1/13. Reversible monoclonal lymphadenopathy in autoimmune lymphoproliferative syndrome with functional FAS (CD95/APO-1) deficiency.The FAS (CD95/APO-1) receptor and its ligand play an important role in the initiation of apoptosis under many physiologic conditions. Loss of function mutations of the FAS gene have been described in lpr mice and in humans with autoimmune phenomena, recurrent lymphadenopathies, and hepatosplenomegaly. This syndrome is now called autoimmune lymphoproliferative syndrome type I (ALPS I). Recently, patients with similar clinical symptoms due to a functional FAS deficiency without FAS gene mutations have been distinguished. This disease has been termed autoimmune lymphoproliferative syndrome type II (ALPS II) or autoimmune lymphoproliferative disease (ALD). This report is the first description of the lymph node pathology and immunohistochemistry in a patient with ALPS II. After recurrent bacterial infections, a 4-year-old child developed cervical giant lymphadenopathy suggesting lymphoma. Lymph node histology resembled the findings in Epstein Barr virus-associated posttransplant atypical lymphoproliferations. Confluent sheets of immunoblasts, however, showed a monoclonal expression of IgG/lambda and a monoclonal rearrangement of the JH chain. The same clone was also present in the peripheral blood. Although high-grade lymphoma could not be excluded, the patient's parents insisted on the patient's leaving the hospital with only antibiotic treatment. Surprisingly, the giant lymphadenopathy completely resolved within 7 weeks, and the clone was no longer detectable in the peripheral blood. Twelve months later the patient was still free from lymphoma and was doing well. Retrospectively, transient monoclonal B-cell populations could be identified in an archival frozen blood sample taken when the patient was 3 years old. Increased FAS-independent spontaneous apoptosis was a feature of the patient's lymphocytes and could be the molecular basis for self-elimination of B-cell clones. We conclude that the diagnosis of a FAS-FAS-L deficiency should be considered in children with an otherwise unexplained atypical lymphoproliferation and that a diagnosis of lymphoma in patients with functional FAS deficiency should be made with considerable reservation.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
2/13. autoimmune lymphoproliferative syndrome associated with severe humoral immunodeficiency and monoclonal gammopathy.A boy of Caucasian origin with a new subtype of autoimmune lymphoproliferative syndrome (ALPS) is described. The clinical picture was dominated by chronic noninfectious lymphadenopathy, splenomegaly, and recurrent bacterial infections. At the age of 6 the patient died of pneumococcal meningitis. Laboratory investigation disclosed impaired apoptosis in both B- and t-lymphocyte subsets and expanded populations of CD3 CD4-CD8- T lymphocytes. Furthermore, marked dysregulation of humoral immune responses with transient expansion of monoclonal B cells, corresponding monoclonal gammopathy, and the presence of autoantibodies was found. Functional and molecular analysis revealed that Fas protein expression was normal, a mutation in the Fas gene was not found. Moreover, transcription of the downstream effector caspase-10 was unremarkable. This patient is unique compared to previously described patients as severe humoral immunodeficiency and monoclonal gammopathy are usually not described in patients with ALPS. This case points out the important role of apoptosis in regulating the degree of humoral immune responses at a clonal level in humans and gives further evidence for the phenotypic diversity of ALPS.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
3/13. A new disorder of lymphocyte apoptosis: combination of autoimmunity, infectious lymphadenopathy, double negative T cells, and impaired activation-induced cell death.A new symptom-complex is described characterized by manifestations of autoimmune disease, infectious lymphadenopathy, double negative T cells, and impaired activation-induced cell death that developed in late adolescence. Similarities, but also significant differences, to autoimmune lymphoproliferative syndromes (ALPS, Canale-Smith syndrome) and autoimmune lymphoproliferative disease (ALD, Dianzani syndrome), were observed. The main clinical features were recurrent bacterial infections with subsequent lymphadenopathy due to autoimmune neutropenia. Laboratory results revealed a large proportion of alphabetaTCR positive, CD4 negative, CD8 negative, peripheral T cells, and a decreased apoptosis upon activation with phytohemagglutinin and interleukin 2, but normal Fas-mediated apoptosis. Genetic investigations excluded mutations in Fas gene death domain and in the 4 exons of Fas ligand gene. Despite unknown pathogenesis, this new syndrome might belong to the growing group of diseases with defects in apoptosis.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
4/13. Systemic lupus erythematosus-like autoimmune abnormalities induced by bacterial infection.Recent evidence has revealed that bacterial dna can promote several of the autoimmune abnormalities observed in systemic lupus erythematosus (SLE), and a possible pathogenic role in the induction of SLE has been highlighted. We have recently encountered patients in whom bacterial infection (septicemia) triggered the production of several autoantibodies. This seems to be interesting with respect to the consideration of the relationship between SLE and bacterial infection.- - - - - - - - - - ranking = 6keywords = bacterial infection (Clic here for more details about this article) |
5/13. Sudden sensorineural hearing loss: a case report supporting the immunologic theory.Sudden sensorineural hearing loss (SSNHL) is one of the autoimmune diseases of the inner ear (AIED), which is characterized by a hearing loss of above 30 decibels in at least three contiguous audiometric frequencies over a time course of 72 hours or shorter. Its cause can be found in only 10% to 15% of patients. Histopathologic findings have reported retrograde neuronal degeneration and atrophy of Corti's organ and of the vascular stria. This paper describes a case of a middle-aged female patient undergoing a treatment for hyperthyroidism who developed bilateral SSNHL. The patient was treated with methylprednisone (1 mg/kg/day) for three days with considerable hearing improvement. This treatment was followed by lung and kidney tuberculosis. The immune mechanism of this entity and the possibility of interconnected participation of the antigen type, of an autoimmune disease and of bacterial infection are discussed.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/13. Autoimmune neutropenia of infancy with multiple brain abscesses during the course of human herpesvirus-6 infection.Autoimmune neutropenia of infancy is characterized by recurrent infections such as pneumonia, otitis media, impetigo, purulent skin regions, gastritis, and upper respiratory infection. However, severe bacterial infection is uncommon. This report documents a 9-month-old boy presenting with autoimmune neutropenia in association with multiple brain abscesses during the course of human herpesvirus (HHV)-6 infection. HHV-6 has a tendency of neurovirulence, which can destroy the blood-brain barrier and facilitate the easy invasion of agents inside the brain. Although autoimmune neutropenia of infancy is benign and self limiting, it must be emphasized that severe bacterial infection will be induced by concurrent viral infection in this specific disorder.- - - - - - - - - - ranking = 2keywords = bacterial infection (Clic here for more details about this article) |
7/13. Analysis of antibody to neutrophils associated with autoimmune neutropenia: possible recognition of Fc-receptor-related molecules.The serum of a 50-year-old male with neutropenia and a bout of bacterial infection was studied. Anti-neutrophil IgG antibody was detected by indirect immunofluorescence using a laser flow cytometry system. Purified IgG from our patient's serum did not inhibit chemotaxis of neutrophils, but inhibited rosette formation of neutrophils with ox red blood cells (ORBC) coated with anti-ORBC rabbit IgG dose-dependently. Surface iodination of neutrophils followed by their immunoprecipitation by purified IgG and sodium dodecylsulfate polyacrylamide gel electrophoresis showed a single band that corresponded to 45 kilodaltons. Possibly the IgG antibody in our patient's serum recognizes molecules related to Fc receptors.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
8/13. Long-term remission of immune neutropenia after treatment with high-dose intravenous 7S-immunoglobulin.In a 61-year-old woman recurrent bacterial infections were caused by immune neutropenia which was resistant to high-dose cortisone and plasmapheresis. However, high-dose intravenous 7S-immunoglobulin therapy (30 g for 6 days) induced a long-term remission for 35 months. This treatment modality is safe and should be used as a first-line agent.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
9/13. Severe autoimmune protein s deficiency in a boy with idiopathic purpura fulminans.Idiopathic purpura fulminans usually occurs in young children and is frequently preceded by a preparatory viral or bacterial infection. Following a severe streptococcal pharyngitis, an 8-year-old boy developed purpura fulminans with disseminated intravascular coagulation and severe protein s deficiency (total antigen < 0.05 u/ml). Despite generous plasma infusions, skin necrosis progressed rapidly into compartment syndrome which required fasciotomy and skin grafting and resulted in the loss of three digits of the right foot. Total protein S remained low for over a month despite plasma supplementation and complete normalization of protein c levels. A polyclonal anti-protein S IgG was demonstrated in the patient's plasma, which decreased to 25% of baseline titre after 1 month and was undetectable 6 months after purpura fulminans, when plasma protein S had returned to normal. Transient, isolated and severe deficiencies of protein S have been reported in patients with idiopathic purpura fulminans and a previous preparatory infection. Autoimmune protein s deficiency may play a key role in the aetiopathogenesis of idiopathic purpura fulminans.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
10/13. Polyautoimmune syndrome in common variable immunodeficiency.common variable immunodeficiency (CVI) is a heterogenous disorder with hypogammaglobulinaemia and multiple bacterial infections primarily involving the sinopulmonary tract. CVI patients have been known to have an increased tendency to develop autoimmune manifestations. Commonly associated autoimmune diseases include immune thrombocytopenic purpura, autoimmune haemolytic anaemia, and rheumatoid arthritis. In this paper we report a case of CVI presenting with multiple unusual autoimmune diseases including parotitis, vitiligo, atrophic gastritis, pernicious anaemia, and primary biliary cirrhosis. To our knowledge, this is the first case of CVI with polyautoimmunity and antimitochondria antibody. Recognition of this association is important because early diagnosis and treatment can greatly influence the prognosis.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
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