1/14. Molecular and cytogenetic remission in a case of subtype M4E acute myelogenous leukemia with minimal monochemotherapy: high sensitivity or spontaneous remission?Complete remission was observed in an adult patient with acute myelogenous leukemia after minimal monochemotherapy. Remission occurred after a severe febrile pneumonia and was accompanied by cytogenetic and molecular remission. The hypothesis of spontaneous remission was raised, even if a high sensitivity to low-dose cytostatics cannot be excluded. Such spontaneous complete remissions, often associated with bacterial infections and blood transfusions, are extremely rare, and are usually of short duration. Previous cases are summarized, and the role of etiologic factors is discussed.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
2/14. Recurrent pneumonia with mild hypogammaglobulinemia diagnosed as X-linked agammaglobulinemia in adults.BACKGROUND: X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency caused by disruption of the Bruton's tyrosine kinase (BTK) gene. Typical XLA patients suffer recurrent and severe bacterial infections in childhood. methods: Flow cytometric analysis of the peripheral monocytes using the anti-BTK antibody was used to characterize a 27 year old male patient with mild hypogammaglobulinemia (IgG, 635 mg/dl; IgM, 11 mg/dl; IgA, <5 mg/dl). He had suffered from frequent pneumonia since age 25 but had no history of frequent infections in his childhood or in adolescence. Sequencing of the BTK cDNA obtained from an Epstein-Barr virus-transformed B lymphoblastoid cell line derived from the bone marrow of the patient was performed to confirm a genetic defect. RESULTS: Flow cytometric analysis of cytoplasmic BTK protein in peripheral monocytes indicated that the patient presents a rare case of adult-onset XLA and that his mother is an XLA carrier. Sequencing of the BTK gene revealed a deletion of AG in the codon for Glu605 (AGT), resulting in an aberrant stop codon that truncates the BTK protein in its kinase domain. CONCLUSIONS: This case suggests that some XLA cases may remain undiagnosed because they only show mild hypogammaglobulinemia and they lack repeated infections in childhood. Flow cytometric analysis is a powerful method to screen these patients.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
3/14. An extremely basic monoclonal IgG in an aged apoplectic patient with prolonged bacterial infection.A case with prolonged bacterial infection accompanied by an abnormal serum protein which migrated in the post-gamma region on electrophoresis is presented. The abnormal protein was identified as IgG with gamma-type light chain moiety. The patient suffered from prolonged pneumonia and cholecystitis, Bone marrow aspiration and skeletal x-rays did not indicate multiple myeloma.- - - - - - - - - - ranking = 5keywords = bacterial infection (Clic here for more details about this article) |
4/14. De novo mutation causing X-linked hyper-IgM syndrome: a family study in taiwan.X-linked hyper-IgM syndrome (XHIM) is a rare primary immunodeficiency disorder caused by mutations of the gene encoding the cd40 ligand (CD40L). It is characterized by recurrent infections with markedly decreased serum IgG, IgA and IgE levels but normal or elevated IgM levels. We report the clinical manifestations and complete immune studies in the first family with molecularly proven XHIM in taiwan. A 5-month-old boy presented with rapidly progressive pneumonia which responded poorly to antibiotics. High levels of IgM and very low levels of IgG, IgA, and IgE were noted in his plasma specimen: IgM, 128 mg/dl; IgG, 18 mg/dl; IgA, 4 mg/dl); IgE, 1 IU/ml. Whole blood flow cytometry when he was 21 months old showed that only a small percentage (0.48%) of his in vitro-activated CD4 T cells expressed CD40L. When he was 3 years old, repeated flow cytometry showed essentially the same result (0.4%), compared with his father's CD40L expression of over 85%. The patient's mother had moderately decreased CD40L expression (74.4%). Hyper-IgM syndrome was confirmed by CD40L mutation analysis in the boy, which revealed a Lys 96 stop (nucleotide A307T) in exon 2 of CD40L, with a truncated protein resulting in the loss of the entire TNF domain. His mother was a carrier and apparently the individual in whom the mutation originated. Eleven other family members, including the patient's father, sister, and grandmother, and the mother's sisters and their children, all had normal results on CD40L mutation analysis. The patient has remained without significant bacterial infection on a regimen of monthly IVIG infusion and oral trimethoprim-sulfamethoxazole for pneumocystis carinii pneumonia (PCP) prophylaxis, although he has had recurrent oral ulcers and neutropenia. bone marrow transplantation is planned.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
5/14. common variable immunodeficiency mimicking rheumatoid arthritis with sjogren's syndrome.Several autoimmune diseases have been reported to be associated with common variable immunodeficiency disease (CVID), including rheumatoid arthritis and sjogren's syndrome. On the other hand, approximately 20-30% of patients with rheumatoid arthritis develop secondary sjogren's syndrome. A 26-year-old woman had a 6-year history of chronic symmetric polyarthritis and 3-year history of sicca syndrome prior to admission for pneumonia. Rheumatoid arthritis with secondary sjogren's syndrome had been diagnosed 1 year before. The patient had experienced 3 episodes of pneumonia during the previous 3 years. Markedly depressed serum immunoglobulin levels prompted a suspicion of common variable immunodeficiency, and the impression was confirmed after a series of examinations. Monthly administration of intravenous immunoglobulin (IVIG) alleviated the polyarthritis and improved the sicca syndrome. IVIG replacement therapy was ultimately successful in curing recurrent bacterial infections, chronic polyarthritis, and improving the severity of sicca syndrome.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
6/14. Epithelioid germinal centers in overwhelming childhood infections. The aftermath of nonspecific destruction of follicular B cells by natural killer cells.The destruction of proliferating lymphoid cells within germinal centers with subsequent replacement by histiocytoid cells has been described in infants and children dying of viral and bacterial infections. The etiology and significance of "epithelioid germinal centers" (EGCs) are unknown. The cells implicated in forming EGCs have included histiocytes and dendritic reticulum cells. We have studied four children at autopsy who died at ages ranging from 10 months to 7 years. Three contracted fatal infections, one with fulminant meningococcemia, one with bacterial sepsis, and one with viral hepatitis. The fourth child contracted viral pneumonitis and died of acetaminophen toxicity. Epithelioid germinal centers were found in numerous lymphoid organs (spleen, lymph nodes, and Peyer's patches) in all four cases. avidin-biotin complex immunohistochemical analysis performed on formalin-fixed splenic tissue from the first three cases and snap-frozen splenic tissue from the second case revealed an absence of B cells in the follicular centers. The mantle zones surrounding follicles were thin but intact. The histiocytoid cells expanding the germinal centers were positive for S100 and R4/23 (dendritic reticulum cells) and negative for numerous histiocyte markers (alpha 1-antitrypsin, alpha 1-antichymotrypsin, and lysozyme). Increased numbers of killer cells (Leu-7) were present within the affected germinal centers in the three cases in which material was available for immunohistochemical studies. Overwhelming infections in these patients seem to result in anomalous natural killer cell activation resulting in localized nonselective destruction of follicular centers similar to anomalous natural killer cell activity reported to occur in fatal infectious mononucleosis. This may lead to an acquired immunodeficiency that precludes long-term survival in affected patients.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
7/14. Increased susceptibility to infection in hypothermic children: possible role of acquired neutrophil dysfunction.The addition of hypothermia to regimens to control cerebral edema in children at our institution has been associated with a substantial incidence of infectious complications. Of the 13 children maintained at 30 degrees C to prevent cerebral edema, 3 developed haemophilus influenzae pneumonia and 2 developed streptococcus pneumoniae sepsis (one with pneumonia). The importance of neutrophil (PMN) function for elimination of bacterial pathogens prompted in vitro studies of PMN function at clinically attainable hypothermic temperatures. neutrophils at 30 degrees C had significantly less ability to migrate towards a chemotactic stimulus (45 /- 10% inhibition; P less than 0.02), to ingest staphylococci (22 /- 5% inhibition; P less than 0.01) and to be metabolically activated as measured by superoxide production (35 /- 10% inhibition; P less than 0.01) or by chemiluminescence (18 /- 8% inhibition; P less than 0.05). These in vitro findings support the clinical observation that persons with decreased body temperature may be at an increased risk for bacterial infections secondary to PMN dysfunction.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
8/14. haemophilus influenzae infections in adults: report of nine cases and a review of the literature.haemophilus influenzae is an aerobic pleomorphic gram-negative coccobacillus that requires both X and V factors for growth. It grows poorly, if at all, on ordinary blood agar unless streaked with Staph. aureus. It grows well on chocolate agar. Because this medium is often not used in culturing specimens from adults and because the organism may be overgrown by other bacteria, the frequency of H. influenzae infections has undoubtedly been seriously underestimated. This is aggravated by the failure of many physicians to obtain blood cultures in suspected bacterial infections and the failure of many laboratories to subculture them routinely onto chocolate agar. H. influenzae, along with streptococcus pneumoniae, is a major factor in acute sinusitis. It is probably the most frequent etiologic agent of acute epiglottitis. It is probably a common, but commonly unrecognized, cause of bacterial pneumonia, where it has a distinctive appearance on Gram stain. It is unusual in adult meningitis, but should particularly be considered in alcoholics; in those with recent or remote head trauma, especially with cerebrospinal fluid rhinorrhea; in patients with splenectomies and those with primary or secondary hypogammaglobulinemia. It may rarely cause a wide variety of other infections in adults, including purulent pericarditis, endocarditis, septic arthritis, obstetrical and gynecologic infections, urinary and biliary tract infections, and cellulitis. Antimicrobial susceptibility testing is somewhat capricious in part from the marked effect of inoculum size in some circumstances. in vitro and in vivo results support the use of ampicillin, unless the organism produces beta-lactamase. Alternatives in minor infections include tetracycline, erythromycin, and sulfamethoxazole-trimethoprim. For serious infections chloramphenicol is the best choice if the organism is ampicillin-resistant or the patient is penicillin-allergic.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
9/14. Spontaneous remission of acute myeloid leukemia. A report of a case and brief review of the literature.A patient with acute myelomonocytic leukemia who experienced a spontaneous remission, is reported. He had precedent and concurrent bacterial infections as most of these cases described. Low peripheral WBC and myeloblasts, Auer-rod positive blasts, bone marrow eosinophilia with atypical eosinophils, and a partial deletion of chromosome 16 were favorable prognostic parameters. A brief review of the literature and possible explanations for the regulation of granulopoiesis are presented.- - - - - - - - - - ranking = 1keywords = bacterial infection (Clic here for more details about this article) |
10/14. C2 deficiency, moderately low IgG2 concentrations and lack of the G2m(23) allotype marker in a child with repeated bacterial infections.C2 deficiency was demonstrated in an 11-year-old boy with a past history of recurrent purulent otitis media, pneumonia, H. influenzae meningitis and S. pneumoniae septicaemia. The major histocompatibility complex haplotypes present, A10, B18, DR2, BF*S, C2*QO, C4*A4, C4*B2 and A28, B18, DR2, BF*S, C2*Q0, C4*A4, C4*B2, were in accord with previous observations in C2 deficiency. The concentrations of C1q, C5, factor B and factor D were in the low normal range and the hemolytic activity of the alternative pathway was slightly decreased. In addition, the patient showed moderately low IgG2 concentrations and lacked the IgG2 subclass marker G2m(23). The findings indicate that the patient's susceptibility to bacterial infections may be due to C2 deficiency in combination with the presence of an IgG allotype associated with impaired antibody responses to carbohydrate antigens.- - - - - - - - - - ranking = 5keywords = bacterial infection (Clic here for more details about this article) |
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