Cases reported "Hemophilia B"

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1/3. Clinical, immunological, and virological aspects in Japanese hemophiliacs and AIDS patients.

    To date, 16 cases of AIDS have been officially reported in japan: 8 of them were hemophiliacs and the others were male homosexuals. We had two hemophiliacs with AIDS, and describe these cases in this paper. One was a 48-year-old man with hemophilia b and the other was a 62-year-old man with hemophilia a. Both had ARC symptoms before developing overt and fatal opportunistic infections, candidiasis and a combination of atypical mycobacteriosis and aspergillosis. Impairment of cellular immunity such as lowered T4/T8 ratio, depressed lymphocyte response to mitogen and NK activity, and negative PPD skin reaction was evident in both patients. Anti-LAV/HTLV-III antibodies were detected in their sera, and the retrovirus was demonstrated in the lymph nodes and other organs on the postmortem electron microscopic examination. The assay for anti-LAV/HTLV-III on 70 other hemophiliacs in our clinic revealed seropositivity up to 50%. The seropositive group showed a significantly lower T4/T8 ratio on an average than the seronegative group. The prevalence of seropositivity for antibodies to other viruses including HBV, EBV, CMV, PV, and HTLV-I was markedly higher in the hemophiliacs. In LAV/HTLV-III seropositive hemophiliacs, however, no distinct correlation was found between T4/T8 ratio and the presence of antibodies to other viruses. There is a retrospective study to show that LAV/HTLV-III seropositive hemophiliacs appeared as early as 1980 in japan. For prevention of LAV/HTLV-infection in hemophiliacs via blood products, heat-treated factor concentrates have recently become available. In a trial of the heat-treated products on 15 "virgin" hemophiliacs, no seroconversion has occurred thus far.
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keywords = retrovirus
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2/3. Isolation of new lymphotropic retrovirus from two siblings with haemophilia B, one with AIDS.

    A human T-lymphotropic retrovirus was isolated from cultured T lymphocytes from two siblings with haemophilia B. Patient 2 was healthy, but patient 1 had acquired immunodeficiency syndrome. The retrovirus differed from human T-cell leukaemia virus (HTLV) but it was similar to the lymphadenopathy-associated retrovirus (LAV) in its morphology and its major core protein (P25). Both patients had antibodies against LAV and patient 1's retrovirus, detected by an enzyme-linked immunosorbent assay or a radioimmunoprecipitation assay. Seroepidemiological data indicated the transmission of this retrovirus by plasma products.
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ranking = 9
keywords = retrovirus
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3/3. Implantation of autologous skin fibroblast genetically modified to secrete clotting factor ix partially corrects the hemorrhagic tendencies in two hemophilia b patients.

    OBJECTIVE: To examine the safety and effects of gene therapy for hemophilia b by implantation of autologous fibroblasts genetically modified to secrete clotting factor ix (hFIX). patients AND methods: Two hemophilia b patients LD and LW were selected from one family to accept gene transfer study. The hFIX protein of both patients were about 100 ng/ml plasma and hFIX activity was about 2%. The autologous skin fibroblasts of the two patients were genetically modified by retrovirus-mediated gene transfer with XL-IX and N2CMVIX vector (HBSF-IX). Human hFIX protein was measured by ELISA, hFIX activity was measured by one-stage clotting assay and barium citrate sorbent method. hFIX inhibitor was assayed by Bethesda methods. Human hFIX cDNA was detected by PCR. HBSF-IX cells were mixed with collagen for injection after safety assessments. RESULTS: The HBSF-IX cells from the two patients secreted hFIX at high levels in vitro. After implantation of autologous HBSF-IX cells, no treatment-related side effects were observed. plasma hFIX protein in both patients increased over 2 folds after several injections of HBSF-IX cells and persisted for more than 420 days. blood clotting activity increased significantly in both patients, hemorrhagic tendencies have been partially corrected after treatment. Further elevation of hFIX can be achieved by repeating the same treatment 420 days later in Patient LD. CONCLUSIONS: Implantation of autologous fibroblast genetically modified to secrete human hFIX offers a simple, safe and effective approach to gene therapy of hemophilia b.
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ranking = 1
keywords = retrovirus
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