and ten healthy controls were also included in this study. Patients without splenectomy compared to controls and to patients after splenectomy showed a significant decrease of both percentage (p less than 0.001) and absolute numbers (p less than 0.001) of Leu-7+ cells without significant abnormalities of T4/T8 ratio (1.56 +/- 0.4). Patients after splenectomy compared to controls and to patients without splenectomy showed a significant increase of the absolute numbers of lymphocytes and lymphocytes subsets T11+, T3+, T4+, T8+ and SmIg+ cells. In the seropositive patients for HIV only a significant increase of the absolute number of T8+ cells was observed while the T4/T8 ratio was 1.24 +/- 0.73. The decrease in the percentage of Leu-7+ cells in patients without splenectomy correlated inversely to the total amount of blood transfused. In conclusion patients with thalassemia had normal T4/T8 ratio and did not show the abnormal immunologic profile that has been reported in haemophiliacs.
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PMID:Immune status of Greek patients with beta-thalassemia major negative for anti-HIV. 310 26

We studied 66 Israeli hemophiliacs for antibodies to HIV in blood samples collected between 1978 and 1985. By May 1985, 2 had AIDS, 2 had ARC, 4 had lymphadenopathy with some immunologic dysfunction, and 58 were asymptomatic. Antibodies to HIV were detected in 40 (60.6%) patients, including all 8 with disease. Presence of HIV antibodies was significantly associated with receipt of non-heat-treated commercial factor VIII concentrates (NHT fac VIII) between 1980 and 1983. Thirty-eight of 45 (84.44%) patients treated with NHT fac VIII developed antibodies to HIV, compared to 1 of 16 (6.25%) treated with cryoprecipitates and fresh plasma only. Of 40 seropositive patients, 1 (2.5%) had antibodies by 1980, 4 (10%) by 1982, 14 (35%) by 1983, 10 (25.0%) by 1984, and 11 (27.5%) by May 1985. The decline in the rate of seroconversion can be attributed to the replacement of NHT fac VIII concentrate with heat-inactivated factor VIII (HT fac VIII) concentrate by November 1983. As of January 1984 only HT fac VIII was administered. Twenty-nine multitransfused thalassemia patients as well as 20 healthy Israeli blood donors were seronegative to HIV. All 40 (100%) seropositive hemophiliacs had antibodies to viral env gene encoded gp120/gp160 antigens. Twenty-four (60.05%) also had antibodies to viral gag gene encoded p24 and/or p55 antigens. While antibodies to gp120/160 persisted during the follow-up time, a loss of antibodies to p24/55 was observed in 5 of 16 (31.25%) seropositive patients from whom multiple samples were available. gp120/160 positive, p24/55 negative hemophiliacs had significantly lower absolute T-helper cell counts and reversed Th/Ts ratios when compared to gp120/160 p24/55 seropositive patients. Four of the 16 (25.0%) asymptomatic gp120/160 positive, p24/55 negative patients developed overt disease within 15 months of the last blood collection. The data suggest that exposure to HIV antigens is widespread among hemophiliacs in Israel, and can be attributed to receipt of NHT fac VIII concentrates prior to 1984. Antibodies to gp120/160 are of the most important diagnostic value while loss of antibodies to p24/p55 may be of prognostic value.
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PMID:Antibodies to HIV in Israeli hemophiliacs: relationship between serological profile and disease development. 312 74

Anti-HIV 1 antibodies were detected in 4 groups of subjects (peoples attending hospitals or medical clinics for anti-HIV investigation, blood donors, women in massage parlours and thalassemia patients) in the north, northeast and central Thailand. A total number of 1,726 blood samples were initially tested with ELISA. The ELISA reactive samples were confirmed by the Western blot analysis. Using ELISA as a screening test, the highest incidence (9.09%) of anti-HIV 1 antibodies was found in thalassemic children (4 of 44). Six (0.72%) and 4 (1.02%) samples in the first, second and third groups had a repeatedly reactive ELISA respectively. The Western blot analysis confirmed that 7 cases (3 thalassemia and 4 subjects in the first group) had antibodies to HIV 1. Two cases with reactive Western blot test were Westerners while the rest were symptomatic and asymptomatic Thais. The HIV infection has spread to thalassemia patients probably via blood transfusion.
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PMID:Epidemiological assessment of anti-HIV 1 antibodies in Thailand. 323 68

We investigated the incidence, clinical and immunological characteristics of human immuno-deficiency virus (HIV) infection in a group of multi-transfused patients with thalassaemia major who were exposed to transfusion-associated HIV infection. Seropositivity to HIV by Western blot and immunofluorescence analysis was detected in 26 out of 590 patients. At a follow up 21-40 months later, none of these seropositive patients had developed acquired immuno-deficiency syndrome (AIDS), and six manifested the AIDS related complex (ARC). ARC was unusually mild and consisted of moderate laterocervical and submandibular lymph node enlargement associated with hypergammaglobulinaemia and a reduced CD4/CD8 ratio resulting from the decreased number of CD4 lymphocytes. These findings suggest that multi-transfused patients with thalassaemia major are relatively resistant to the development of severe manifestations of HIV infection, presumably because their immune status is relatively better preserved than that of other infected populations. Longer follow up is, however, necessary to determine whether the incidence of AIDS will be lower in this population or whether overt AIDS merely takes longer to develop.
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PMID:Human immunodeficiency virus infection in multi-transfused patients with thalassaemia major. 339 Dec 24

We have studied both the humoral and cell mediated immune systems of 23 children with beta-thalassaemia major. In children who had not been splenectomized, a 3-fold expansion in the number of circulating B cells and a modest polyclonal gammopathy was present. Of these patients 70% had decreased numbers of circulating T4 cells; 83% were unresponsive to skin testing with Candida albicans, and the majority had decreased lymphocyte proliferative responses in vitro. In children who had been splenectomized, there was a 10-fold increase in the number of circulating B lymphocytes and a 2-fold increase in the number of T4 and T8 cells present in peripheral blood. Additionally, these patients as a group were more responsive to both skin testing and lymphocyte stimulation in vitro with Candida albicans. Seven patients had an inverted T4/T8 ratio. One child has positive serology to HIV by ELISA and Western Blot techniques with a normal T4/T8 ratio. Thus, while children with thalassaemia are at risk for exposure to HIV, the immunological abnormalities associated with the disease and/or its treatment necessitates cautious interpretation of any AIDS-related immunological changes.
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PMID:Abnormalities in the immune system of children with beta-thalassaemia major. 349 80

38.5% or 10 of 26 children with thalassemia who had received blood transfusion have tested positive for HIV at the Hamad General Hospital, Doha, Qatar. Blood for transfusion was obtained from commercial suppliers in Miami, Florida, since late 1970. Since December 1985 all blood for transfusion has been tested for HIV for ELISA kits from Abbott Laboratories, Chicago, Ill, and confirmed by Western Blot. These children ranged from 3-13 years old, mean 7.7, and had received an average of 63.5 transfusions. All had been given at least 30 transfusions. Children testing negative for HIV averaged 35.0 lifetime transfusions. 4 of the affected children had persistent generalized lymphadenopathy, but no other symptoms were recorded.
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PMID:High prevalence of human immunodeficiency virus infection in children with thalassemia exposed to blood imported from the United States. 367 Sep 41

Seroconversion of human immunodeficient virus (HIV)-antibody post blood transfusion has been reported (Jett et al, 1983; Cumming et al, 1989). We report here, six hematologic patients who became HIV-antibody positive after receiving HIV seronegative blood and blood components during their illness. There were three cases of acute non-lymphocytic leukemia, one thalassemia, one dyshemopoiesis and one hemophilia A. Thus, the risk of acquiring HIV infection from transfusion remains, despite the routine serological screening of donated blood by HIV ELISA tests. So the laboratory screening of blood should be improved by using more sensitive and specific antibody kits, including the use of HIV antigen testing, which have been reported to be useful in the diagnosis of patients with the early HIV infection.
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PMID:Six cases of seroconversion of human immunodeficient virus (HIV) antibody post-transfusion in HIV seronegative bloods. 788 55

Recently there have been increasing reports of HIV infection acquired through transfusion of HIV seronegative blood in Thailand due to high incidence of HIV new infection in blood donors. Blood or blood components (BC) prepared from HIV seronegative blood donation pose significant hazards to recipients because of the risk of viremia during the "window period" of HIV infection. This paper presents the HIV seroprevalence in hematologic patients other than hemophiliacs who received multiple blood transfusion at Ramathibodi Hospital. The retrospective analysis was done on 167 patients: 132 thalassemia, 19 leukemia, 5 aplastic anemia, 5 ITP, 2 pure red cell aplasia, 2 congenital non spherocytic hemolytic anemia, 1 hereditary spherocytosis and 1 autoimmune hemolytic anemia patients, who received blood transfusion during January 1, 1987 till February 29, 1992 at the Department of Pediatrics, Ramathibodi Hospital. The number of blood or BC transfused in each patient was 1-154 units with the average of 23 units per patient per 5 years with a total 4,000 units. All were HIV sero-negative. Anti-HIV screening was performed periodically in these patients about 1-2 times per year or as necessary. The results were HIV seronegative in all cases. The reason for negative results cannot be explained clearly. It should be noted that our thalassemic patients receive leukocyte poor blood and avoid a hypertransfusion program. Patients with other blood diseases received both whole blood and BC. The HIV contaminated blood in the window period was estimated to be 1:10,000 in Thailand which showed HIV antigen positive but antibody negative. These patients may be fortunately received HIV non contaminated blood.
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PMID:HIV seroprevalence in hematologic patients other than hemophiliacs at Ramathibodi Hospital. 788 70

To study the relationship between the dose of desferrioxamine (DFX) and the progression of the HIV-1 disease in thalassaemia major patients (TMP), 64 seropositive TMP patients were studied. Cumulative incidence of CDC stage IV was calculated using a non-parametric life-table method. The association with the mean daily dose of DFX was tested with a Cox proportional hazards model which was also used to adjust for confounding variables. The median of the mean daily dose of DFX over the seropositive period was 40 mg/kg (range 0-65 mg/kg). Age at seroconversion (P < 0.02) and splenectomy (P < 0.03) were found to be associated with the mean daily dose of DFX. 6.5 years after seroconversion, 11% of those who had been prescribed more than 40 mg/kg of DFX daily had entered stage IV versus 35% of those who had been prescribed a lower dose (P < 0.01). When the dose was taken as a continuous variable it was found that the rate of progression was significantly smaller in TMP receiving a higher dose (P < 0.002), even after adjusting for age and splenectomy (P < 0.02). Although it should be noted that these results were obtained in an observational study, possibly biased by a non-random allocation of the DFX dose, we believe that they are striking enough to support the claim that the role of DFX in the progression of HIV disease should be further evaluated.
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PMID:Dose of desferrioxamine and evolution of HIV-1 infection in thalassaemic patients. 798 27

Ninety patients with thalassaemia major were investigated for the occurrence of antinuclear antibodies (ANA), and those with ANA were tested for antibodies to histones (AHA). ANA were detected in 7 of 27 thalassemics on oral iron chelator L1, and in 2 of 63 thalassaemics not on L1 (p < 0.01). AHA were seen in 4 of 7 thalassemics receiving L1 with positive ANA, and in none of the 2 not receiving L1 (p < 0.03). Joint pains were seen in patients receiving L1, but in none of the patients not receiving L1. There was no correlation between hepatitis B or HIV positivity and presence of ANA or joint pains. While some amount of background ANA-positivity was found in patients with thalassaemia major, it was significantly more in patients receiving L1. Laboratory evidence of drug-induced lupus-like reaction was seen only in patients who received L1. In view of serious concerns about the safety of L1 and wide variations in the incidence and severity of adverse reactions reported by different sources, an urgent regulatory audit of all trial centres is essential.
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PMID:Autoantibodies in thalassaemia major: relationship with oral iron chelator L1. 786 14


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