1/26. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/26. clozapine-induced agranulocytosis in a Native American: HLA typing and further support for an immune-mediated mechanism.BACKGROUND: Approximately 30% of schizophrenic patients defined as treatment refractory significantly improve with clozapine. However, clozapine produces agranulocytosis in approximately 1% to 2% of patients in the united states. The mechanism of clozapine-induced agranulocytosis has not been established, but evidence suggests an immune-mediated mechanism. METHOD: Human leukocyte antigen (HLA) typing was performed in a native American with clozapine-induced agranulocytosis. RESULTS: Our findings support previous observations of a role of the HLA-B16, DR4, DQw3 haplotype in predicting susceptibility to agranulocytosis in clozapine-treated patients. CONCLUSION: We suggest that HLA typing of clozapine candidates may be useful for predicting the risk for clozapine-induced agranulocytosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/26. agranulocytosis and anaemia induced by sulfametopyrazine in a sulfametopyrazine-trimethoprim combination.We report a case of prolonged fever, agranulocytosis, and anaemia associated with the long acting sulphametopyrazine-trimethoprim combination (Kelfiprim). A woman of 23 years took an overdose of 13 tablets over five days for presumed cystitis. One day after the last dose the patient developed fever and a generalised rash. The fever persisted and her previously normal leukocyte count decreased to 1.8 x 10(9)/1. After treatment with paracetamol the fever settled briefly, and then recurred for another 16 days. A later peripheral blood leukocyte count of 0.77 x 10(9)/1, haemoglobin of 10.8 g/dl, and a hypocellular bone marrow with depressed granulopoiesis and haemopoiesis suggested marrow suppression induced by sulfametopyrazine. Since the IgM antibody against the Epstein-Barr virus capsid antigen was detected, the adverse drug reaction might have been aggravated by this virus. The case highlights the risk of severe haematological adverse reactions associated with sulphonamide treatment, and argues for the use of trimethoprim alone for uncomplicated cystitis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/26. Hepatosplenic candidiasis: an overlooked cause of prolonged fever during recovery from an episode of neutropenia.Two cases of hepatosplenic candidiasis (HSC) are reported occurring after protracted episodes of neutropenia, induced by chemotherapy for acute leukemia in one case and drug hypersensitivity in the other. The disease presented with persistent or recurrent fever after correction of the neutropenia and with splenomegaly. The alkaline phosphatases were elevated. The diagnosis was strongly suggested by abdominal ultrasonography, CT scan and MRI, which showed multiple hepatosplenic defects. It was confirmed by serologic tests for candidiasis, the presence, in 1 case, of circulating candida antigens, and the rapid response to amphotericin b. The diagnosis of HSC should be considered in patients with persistent fever after an episode of neutropenia. Ideally, histologic confirmation is desirable, but this is often obtainable only by open liver biopsy, an aggressive procedure in such patients. Failing this, our 2 cases stress the diagnostic value of noninvasive imaging techniques, serological testing (in particular the discovery of circulating candida antigens) and the response to amphotericin b.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
5/26. Autoimmune neutropenia in infancy due to anti-NA1 antibody: detection of antibody with immunofluorescence and agglutination test.The sera from two patients with chronic neutropenia in infancy were examined for the presence of antineutrophil antibodies and their specificity against neutrophil antigen by using granulocyte indirect immunofluorescence test and microleukocyte agglutination test. In the microleukocyte agglutination test, the patients' sera reacted with neutrophils from their parents and normal unrelated donors having the neutrophil antigen NA1, but not with neutrophils from NA1- donors. After the absorption of patients' sera with NA1 neutrophils, the antibody activity was completely abolished, resulting in the confirmation of the anti-NA1 antibody. In contrast, the granulocyte indirect immunofluorescence test showed positive reactions against both NA1 and NA1- neutrophils, and the specificity for anti-NA1 was found in the results of the sera absorbed with NA1 neutrophils. This suggested that the absorption experiment might be necessary to determine the specificity of the antibody for neutrophil antigen. Thus, we confirmed two cases with autoimmune neutropenia caused by anti-NA1 antibody. A combination of agglutination and immunofluorescence techniques would be recommended for investigation of neutrophil antibodies against the neutrophil-specific antigen.- - - - - - - - - - ranking = 4keywords = antigen (Clic here for more details about this article) |
6/26. Granulocyte transfusion therapy and amphotericin b: adverse reactions?One hundred twenty-five granulocyte transfusions were given concurrently with amphotericin b to 31 granulocytopenic patients with acute leukemia during a four year period. Twenty-six patients had culture-documented, and 5 had presumed fungal infections; pulmonary infiltrates were present in 26 patient courses. Eight patients developed pulmonary deterioration temporally related to therapy with amphotericin, granulocyte transfusions, or both. One event occurred following amphotericin alone. Three additional reactions occurred in alloimmunized patients with antibodies to human leukocyte antigens (HLA) who received random donor granulocytes, which may indicate a potential mechanism for the pulmonary reactions. Two reactions potentially represent an adverse interaction between amphotericin and granulocytes, but these were reversible and were not unlike reactions expected with each modality alone. Our data fail to document a specific detrimental interaction between granulocyte transfusions and amphotericin beyond the reactions associated with each modality, and the data suggest that other clinical factors, particularly infection and alloimmunization, also contribute to pulmonary decompensation. We nevertheless recommend great care and attention be given to administering these modalities in the setting of severely ill patients.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/26. Immunological functions and T-cell receptor gene rearrangement of proliferating lymphocytes in a case of T gamma lymphocytosis with neutropenia.A case of T gamma lymphocytosis with neutropenia is presented. The patient showed mild lymphocytosis, splenomegaly, anemia, neutropenia and recurrent infections without progression for 15 years. The expanded lymphoid cells were morphologically large granular lymphocytes (LGL), had receptors for both sheep red blood cells and IgG-Fc portion and were positive for OKT3 and 8 antigens. They displayed ADCC activity, whereas they showed low responses to T-cell mitogens and deficient NK activity. They showed neither suppressor activity on antibody production by B-cells nor suppressor activity on CFU-C formation. The dna isolated from the expanded cells of the patient showed T-cell beta-chain (T beta) gene rearrangement, indicating monoclonality of the proliferation. This finding supports that the proliferation of T8 lymphocytes in the present case is neoplastic rather than reactive, regardless of the benign clinical course.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/26. Chronic T-cell lymphocytosis with neutropenia. Its association with Epstein-Barr virus infection.Chronic T-cell lymphocytosis with neutropenia has primarily been reported in adults. We have cared for a 14-year-old patient with this condition for over five years. This patient consistently had greater than 90% lymphocytes in his differential cell count. The majority of these cells had the OKT8 phenotype. Serologic studies demonstrated evidence for a chronic Epstein-Barr infection with elevated antibody titers to both early antigen and to the viral capsid antigen. patients presenting with this condition should be carefully evaluated for possible viral infection.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/26. natural history of primary autoimmune neutropenia in infancy.Five patients with primary autoimmune neutropenia were evaluated during their first 2 years of life. Their illness resolved spontaneously after 6 to 41 months (median 13 months), and the patients were subsequently followed for 13 to 73 months (median 28 months). None required immunosuppressive therapy to induce remission, and routine antibiotic therapy adequately controlled all infectious episodes. An increased rate of infection, particularly otitis media and upper respiratory tract infection, occurred during the neutropenic period. No other noninfectious illnesses, particularly no other autoimmune diseases, were reported in any of these patients at any time. In each case, resolution of neutropenia paralleled the disappearance of neutrophil autoantibodies which were specific for the NA1 antigen. This report describes the clinical and laboratory findings and the long-term history of primary autoimmune neutropenia in these five patients.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/26. neutropenia associated with large granular lymphocytes responsive to corticosteroids in vitro and in vivo.We describe a patient with neutropenia associated with increased circulating large granular lymphocytes (LGL). Absolute neutropenia was accompanied by the absence of myeloid precursor cells in the bone marrow. No myeloid progenitor cells (CFU-C) could be detected by in vitro colony culture. The peripheral blood was also remarkable for the presence of a population of large granular lymphocytes demonstrable by conventional staining. These cells in flow microfluorometry studies expressed antigens Leu 4 (T-cell antigen receptor), Leu 7 (natural killer cell marker), Leu 2 (suppressor cell marker), and HLA-DR (activation marker); they lacked Leu 1 (a pan-T cell antigen), Leu 3 (helper cell marker) and Tac (interleukin 2 receptor). Hematopoietic colony formation in vitro improved with addition of corticosteroids to the culture medium or elimination of the LGL population with complement-mediated cytotoxicity. Anti-neutrophil antibodies were present prior to and following therapy. Clinically, administration of prednisone resulted in a normalization of the total white blood cell count and absolute polymorphonuclear cell number, an increase into the normal range of the number of CFU-C, and elimination of the LGL population. In this case of steroid-responsive LGL-associated neutropenia, laboratory studies suggested direct suppression of myelopoiesis by steroid-responsive LGL.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
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