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1/6. mycetoma due to exophiala jeanselmei and mycobacterium chelonae in a 73-year-old man with idiopathic CD4 T lymphocytopenia.

    exophiala jeanselmei and mycobacterium chelonae were isolated from cutaneous nodules in a 73-year-old man with mycetoma of the right lower leg. Further evaluation revealed CD4 lymphocytopenia without evidence of HIV infection. Antibodies to HIV 1/2, p24 antigen and HIV 1/2 (PCR) and reverse transcriptase activity were not detectable. The patient was not a member of any HIV risk group. He had not previously undergone therapy or suffered from immunodeficiency. This case clearly demonstrates that infections with opportunistic moulds and/or atypical mycobacteria should be taken into consideration not only in patients with classical immundeficiency diseases but also in apparently healthy patients because infection with these agents can be the first sign of underlying immunodeficiency.
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2/6. CD4 T-lymphocytopenia without HIV infection: increased prevalence among patients with primary sjogren's syndrome.

    OBJECTIVE. Primary sjogren's syndrome (1 degree SS) is an autoimmune disease, usually accompanied by manifest immune hyperactivity. In some cases the disease converts to malignant neoplasia. On the other hand, there are clinical similarities to HIV infection. Since the rare phenomenon of persistent depletion of CD4 t-lymphocytes in peripheral blood without HIV infection was recently defined as idiopathic CD4 T-lymphocytopenia (ICL), we have used the ICL criteria to investigate the prevalence of this phenomenon among 1 degree SS patients. methods. During the period 1988-94, 115 caucasian patients (10 males), mean age 57.8 (range 19-82) years, with 1 degree SS were prospectively studied. lymphocyte subsets were investigated by means of monoclonal antibodies and flow cytometry. For the detection of HIV and HTLV antibodies, we used an enzyme immunoassay (for hiv-1 and hiv-2), Western blot techniques (hiv-1, hiv-2, HTLV-I and HTLV-II), and the polymerase chain reaction procedure (hiv-1, HTLV-I and HTLV-II). hiv antigens were tested for with the hiv-1 p-24 Ag test. RESULTS. Six patients with 1 degree SS fulfilled the criteria for ICL. While the clinical condition of 5 of those six patients remained stable, one patient developed malignant lymphoma three years after her disease was classified as a case of ICL. The prevalence of ICL among our 115 patients with 1 degree SS was 5.2%, which is significantly higher than the rates reported for any other patient or population group. We have estimated the relative risk of ICL in 1 degree SS patients to vary from 3.4 to 6,000 (P values of 0.0001-0.025). CONCLUSION. We suggest that subjects with ICL should be carefully examined for 1 degree SS and, if its presence is confirmed, that they should be followed with regard to the possible complications of this disease, including the development of malignant lymphoma.
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3/6. Antibodies against retroviral proteins and nuclear antigens in a subset of idiopathic CD4 T lymphocytopenia patients.

    Idiopathic CD4 T lymphocytopenia (ICL) is an immunodeficiency syndrome characterized by severe depletion of CD4 T lymphocytes, but in which human immunodeficiency virus cannot be detected. Peripheral blood mononuclear cells (BPMCs) from an ICL patient were cocultured with HUT78 T-lymphoblastoid cells, and an acute cytopathic effect and formation of multinucleated cells were observed. A human intracisternal A-type retroviral particle designated HIAP-II was detected in cells surviving the acute cytopathic effect. Eight of 13 ICL patients in a blinded screen of a serological panel provided by the National Centers for Disease Control and Prevention (CDC) had serum antibodies that specifically reacted with HIAP-II associated proteins by Western immunoblotting. None of 19 control sera in the panel that were unreactive with HIV Gag proteins produced a positive result on HIAP-II immunoblots. Comparable results were obtained in a blinded screen of a second CDC serological panel. Sera from 8 of 14 ICL patients in the second serological panel were positive for antinuclear autoantibodies (ANAs) commonly observed in patients with systemic autoimmune diseases. These results suggest the possible involvement of an A-type retrovirus or autoimmunity in development of ICL in a subset of patients.
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4/6. Behcet's-like syndrome associated with idiopathic CD4 T-lymphocytopenia, opportunistic infections, and a large population of TCR alpha beta CD4- CD8- T cells.

    Herein we report a patient with Behcet's like syndrome, idiopathic CD4 T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta CD4- CD8- T cells. Microfluorimetric analysis of peripheral blood mononuclear cells revealed CD4 T-cell counts of 10 /- 5/mm3. The CD3 T cells were 99% TCR alpha beta , of which 74 /- 5% were CD4- CD8-. No clonal populations were detected by southern analysis for T-cell receptor V beta gene rearrangements. No evidence of human immunodeficiency virus infection was present, although nocardia, candida, pneumocystis, cytomegalovirus, and herpes infections were documented. The concomitant presence of opportunistic infections and a large population of TCR alpha beta CD4- CD8- T cells suggests a pathogenic association and an intense immune response to microbial lipid or lipoglycan antigens presented in the context of CD1 molecules. This case demonstrates the potential for idiopathic CD4 T-lymphocytopenia to occur in Behcet's-like syndrome with lethal consequences.
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5/6. Active psoriasis and profound CD4 lymphocytopenia.

    We report the case of a patient with a long-standing history of widespread chronic plaque psoriasis, who was recently found to have a profound CD4 lymphocytopenia. He is human immunodeficiency virus (HIV) negative. His psoriasis remains active and widespread, and he has had 60 cutaneous malignancies, including many squamous cell carcinomas, excised over the last 10 years. In the past he has had numerous cutaneous viral warts. Despite a low peripheral blood CD4 T-cell count, similar numbers of activated T cells, identified by double labelling for CD4 and hla-dr antigens, were found in the epidermis of our patient as other individuals with psoriasis. Thus, there appear to be sufficient activated CD4 T cells in our patient's psoriatic plaques to maintain the psoriatic process.
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6/6. Extensive ulcerative colitis and extraintestinal manifestations in a patient with HIV infection and significant CD4 T-cell lymphopenia.

    We report a heterosexual patient with HIV infection and a CD4 T-cell count of 0.45 x 10(9)/L who developed mild ulcerative proctitis, sacroileitis and oligoarthiritis. While he was treated with 5-aminosalicylic enemas, the patient rapidly developed severe pancolitis. An emergency colectomy without procetectomy was performed. A few months later, he suffered recurrence of ulcerative proctitis, aggravation of arthritic pain and developed anterior uveitis. All symptoms disappeared after proctectomy. There was no evidence for opportunistic infection or Kaposi's sarcoma. Antineutrophil cytoplasmic antibodies were positive and the hla-b27 antigen was present. CD4 counts were lower during the phases of active disease than during remission. This case demonstrates that severe ulcerative colitis can occur in the presence of moderate T-cell defects. In view of a recent report of remission of Crohn's disease under comparable circumstances, it is possible that the extent of T-cell involvement in both diseases is radically different.
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