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Immunological studies were performed on a group of 44 haemophilia A and 15 haemophilia B patients who were treated exclusively with blood products manufactured by the Scottish National Blood Transfusion Service (SNBTS). All patients were HIV seronegative throughout the study. Of the haemophilia A patients 14 (32%) had CD4+ lymphocyte subset counts less than or equal to 0.5 x 10(9)/l, compared with one (6%) haemophilia B patient and four (8%) controls. The percentage of activated T cells was greater than 5% in 19/33 (57%) with haemophilia A, 5/9 (55%) haemophilia B and 14/50 (28%) of control subjects. beta 2 microglobulin values greater than or equal to 2.0 mg/l were observed in 19 (43%) haemophilia A and four (26%) haemophilia B patients, compared with one (2%) control. No significant increases in serum interleukin-2 receptor concentrations were observed in 15 haemophilia A and one haemophilia B patients. Significantly elevated levels of IgG, IgM and IgA were observed in the haemophilia A group, but elevation of immunoglobulins was restricted to the IgG class in the haemophilia B group. Of the haemophilia A patients 16/30 (53%) and 6/11 (54%) haemophilia B patients had depression of cell-mediated immunity (CMI) as assessed by delayed-type hypersensitivity responses to intradermally injected recall antigens. There was no correlation between factor VIII or factor IX usage and changes in lymphocyte subsets, beta 2 microglobulin, and immunoglobulin levels. There was, however, a strong correlation between annual factor VIII usage and the degree of depression of CMI for those with haemophilia A but not for those with haemophilia B. No correlation between alterations in the immune parameters and disturbance of liver function tests was observed in either haemophilia A or haemophilia B patients. We conclude that alloantigen or non-HIV viral exposure due to repeated administration of factor concentrates brings about alterations in the immune response, and that these changes are more marked following exposure to intermediate purity factor VIII compared with factor IX concentrate.
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PMID:Immunological studies in HIV seronegative haemophiliacs: relationships to blood product therapy. 158 Dec 16

At the Oxford Haemophilia Centre at Churchill Hospital in Oxford, England, psychiatrists compared data on 37 HIV seropositive hemophiliacs (31 asymptomatics and 6 symptomatics) with data on 36 HIV seronegative hemophiliacs to determine the prevalence of psychosocial conditions in HIV seropositive men and factors related to those conditions. HIV seropositive men had much higher Present State Examination scores than did the HIV seronegative men (5.9 vs. 2.2; p=.005). This was true for both symptomatic and asymptomatic HIV seropositive men (7.3 and 5.7, respectively). Nevertheless, these levels of psychological disturbance were basically the same as the general outpatient medical population as was also the case with depression scores (POMS). Still symptomatic HIV seropositive men were more likely to be depressed than HIV seronegative men (6.4 vs. 3.6; p=.02). The most important finding was that both symptomatic and asymptomatic HIV seropositive men felt significantly higher levels of hopelessness than did the HIV seronegative men (6.5 vs. 2.6; p=.0004, asymptomatic-5.8 and symptomatic=9.7). Hopelessness indicated an increased risk of suicide which is independent of depression. Past psychiatric history (r=0.38), hopelessness (r=0.55), and poor social adjustment (r=0.55) accounted for 49.8% of the variance (p.001). HIV seropositive men exhibited more psychosexual dysfunction especially premature and delayed ejaculation (30% vs. 4% and 20% vs. 0, respectively; p.05) than HIV seronegative men. Among the men who had a sexual relationship, HIV seropositive men tended to always use condoms during intercourse (79% of asymptomatics and 100% of symptomatics vs. 25% of HIV seronegatives; p=.0004). Even though they used condoms, HIV seropositive men were more likely to worry about infecting their partners than HIV seronegative men (p=.02).
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PMID:The psychosocial impact of HIV infection in men with haemophilia: controlled investigation and factors associated with psychiatric morbidity. 161 81

We examined psychiatric correlates of human immunodeficiency virus (HIV) infection in a major risk group for acquired immunodeficiency syndrome, men with hemophilia. A central goal was to identify psychosocial factors associated with increased vulnerability to psychiatric distress after infection with HIV. Seventy-five hemophiliacs, 31 of whom were HIV seropositive (HIV+), were studied. The HIV+ men had elevated depression, anxiety, and anger-hostility symptom scores relative to those of men who were seronegative for HIV. There were no additional symptom differences among men according to infection stage or clinical severity of hemophilia. Men with any of eight psychosocial characteristics were particularly susceptible to effects of infection on mental health: a personal history of psychiatric distress before HIV diagnosis; familial psychiatric history; a high school education or less; low social support from one's wife; low family support; low friend support; a poor sense of mastery over one's life; and experiencing recent life events involving loss. The HIV+ men with one or more such characteristics were highly symptomatic; remaining HIV+ men had significantly lower symptom levels, similar to the low levels noted in the men seronegative for HIV. The findings provide initial empiric support for the notion that clinical services to alleviate emotional distress should be targeted to intervene on HIV+ persons' psychosocial assets and liabilities.
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PMID:Infection with human immunodeficiency virus and vulnerability to psychiatric distress. A study of men with hemophilia. 237 44

The authors report the first two cases of lethal AIDS in haemophilia A in France. One French case of AIDS in haemophilia B has already been reported. The diagnosis was based on the observation of opportunist infections associated with severe depression of cell-mediated immunity in both cases. The possibility of active transmission of the LAV retrovirus by factor VII concentrate is discussed. This raises the problem of the signification of anti-LAV antibodies in haemophiliac patients and the control of products used for the treatment of haemophilia.
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PMID:[2 cases of AIDS in patients with hemophilia A]. 302 24

A previously healthy patient with classic hemophilia who was on a home infusion program with factor VIII concentrates developed an acquired immunodeficiency syndrome manifested by a dramatic weight loss (47 kg over 12 months), lassitude, transient thrombocytopenia, and opportunistic infections with Varicella zoster, Pneumocystis carinii, and Mycobacterium avium-intracellulare. The patient was not homosexual and had no history of intravenous drug abuse. Immunologic studies showed a persistent lymphopenia with reversal of helper/suppressor-cytotoxic T-lymphocyte ratios, depression of human natural killer cell function, and in-vitro lymphocyte proliferative responses to mitogens and viral antigens. Serum IgA levels were also elevated. Serum antibodies against cytomegalovirus, herpes simplex viruses 1 and 2, Epstein-Barr virus, Varicella zoster, and hepatitis B virus were shown, suggesting previous infection by these agents. Reactivation of cytomegalovirus infection was suggested by a rising titer of antibodies against cytomegalovirus concurrent with pneumocystis pneumonia, and was confirmed by the growth of this virus in a throat culture 2 months later.
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PMID:Acquired immunodeficiency syndrome with Pneumocystis carinii pneumonia and Mycobacterium avium-intracellulare infection in a previously healthy patient with classic hemophilia. Clinical, immunologic, and virologic findings. 629 53

World-wide blood demands are increasing at 18% per year. Around 50% of collected blood is used whole -- for surgery, accident victims, anaemias, malignancies, and haemoglobinopathies. Blood component therapy is placing extraordinary demands upon Blood Collection Services. This therapy uses multiple or single cell types, and a variety of plasma-derived components. Malignant over-growth depresses marrow function; and, active treatment, temporarily, similarly depresses cell production. Specific transfusion cells are available for the patient's protective mechanism. Platelets are required, fairly fresh, for depletion and for their primary clotting ability. Similarly, infused platelets are used to counter local or systemic drug sensitive platelet depression. From plasma components, specific coagulation products are available for health and during surgery for haemophilia, Christmas, and Von Willebrand's diseases. Prior to oral surgery, all possible coagulation abnormality problems need investigation and specific factors. Anti-coagulation therapy must be reduced or stopped as indicated by the need for therapy and the availability of the appropriate, previously depressed, Vitamin K dependent-clotting factors (II, VII, IX, X). Local measures, formerly the only treatment, are now of less importance. Some countries do not have these blood factors available.
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PMID:Blood components for oral surgery. 680 79

Described the impact of the repressive personality style on the measurement of psychological distress among children and adolescents with hemophilia. Two groups were compared on parent and self-report measures of anxiety and depression: a nondefensive group (n = 34) with low distress; and a highly defensive group (n = 26) who were identified as having a repressive personality style and who also reported low distress. Consistent with hypotheses, highly defensive children reported comparable levels of anxiety and lower levels of depression than nondefensive children. On the other hand, mothers of highly defensive children and adolescents described them as more distressed than mothers of nondefensive (self-assured) children. Findings underscore the importance of including data from other informants, (e.g., parents, teachers, or peers) to avoid misleading findings based on self-reports of anxiety and depression obtained from highly defensive children.
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PMID:Impact of the repressive personality style on the measurement of psychological distress in children and adolescents with chronic illness: an example from hemophilia. 892 Jan 58

The relationships among illness stress, perceived support, and child psychosocial adjustment were examined for children living with a chronically ill father. Participants included fathers, mothers, and one child from 53 families in which the father had hemophilia and, in some cases, was HIV seropositive. Objective indicators of severity of illness and subjective measures of the physical and psychological impact of illness were used as sources of children's stress. Results indicated that the impact of illness, but not the severity of illness itself, related to child psychosocial adjustment. Main effects were observed for parental support on child- and parent-reported internalizing problems and stress-buffering effects were obtained for parental support and extrafamilial support on parent-reported internalizing problems. Parental support also demonstrated a stress-buffering effect for child-reported depression. Assessment and intervention implications for behavioral clinicians and researchers are discussed.
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PMID:The role of parental and extrafamilial social support in the psychosocial adjustment of children with a chronically ill father. 933 99

This study outlines the development and evaluation of a structural equation model for establishing the consequences of haemophilia. The hereditary disorder is characterized by a high tendency to haemorrhages, with recurrent bleeding into the joints causing irreversible joint damage. The model is, in general, an attempt to answer the following questions: what is the effect of haemophilia on the well-being (i.e. satisfaction, health, somatic complaints and self-esteem) of patients and what is the additional or mediating role of other individual characteristics in this pathway? Disease severity, joint impairment and disability are defined as antecedents of well-being and the mediating roles of appraisal (i.e. the personal evaluation of the disease), health beliefs (i.e. locus of control), psychological characteristics (i.e. anxiety, anger, depression and optimism) and social support are investigated. Psychological variables turned out to be the strongest determinants of well-being and partly mediated the detrimental effect of disability on well-being. The role of appraisal remained somewhat unclear, as no significant relationship was established between this personal evaluation of haemophilia and well-being. Nevertheless, appraisal very well reflected the level of disability. An internal locus of control and favourable psychological characteristics appeared to reduce the perceived seriousness of haemophilia. No evidence was found for social support to act as a mediator between disability and well-being. The perception of support did show moderately strong associations with psychological characteristics (i.e. anxiety and depression) and satisfaction ratings. The study merits further research on quantifying the relationships between clinical parameters and psychosocial outcomes in patients with a chronic disease.
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PMID:Well-being of haemophilia patients: a model for direct and indirect effects of medical parameters on the physical and psychosocial functioning. 969 Aug 41

This study presents the final report of a long-term psychological assessment of men with haemophilia and HIV infection. The knowledge, emotional impact regarding HIV infection and prospective changes over time and the need for psychological support were evaluated. The study group comprised 118 men with haemophilia, 66 HIV seropositive and 52 seronegative, from the Haemophilia Centres in Bari, Florence, Milan and Naples. All subjects performed psychological tests (STAI: state and trait anxiety inventory; SDS: self-rating depression scale) and completed questionnaires to ascertain their knowledge and the emotional impact of AIDS. After enrollment (1992-93) the assessment was repeated twice over a 2-year period. A high percentage of subjects in both groups answered the questionnaire on knowledge correctly and, more specifically, all (100%) admitted knowing that sexual intercourse was a risk factor for HIV infection, adding that sexual partners of haemophiliacs with HIV should be regularly tested. The percentage, however, decreased for seropositives who admitted to always using a condom during sexual intercourse (86%) and for those who declared that partners were periodically tested for HIV (60%). The most important feature of the study is that, contrary to predicted expectations, seropositive and seronegative subjects presented the same degree of emotional involvement: there are no statistically significant differences in average scores between groups either on the anxiety or depression scales. Moreover, for certain aspects, seronegatives revealed greater emotional involvement: at baseline evaluation, they felt more fear and unhappiness with statistically significant differences compared to asymptomatics. Furthermore, seronegatives more than seropositives continue to feel reluctance towards infusion and avoid blood products after learning of AIDS. These results emphasize the importance of paying due attention to the emotional status of seronegatives. Their reluctance towards the use of blood products (despite present safety) is a very important issue for the possible consequences of treatment with the risk of worsening the clinical condition. In conclusion, we believe that counselling on HIV infection/AIDS needs to address every person with haemophilia regardless of HIV status.
Haemophilia 1998 Nov
PMID:Knowledge of HIV/AIDS and emotional adjustment in a cohort of men with haemophilia and HIV infection: final report. 1002 4


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