Filter by keywords:

Retrieving documents. Please wait...

1/60. Idiopathic CD4 T lymphocytopenia disclosed by the onset of empyema thoracis.

    A 56-year-old man was admitted to our hospital in December 1996 due to empyema thoracis. A laboratory examination revealed lymphocytopenia and CD4 T lymphocytopenia (<300 cells/ microl). No evidence for a human immunodeficiency virus (HIV) infection was found. No malignant, hematological or autoimmune disease was detected. We thus diagnosed this case as being idiopathic CD4 T lymphocytopenia (ICL). During his hospital treatment, he was affected with cytomegaloviral retinitis and cured by therapy. His subsequent treatment went well without a recurrence of severe infection although a low CD4 T lymphocyte count continued after the recovery from empyema thoracis. ( info)

2/60. Cutaneous infections by papillomavirus, herpes zoster and candida albicans as the only manifestation of idiopathic CD4 T lymphocytopenia.

    BACKGROUND: Selective depletion of CD4 T lymphocytes is common in both primary and secondary immunodeficiencies. Idiopathic CD4 T lymphocytopenia (ICL) cases are defined as a persistent CD4 T lymphocyte count of less than 300x10(6) cells/L and/or less than 20% of the total T-cell count. METHOD: A 40-year-old woman, with a history of psoriasis and paracetamol allergy, presented with persistent warts of the hands and condylomas of the ano-genitalia. Histological and virological analysis was carried out on genital and cutaneous lesions and peripheral blood. RESULTS: serology for hiv-1, hiv-2, Epstein-Barr virus and parvovirus B19 were negative. There was lymphopenia of 10% CD4 cells, with normal numbers of total leukocytes; there were no other-abnormal immunological findings. dna analysis of cutaneous lesions revealed HPV-49 and HPV-3 in the hands and HPV-6 in the genital region. CONCLUSIONS: The cause of the ICL in this patient is unknown. HPV is not known to be an immunosuppressive agent; it remains to be determined whether the HPV-associated lesions are the cause or the result of immunosuppression. ( info)

3/60. Active intestinal tuberculosis with esophageal candidiasis due to idiopathic CD4( ) T-lymphocytopenia in an elderly woman.

    We describe a case of intestinal tuberculosis and esophageal candidiasis in an 85-year-old Japanese woman with idiopathic CD4 T-lymphocytopenia (ICL). The patient exhibited clinical symptoms of odynophagia, bloody diarrhea, and high fever. physical examination on admission showed a poor nutritional status. Endoscopic examination of the upper digestive tract revealed the esophageal mucosa to be covered with yellowish-white plaque-like lesions. Colonoscopic examination revealed multiple annular ulcerations with bleeding. She was diagnosed with intestinal tuberculosis by polymerase chain reaction (PCR) and fecal culture. Her CD4 T-lymphocyte count was 178/mm3 and no evidence of human immunodeficiency virus (HIV) infection was found. She was successfully treated with fluconazole and antituberculosis drugs. This case emphasizes the importance of opportunistic infections in elderly patients with predisposing conditions such as ICL. ( info)

4/60. Defective p56Lck activity in T cells from an adult patient with idiopathic CD4 lymphocytopenia.

    Idiopathic CD4( ) lymphocytopenia (ICL) is defined by a stable loss of CD4( ) T cells in the absence of any known cause of immune deficiency. This syndrome is still of undetermined origin. It affects adult patients, some of them displaying opportunistic infections similar to HIV-infected subjects. The hypothesis that the cellular immune defect may be due to biochemical failures of the CD3-TCR pathway is investigated here in a patient associating a severe selective CD4( ) lymphocytopenia with an increased CD8( ) T cell count discovered in the course of a cryptococcal meningitidis. A 40% reduction of T cell proliferation to CD3-TCR stimulation is observed only in the CD4( ) subpopulation. The early CD3-induced protein tyrosine phosphorylations are conserved in both CD4( ) and CD8( ) subsets, and the levels of the T cell protein tyrosine kinases p56(Lck), p59(Fyn) and ZAP-70 are normal. However, we find a 50% reduction of p56(Lck) kinase activity in the patient's T cells compared to a healthy control donor. p59(Fyn) activity does not appear to be altered. Nevertheless, we do not find any genetic abnormality of p56(Lck). These results thus suggest that a defect of an unknown protein regulating p56(Lck) activity takes place in this patient's T cells. Taken together, these findings reveal p56(Lck) alteration in ICL and confirm the critical role of this kinase in the maintenance of the peripheral CD4( ) T cell subpopulation. ( info)

5/60. Characterization of gammadelta T cells expressing CD158b, a killer cell inhibitory receptor, in a patient with chronic CD4( ) lymphocytopenia and disseminated mycobacterium intracellulare infection.

    A population of Vdelta1( )Vgamma9(-) gammadelta T cells that represented almost the totality (84%) of circulating lymphocytes in a patient with chronic, non-HIV-related, CD4 lymphocytopenia complicated by a disseminated mycobacterium intracellulare infection was characterized. These gammadelta( ) T cells expressed a single killer inhibitory receptor (CD158b) and their phenotype (CD8( )CD57( )CD27(-)CD28(-)) indicated that, although CD45RA( ), they were not naive. However, the absence of large granular lymphocyte morphology, the impaired proliferative activity, the high susceptibility to apoptosis, and the total lack of cytotoxic ability suggested that these gammadelta cells were in a resting state. A high percentage of the cells did not harbor the CD11b integrin alpha chain and exhibited a decreased capability to bind endothelial cells. This defect might represent the mechanism whereby they remained trapped in the circulation. ( info)

6/60. Retinopathy following measles, mumps, and rubella vaccination in an immuno-incompetent girl.

    We describe a 4-year-old girl with subnormal visual acuity due to a bilateral retinopathy. The child had a history of encephalitis following MMR vaccination. Temporary retinopathy associated with measles, mumps, and rubella (MMR) vaccination has been described. Recently an idiopathic CD4 T lymphocytopenia in the child was diagnosed. This cellular immunodeficiency supports our hypothesis of measles retinopathy after vaccination of an immuno-deficient child. ( info)

7/60. Restriction of T-cell receptor repertoires in idiopathic CD4 lymphocytopenia.

    We report that alpha/beta and gamma/delta T-cell repertoires of three patients with idiopathic CD4 lymphocytopenia, who showed different clinical manifestations and outcomes over time, were highly restricted. The disruption of T-cell repertoires does not influence the susceptibility to infections: the first patient was unable to attain a protective response to mycobacterium, the second showed clinical improvement and the third did not develop opportunistic infections. These results indicate that idiopathic CD4 lymphocytopenia could give rise to mono-/oligoclonal T-cell expansions, but the degree of repertoire disturbance is not indicative of the severity of disease progression. ( info)

8/60. Successful IL-2 therapy for relapsing herpes zoster infection in a patient with idiopathic CD4 T lymphocytopenia.

    Idiopathic CD4 T lymphocytopenia (ICL) has been defined by the center of disease control as a rare cause of immunodeficiency with a variable clinical course and an unknown aetiology. Here we describe a 65-year old patient with relapsing generalized herpes zoster infection due to ICL and a severe panlymphocytopenia. in vitro assays revealed an enhanced activation of CD8 T cells and an increased sensitivity of activated CD4 T cells for cell death. The clinical outcome was substantially improved after starting the patient on a subcutaneous therapy with IL-2. ( info)

9/60. Progressive multifocal leukoencephalopathy and idiopathic CD4 lymphocytopenia: a case report and review of reported cases.

    Progressive multifocal leukoencephalopathy (PML) is a well recognized demyelinating neurological disorder caused by jc virus. Idiopathic CD4( ) lymphocytopenia (ICL) is a syndrome first described by the Centers for Disease Control and Prevention as a CD4( ) count <300 cells/mm(3) or a CD4( ) count that is <20% of the total T cell count on 2 occasions, with no evidence of human immunodeficiency virus (HIV) infection on testing, and absence of any defined immunodeficiency or therapy that depresses the levels of CD4( ) T cells. To the best of our knowledge, this is the third reported case of PML and ICL, and also the first reported case of the use of cidofovir to treat PML in a patient not infected with human immunodeficiency virus. ( info)

10/60. Idiopathic CD4 T cell lymphocytopenia evolving to monoclonal immunoglobulins and progressive renal damage responsive to IL-2 therapy.

    Idiopathic CD4 T cell lymphocytopenia was unexpectedly detected in a 33-year-old, otherwise healthy young woman with no HIV or other viral infection, autoimmune, or neoplastic disease or increased susceptibility to infection. CD4 T cell levels were 60-140/microl over a 3.5-year period. Following an uneventful pregnancy, the patient developed anemia and interstitial nephritis associated with a plasma cell dyscrasia with a monoclonal IgA gammopathy and a shifting immunoglobulin pattern that included IgG and IgA monoclonal proteins and increased urinary light chains. Osteolytic lesions were never detected and bone marrow aspirations revealed up to 10% atypical plasma cells. Various therapies often used in treating multiple myeloma only temporarily controlled the increasing renal damage. IL-2 therapy of 600,000 to 1 million units subcutaneously daily resulted in increased CD4 T cells to normal levels, a decrease in the gammopathy, a return of renal function, energy, and weight gain, and apparently normal health status sustained for 2 years. The findings are compatible with a potentially fatal but nonmalignant immunoregulatory disorder that can be controlled by IL-2 administration. ( info)
| Next ->

We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.