Cases reported "Chronic Disease"

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1/9. 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.
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keywords = apoptosis
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2/9. Decreased natural killer (NK) cell function in chronic NK cell lymphocytosis associated with decreased surface expression of CD11b.

    Chronic natural killer cell lymphocytosis (CNKL) is characterized by greatly increased numbers of natural killer (NK) cells and patients with this disease may survive for long periods. This is in contrast to patients with leukemic proliferations of NK cells who can have a rapidly progressive clinical course. We identified a pediatric patient who was largely healthy who had CNKL and we sought to determine if the expanded CD16( )CD3(-) population in this patient functions differently than classical NK cells. Cytotoxic activity against NK cell-sensitive K562 target cells was present, but lower than that in control donors when calculated as lytic units per CD16( )CD3(-) cell. This cytolytic activity was inducible in patient samples by IL-2/IL-12 stimulation proportionately to that induced in samples from control donors. Intracellular perforin was also present and induced in patient CD16( )CD3(-) cells similarly to controls. Other presumed NK cell activities, such as IL-2/IL-12 induced IFN-gamma expression and initiation of apoptosis evidenced by annexin V binding after CD16 crosslinking were present in patient samples. Patient CD16( )CD3(-) cells, however, differed from classical NK cells, as the majority did not express CD56, CD57, CD8, or CD11b. Most convincingly, there was a 5 log decrease in CD11b expression in patient CD16( )CD3(-) cells compared to control as determined by mean channel fluorescence. These observed differences may explain the relatively benign phenotype of this disorder.
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keywords = apoptosis
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3/9. Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes.

    Chronic infantile neurological cutaneous and articular (CINCA) syndrome is a severe chronic inflammatory disease of early onset, characterized by cutaneous symptoms, central-nervous-system involvement, and arthropathy. In the present study, we report, in seven unrelated patients with CINCA syndrome, distinct missense mutations within the nucleotide-binding site of CIAS1, a gene encoding cryopyrin and previously shown to cause Muckle-Wells syndrome and familial cold urticaria. Because of the severe cartilage overgrowth observed in some patients with CINCA syndrome and the implications of polymorphonuclear cell infiltration in the cutaneous and neurological manifestations of this syndrome, the tissue-specific expression of CIAS1 was evaluated. A high level of expression of CIAS1 was found to be restricted to polymorphonuclear cells and chondrocytes. These findings demonstrate that CIAS1 missense mutations can result in distinct phenotypes with only a few overlapping symptoms and suggest that this gene may function as a potential inducer of apoptosis.
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ranking = 1
keywords = apoptosis
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4/9. Apoptotic colon disease: a new clinico-pathological entity.

    A patient with intractable diarrhea showed, in 6 out of 7 biopsies taken at various colonic levels, a rich number of apoptotic granules at the base of the colonic crypts. Those apoptotic granules stained positive with Feulgen, a reaction that stains nuclear dna. The nuclei of the cells at the base of the crypts stained positive with TUNEL, a method that labels nuclei undergoing apoptosis. No associated inflammatory reaction was present in the biopsies. As no other explanation was found for the phenomenon, the possibility that marked cryptal apoptosis was the cause of the untreatable protracted diarrhea was entertained. A similar histological finding had previously been recorded in a non-human primate dying of intractable diarrhea. That phenomenon was designated apoptotic colonic disease. We propose a similar denomination for this previously unreported finding in a human subject.
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ranking = 2
keywords = apoptosis
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5/9. autoimmune lymphoproliferative syndrome: a cause of chronic splenomegaly, lymphadenopathy, and cytopenias in children-report on diagnosis and management of five patients.

    autoimmune lymphoproliferative syndrome (ALPS) usually manifests in early childhood with splenomegaly, lymphadenopathy, and cytopenias. In most patients, it results from mutations in genes that regulate lymphocyte apoptosis via the Fas pathway. Here, we report five children with ALPS. All five children had splenomegaly, cytopenias, and hypertriglyceridemia at presentation; four had lymphadenopathy. Mutations in the Fas receptor gene were demonstrated in three children. Clinical picture is variable: in only one child manifestations are severe enough to require immunosuppressive therapy. diagnosis of ALPS can be challenging and increased awareness of the disease can result in more directed diagnostic approaches as well as earlier initiation of treatment.
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ranking = 1
keywords = apoptosis
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6/9. Increased chemoattractant induced neutrophil oxidative burst, accelerated apoptosis, and dysregulated tyrosine phosphorylation associated with lifelong bacterial infections.

    A boy with lifelong recurrent bacterial infection at cutaneous and mucosal sites was investigated. PMN oxidative burst to phorbol myristate acetate (PMA) and zymosan was normal but was increased 20- to 50-fold upon C5a or formyl-met-leu-phe (fMLP) chemoattractant stimulation, accompanied by accelerated PMN apoptosis. His PMNs showed increased constitutive tyrosine phosphorylation of 21-, 25-, and 44-kDa proteins, and of src-family kinases (p59(hck), p58(fgr), and p53/56(lyn)). phosphorylation was abnormally enhanced following fMLP stimulation. Expression and activity of the major PMN tyrosine phosphatases, i.e., CD45, CD148, and SHP-1 and -2, was normal. However, dephosphorylation of phospho-p58(fgr) and phospho-p53/56(lyn) by lysates of patient's PMNs was enhanced. Thus, another phosphatase may be overactive, perhaps dephosphorylating a regulatory (inhibitory) site on a protein tyrosine kinase, accounting for the abnormal PMN tyrosine phosphorylation and function. With age (now 13 years), T-cell lymphopenia and loss of T-cell responses developed. This appears to be a unique primary immunodeficiency with abnormal PMN oxidative and apoptotic responses to chemoattractants, dysregulated protein tyrosine phosphorylation, serious bacterial infection, and T-lymphocyte attrition.
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ranking = 5
keywords = apoptosis
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7/9. parvovirus B19 infection associated with unilateral cervical lymphadenopathy, apoptotic sinus histiocytosis, and prolonged fatigue.

    This report describes the case of a 16 year old girl with a history of high fever, prolonged fatigue, and cervical lymphadenopathy of the right side. In addition, the patient showed neutropenia, thrombopenia, and pronounced reticulopenia. Cervical ultrasound showed unilateral hypoechoic lymph nodes up to 23 mm in diameter suspicious for malignant lymphoma. histology of a cervical lymph node specimen revealed massive nodular histiocytic proliferation and prominent apoptosis without necrosis. parvovirus B19 was detected by polymerase chain reaction and immunohistochemistry in the lymph node. In summary, this case is an unusual presentation of parvovirus B19 infection. The virus was identified as the potential causative agent of unilateral cervical lymphoma and apoptotic sinus histocytosis, thus broadening the clinicopathological spectrum of parvovirus B19 induced diseases.
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ranking = 1
keywords = apoptosis
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8/9. apoptosis in perforated cornea of a patient with graft-versus-host disease.

    CASE REPORT: Although ocular complications associated with graft-versus-host disease (GVHD) can include corneal dysfunction, corneal perforation is not common. We report the presence of apoptotic cells in a perforated cornea of a patient with GVHD. A 72-year-old man with the angioimmunoblastic type of malignant lymphoma developed chronic GVHD after allogeneic peripheral blood stem cell transplantation. Despite systemic and topical treatment, both corneas perforated, and penetrating keratoplasty with cataract extraction and intraocular lens implantation was performed on both eyes. COMMENTS: The corneal button excised from the right eye was examined histologically and stained for apoptotic cells by TdT-mediated dUTP nick end labeling (TUNEL). This revealed thinning of the epithelial cell layer and stroma, with cells, including lymphocytes, infiltrating to the site of the perforation. Some of the epithelial cells and keratocytes were TUNEL positive. The presence of apoptotic cells in our case suggests that apoptosis may be involved in the perforation of the cornea in patients with GVHD.
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ranking = 1
keywords = apoptosis
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9/9. Expression of apoptosis-related antigen on eosinophils in chronic eosinophilic pneumonia.

    We examined the expression of apoptosis-related antigens Fas and bcl-2 on eosinophils from peripheral blood (PB) and bronchoalveolar lavage (BAL) in patients with chronic eosinophilic pneumonia (CEP). The expression of those antigens was assessed before and after culture with or without eosinophil chemotactic factors derived from an established T-cell line (STO-2-derived ECFs; ECF-PI5, 6, 7, 8, and 9), granulocyte-macrophage colony stimulating factor, and interleukin 5 (IL-5). We found that the expression of these antigens on eosinophils from PB increased after 24 h culture without any stimulation. In contrast, little or no change was observed even after 24 h culture in eosinophils from BAL. All STO2-derived ECFs and IL-5 suppressed Fas expression on eosinophils from PB. Furthermore, we found that eosinophils which were attracted by ECF-PI9 expressed Fas and bcl-2 more highly than those attracted by other ECFs and IL-5. Such a heterogeneous response of eosinophils to respective ECFs suggests the possibility of a heterogeneous population of eosinophils in patients with CEP.
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ranking = 5
keywords = apoptosis
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