Abstract:
:Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome. Lethal opportunistic infections are a feature of Good's syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good's syndrome. We now describe a 70 year old man with Good's syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Akinosoglou K,Melachrinou M,Siagris D,Koletsis E,Marangos M,Gogos CA,Solomou EEdoi
10.1007/s10875-014-0014-7subject
Has Abstractpub_date
2014-04-01 00:00:00pages
283-8issue
3eissn
0271-9142issn
1573-2592journal_volume
34pub_type
杂志文章,评审abstract::MECP2 (methyl CpG binding protein 2) duplication causes syndromic intellectual disability. Patients often suffer from life-threatening infections, suggesting an additional immunodeficiency. We describe for the first time the detailed infectious and immunological phenotype of MECP2 duplication syndrome. 17/27 analyzed ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-015-0129-5
更新日期:2015-02-01 00:00:00
abstract::The 22q11.2 deletion syndrome (22q11.2 del), also known as DiGeorge syndrome, is a genetic disorder with an estimated incidence of 1:3000 to 1:6000 births. These patients may suffer from affection of many organ systems with cardiac malformations, immunodeficiency, hypoparathyroidism, autoimmunity, palate anomalies, an...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-020-00766-x
更新日期:2020-04-01 00:00:00
abstract:OBJECTIVE:This study was to investigate the evidence for complement activation in renal biopsy specimens of patients with myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibody (ANCA)-associated pauci-immune vasculitis. METHODS:Renal biopsy specimens from seven patients with MPO-ANCA positive pauci-immune necro...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9268-2
更新日期:2009-05-01 00:00:00
abstract::Biallelic mutations in SLC29A3 cause histiocytosis-lymphadenopathy plus syndrome, also known as H syndrome (HS). HS is a complex disorder, with ~ 25% of patients developing autoinflammatory complications consisting of unexplained fevers, persistently elevated inflammatory markers, and unusual lymphadenopathies, with i...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-020-00932-1
更新日期:2020-12-07 00:00:00
abstract::We showed previously that the conserved extended MHC haplotype [HLA-B8, SCO1, DR3] carries recessive susceptibility genes for IgA and IgG4 deficiency and dominant genes for IgD and IgG3 deficiency. [HLA-B18, F1C30, DR3] has similar class II and III regions to [HLA-B8, SC01, DR3] and is common in the Basques. We theref...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1006693614974
更新日期:2000-05-01 00:00:00
abstract::This review describes a series of studies performed in our laboratory which have focused on the activation and subsequent proliferation and differentiation of human B lymphocytes. Utilizing polyclonal signals which activate B cells by interacting with their surface membrane Ig, we have examined the events in the trans...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917136
更新日期:1984-09-01 00:00:00
abstract:OBJECTIVE:The purpose of the study was to determine associations between pre-antiretroviral therapy (ART) senescent CD8+ T lymphocytes and naïve versus non-naive CD8+ and CD4+ T lymphocyte subpopulations and CD4+ responses after initiation of ART in younger versus older individuals. METHODS:Retrospective analysis of 1...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9550-6
更新日期:2011-10-01 00:00:00
abstract::Glucocorticoids (GC) represent the main treatment for pemphigus; however, some patients show GC resistance. GC sensitivity was evaluated in 19 pemphigus patients and 41 controls by the number of binding sites [B(max) (fmol/mg protein)] and the affinity of GC receptor [Kd (nM)] to dexamethasone (DEX) as well as by the ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9679-y
更新日期:2012-08-01 00:00:00
abstract::Palifermin (recombinant human keratinocyte growth factor) prevents the development of acute, lethal graft-versus-host disease (GVHD). It does so, at least in part, by protecting cells from injury. Another property of Palifermin is immune regulation. How the latter influences the evolution of GVHD remains uncertain. We...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-006-9039-x
更新日期:2006-09-01 00:00:00
abstract::A 35-year-old man with common variable hypogammaglobulinemia had repeated anaphylactic reactions to intramuscular human immune serum globulin (HISG), preventing him from receiving the injections. He was able to tolerate slow subcutaneous HISG infusions without local or systemic side effects at a dose of 12 ml/week giv...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915353
更新日期:1983-07-01 00:00:00
abstract::Autism is a complex polygenic neurodevelopmental disorder characterized by deficits in communication and social interactions as well as specific stereotypical behaviors. Both genetic and environmental factors appear to contribute to the pathogenesis of autism. Accumulating data including changes in immune responses, l...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-010-9402-9
更新日期:2010-05-01 00:00:00
abstract::We have evaluated the functional properties of the OK-T8+/OKT4+ T-cell subpopulations in nine patients with a new syndrome of acquired immune deficiency (AIDS). Despite polyclonal hypergammaglobulinemia in the sera of these patients, their peripheral blood lymphocytes (PBL) produced negligible quantities of immunoglob...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915797
更新日期:1983-10-01 00:00:00
abstract:PURPOSE:Oxygen therapy (hyperoxia) is essential for the treatment of some neonatal critical care conditions. The lung is the primary target for the changes induced by hyperoxia. Secretory immunoglobulin A (SIgA), IgA and secretory component (SC) reflect the local immunity in the respiratory tract induced by hyperoxia. ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9891-4
更新日期:2013-07-01 00:00:00
abstract::This study aimed to verify whether rs4986790 A > G single nucleotide polymorphism of toll like receptor 4 (TLR-4) associates with a more severe course of hepatitis B virus (HBV) chronic infection. A cross-sectional study enrolled 191 Caucasian HBV-positive patients: 28 HBsAg + inactive carriers, 121 chronic hepatitis ...
journal_title:Journal of clinical immunology
pub_type: 信件
doi:10.1007/s10875-012-9822-9
更新日期:2013-02-01 00:00:00
abstract::We investigated the humoral (antigen-specific immunoglobulin isotypes, IgG subclasses, and avidity maturation) and cellular (antigen-specific in vitro proliferation) immune response in 18 healthy adult volunteers, following a primary and a single booster vaccination with the T-cell dependent neoantigen rabies administ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/b:joci.0000010429.36461.6b
更新日期:2003-11-01 00:00:00
abstract::Using an enzyme-linked immunoassay we tested the sera of 71 patients with digestive system cancer, 35 patients with various nonmalignant disorders, and 28 normal individuals for anti-CEA activity. Antibodies were found in the sera of 51% of the patients. Most of the patients positive for the antibodies (70%) had no ev...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01540984
更新日期:1994-09-01 00:00:00
abstract:BACKGROUND:The peroxisome proliferator-activated receptors (PPARs) have been implicated in immune regulation. We determined the transcriptional expression of the three isoforms, PPARalpha, PPARgamma1, and PPARgamma2 in the peritoneal macrophages, CD4- and CD8-positive lymphocytes in non-obese diabetic (NOD) mice at 5 a...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9300-1
更新日期:2009-09-01 00:00:00
abstract::Among a population of uniformly differentiated TH(2) cells, only a portion express IL-4 upon stimulation and those that do often express the product of only a single allele. We review the evidence for the basis of IL-4 monoallelism and argue that it depends upon probabilistic expression of the Il4 gene. Further, we ar...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-005-8218-5
更新日期:2005-11-01 00:00:00
abstract::This special article deals with the role of processing enzymes in the generation of bioactive cytokines, particularly IL-1 beta and the novel cytokine IL-18, which was formerly called IFN gamma-inducing factor (IGIF). The "classical" pathways of cytokine processing are described, as well as the importance of alternati...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1023/a:1020506300324
更新日期:1999-01-01 00:00:00
abstract::Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia-telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. pneumoniae is a common pathogen responsible from pulmonary infections and impaired antibody response to pol...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020599810261
更新日期:1999-09-01 00:00:00
abstract::The origin of natural antibodies has long been a subject of controversy. Polyreactive natural antibodies recognize multiple ligands and are thought to arise from B1 B cells. Natural antibodies against carbohydrate antigens such as Gal alpha 1-3Gal or against blood groups A and B are thought to be "elicited" by gut bac...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027378716015
更新日期:1997-07-01 00:00:00
abstract::The original version of this article unfortunately contained mistake in the following sentence in the Abstract. ...
journal_title:Journal of clinical immunology
pub_type: 已发布勘误
doi:10.1007/s10875-019-00653-0
更新日期:2019-07-01 00:00:00
abstract:PURPOSE:The present study was designed to evaluate the efficacy and safety of a novel, 10% liquid formulation of intravenous immunoglobulin, stabilized with 250 mmol/L L-proline (Privigen), in patients with primary immunodeficiency disease. MATERIALS AND METHODS:Eighty adults and children diagnosed with common variabl...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-008-9231-2
更新日期:2009-01-01 00:00:00
abstract:PURPOSE:The aim of the present study was to evaluate whether the anti-Rheumatoid arthritis (RA) effect of curcumin is associated with the regulation of B cell-activating factor belonging to the TNF family (BAFF) production. METHODS:Collagen-induced arthritis (CIA) was induced in DBA/1 J mice by immunization with bovin...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9839-0
更新日期:2013-04-01 00:00:00
abstract:PURPOSE:Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B and sometimes NK cell function. Non-ablative HLA-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9797-6
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase t...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-014-0001-z
更新日期:2014-05-01 00:00:00
abstract::The role of tumor necrosis factor-alpha in the shedding of soluble tumor necrosis factor receptors in endotoxemia was investigated. The appearance of the soluble tumor necrosis factor receptors was assessed in four healthy volunteers following an intravenous injection of tumor necrosis factor-alpha and in eight chimpa...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01489489
更新日期:1995-01-01 00:00:00
abstract:BACKGROUND:Although interleukin-17 (IL-17) has been reported to participate in the pathogenesis of infectious, autoimmune and allergic disorders, the precise role in allograft rejection remains uncertain. This study illustrates that IL-17 contributes to the pathogenesis of chronic allograft rejection. RESULT:Utilizing...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9366-9
更新日期:2010-03-01 00:00:00
abstract::Human peripheral B lymphocytes were transformed with Epstein-Barr virus and probed with an antiidiotypic antibody made against a human monoclonal autoantibody. Six cell lines were isolated that produced antibodies bearing a common idiotope. Despite the presence of this common idiotope, the antibodies showed antigen-bi...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00916951
更新日期:1988-11-01 00:00:00
abstract:BACKGROUND:Recently, two functional IL18 promoter variants, -607C>A (rs1946518) and -137G>C (rs187238), were associated with viral clearance in patients with hepatitis C. The present study focused on their relevance for treatment response. METHODS:Seven hundred fifty-seven chronically infected European patients and 79...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9302-z
更新日期:2009-09-01 00:00:00