Cases reported "Lymphopenia"

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1/147. CD4 depletion in hiv-infected haemophilia patients is associated with rapid clearance of immune complex-coated CD4 lymphocytes.

    The predominant immunological finding in hiv haemophilia patients is a decrease of CD4 lymphocytes during progression of the disease. Depletion of CD4 lymphocytes is paralleled by an increase in the proportion of immune complex-coated CD4 cells. We examined the hypothesis that the formation of immune complexes on CD4 lymphocytes is followed by rapid clearance of immune complex-coated CD4 lymphocytes from the circulation. In this study, the relationship of relative to absolute numbers of immune complex-loaded CD4 blood lymphocytes and their association with viral load were studied. Two measurements of relative and absolute numbers of gp120-, IgG- and/or IgM-loaded CD4 lymphocytes were analysed in hiv and hiv- haemophilia patients, with a median interval of approx. 3 years. Immune complexes on CD4 lymphocytes were determined using double-fluorescence flow cytometry and whole blood samples. viral load was assessed using NASBA and Nuclisens kits. Whereas the proportion of immune complex-coated CD4 lymphocytes increased with progression of the disease, absolute numbers of immune complex-coated CD4 lymphocytes in the blood were consistently low. Relative increases of immune complex-coated CD4 blood lymphocytes were significantly associated with decreases of absolute numbers of circulating CD4 lymphocytes. The gp120 load on CD4 blood lymphocytes increased in parallel with the viral load in the blood. These results indicate that immune complex-coated CD4 lymphocytes are rapidly cleared from the circulation, suggesting that CD4 reactive autoantibodies and immune complexes are relevant factors in the pathogenesis of AIDS. Relative increases of immune complex-positive cells seem to be a consequence of both an increasing retroviral activity as well as a stronger loading with immune complexes of the reduced number of CD4 cells remaining during the process of CD4 depletion. The two mechanisms seem to enhance each other and contribute to the progressive CD4 decrease during the course of the disease. ( info)

2/147. Persistent Sweet's syndrome occurring in a child with a primary immunodeficiency.

    Sweet's syndrome (SS) occurs most commonly in association with inflammatory or neoplastic disorders. Only rarely has it been associated with immunodeficiency disorders. We describe a child with a T-cell immunodeficiency who had a persistent neutrophilic dermatosis that was histologically and clinically consistent with SS. SS associated with immunodeficiencies may occur as a reaction to an underlying infection or a defect in immunoregulation. Such patients, however, may not be able to produce the classic fever and neutrophilia associated with SS. They may fail to respond to standard treatment for SS and may suffer a prolonged and persistent course. ( info)

3/147. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis.

    A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed. ( info)

4/147. Treatment of idiopathic CD4 T lymphocytopenia with IL-2.

    Idiopathic CD4 T lymphocytopenia (ICL) is an unusual immune defect in which there is an unexplained deficit of CD4 T cells, leading to fungal, parasitic or other serious opportunistic infections. Current treatment efforts are directed at eliminating infections. Here we describe the use of a novel treatment, subcutaneous polyethylene glycol (PEG)-IL-2 injections, in a woman with this disorder, who had chronic severe mycobacterial disease which led to repeated hospitalizations, and advancing respiratory insufficiency. For this patient, PEG-IL-2, 50 000 U/m2, has been given by weekly subcutaneous injections for 5.5 years. This treatment has resulted in marked (and still continuing) long-term immunological improvement with normalized T cell functions and increased CD4 cell numbers. She has had substantial clinical improvement with clearing of mycobacterial disease, reducing hospitalizations and improved lung functions. The improvement seen in this patient suggests that low-dose IL-2 is a safe and practical therapy, which might be useful in other subjects with this potentially serious immune defect. ( info)

5/147. Failure of immunosuppression in a severe haemophilia B patient with specific antibody.

    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. ( info)

6/147. Schimke immuno-osseous dysplasia: case report and review of 25 patients.

    Immuno-osseous dysplasia is characterised by spondyloepiphyseal dysplasia, lymphopenia with defective cellular immunity, and progressive renal disease. We describe a patient with a severe form of the disease, review the features of another 24 patients, and discuss the previous classification. The differences between the two groups are not striking, and although similarities are greater between affected sibs, the same diagnosis of Schimke immuno-osseous dysplasia should apply to them all. The aetiology and physiopathology of this rare osteochondrodysplasia of presumed autosomal recessive inheritance remain unknown. ( info)

7/147. proteus syndrome and immunodeficiency.

    A 10 year old boy with proteus syndrome presented with a pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the proteus syndrome. ( info)

8/147. Localized Mycobacterium genavense soft tissue infection in an immunodeficient hiv-negative patient.

    An hiv-negative woman with chronic lymphopenia related to past sarcoidosis situated in the bone marrow presented with an inflammatory lesion in the iliac region due to a localized Mycobacterium genavense soft tissue infection. The lesion resolved after 12 months of antibiotic therapy with clarithromycin, ethambutol and ciprofloxacin. The patient had no recurrence of the subcutaneous abscess during a follow-up period of 14 months after the end of the treatment. ( info)

9/147. adult T cell leukemia/lymphoma with lymphopenia in a Korean.

    We experienced a case of adult T cell leukemia/lymphoma (ATLL) in a 48-year-old Korean female, who has never been abroad since birth and no history of blood transfusion. The patient had hypercalcemia and multiple lymphadenopathy. Histopathologic study of left cervical lymph node (LN) and bone marrow (BM) revealed that infiltrates of malignant lymphoid cells were composed of small, medium and large cells with pleomorphic nuclei. Smears of peripheral blood (PB) showed lymphopenia (16%) with the appearance of a few atypical lymphoid cells (less than 2%), but not the typical clover leaf cells seen in ATLL. Immunophenotypic study of LN and BM revealed T cell phenotype. PB showed increased CD4 T cell (T(H), CD3/CD4 , 57%) and decreased CD8 T cell counts (T(S), CD3/CD8 , 6.7%). The sera of the patient and her family were reactive for HTLV-I antibody. The specific sequences of pol, env, and tax of HTLV-I dna were detected in the lymphoma cells and peripheral blood mononuclear cells (PBMC) using polymerase chain reaction. Ultrastructural examination of PBMC confirmed numerous type c virus particles in extracellular space. This case was an acute type of ATLL without overt leukemic features in PB. Despite chemotherapy and intensive conservative treatment, she died 3 months after admission. ( info)

10/147. Strong alpha beta and gamma delta TCR response in a patient with disseminated mycobacterium avium infection and lack of NK cells and monocytopenia.

    Infection with atypical mycobacteria occurs mainly in patients with a compromised cellular immune system, in particular in those with a defective T cell or monocyte function. Here we analyzed the specific immune response of an adolescent hiv-negative patient with disseminated mycobacterium avium infection and fatal varizella zoster virus infection. The patient presented with dysplastic hematopoesis of all cell lineage's and a bicytopenia of erythrocytes and leukocytes, but a hematological malignancy could not be found. We found a peripheral lymphopenia and monocytopenia, as well as a lack of NK-cells and B-cells. lymphocytes consisted of 95% T cells, which contained up to 40% of TCR gammadelta CD4-CD8-T-cells (mainly TCR gamma9delta2), few monocytes and B-cells. Approximately 50% of CD3 T-cells showed a CD57 NK-like phenotype. Functional analysis of PBMC revealed a good antigen-specific T cell function if antigen-presenting cells were supplemented from a HLA-matched donor. Moreover, a strong M. avium specific cytotoxicity mediated by TCR alphabeta T-cells could be found in vitro and even ex vivo. In contrast, NK-killing was absent. No evidence for a defect in IL-12 or IFN-gamma production and signaling were found. The data indicate that a strong alphabeta and gammadelta T cell immunity tries to compensate for a deficient monocyte and NK cell function in this patient. ( info)
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