Omenn Syndrome (OS) is a rare autosomal recessive form of severe combined immunodeficiency characterized by failure to thrive, erythroderma, persistent diarrhea, lymphadenopathy, hepatosplenomegaly; severe recurrent infections [1]. The syndrome may be accompanied by normal or elevated levels of IgE, lymphocytosis, eosinophilia and hypogammaglobinemia [1]. The syndrome, also known as familial re...

Omenn Syndrome (OS) is a rare autosomal recessive form of severe combined immunodeficiency characterized by failure to thrive, erythroderma, persistent diarrhea, lymphadenopathy, hepatosplenomegaly; severe recurrent infections [1]. The syndrome may be accompanied by normal or elevated levels of IgE, lymphocytosis, eosinophilia and hypogammaglobinemia [1]. The syndrome, also known as familial re...

During recent years a number of primary immunodeficiencies resulting from impaired function of JAK-STAT molecules have been described. One of these is the Hyper-IgE syndrome (HIES) characterized by elevated IgE levels, eczema, recurrent staphylococcal skin and pulmonary infections and pleiotropic somatic manifestations. In 2007 the genetic basis of HIES was revealed by identification of dominan...

H. H. KESARWALA*, R. V. S. K. PRASADt, R. SZEP, E. OLDMAN, S. LANE & P. S. PAPAGEORGIOU The Department ofPediatrics, CMDHJ, Rutgers Medical School, Piscataway, New Jersey, * Division ofAllergy Immunology, Children's Hospital Medical Center, Elland and Bethesda Avenue, Cincinnati, Ohio 45229 and t Division ofAllergy, Department ofPediatrics, Children's Hospital ofMichigan, 3901 Beaubien Boulevar...

Hyper IgE Syndrome (HIES) is an immunodeficiency disorder characterized by increased serum levels of IgE, eczema, and recurrent cutaneous and pulmonary infections. In this report, we present, to our knowledge, the first documented case of renal cell carcinoma (RCC) found in a patient with HIES. The patient received infectious disease clearance prior to obtaining a partial nephrectomy which reve...

Article type: Review Article Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency disease. Most of HIES cases are sporadic. Autosomal dominant HIES is caused by mutation in signal transducer and activator of transcription-3 (STAT-3). A number of mosaicism HIES has been reported that is associated with intermediate phenotype. Autosomal recessive HIES is due to mutation in Dock-8 or cytok...

Immunoglobulin (Ig) class switching in B cells is regulated by stimuli transduced by cytokines and cell-cell contact. Among these stimuli, interleukin (IL)-4 has been considered an absolute prerequisite for class switching to IgE in the mouse. Here we report that IL-4-deficient (IL-4-/-) and wildtype mice had comparably elevated serum IgE levels during the course of a murine retrovirus-induced ...

Increased levels of serum IgE and eosinophilia have been described in human immunodeficiency virus (HIV) infection, almost exclusively in patients with CD4+ cell count < 200 cells/microliters. IgE production is regulated by CD4+ T helper type 2 (Th-2) lymphocytes, producing interleukin 4 (IL-4) and expressing a ligand for the B cell-specific CD40 molecule (CD40 ligand [L]). A shift to a Th-2-li...

Elevated IgE levels can be associated with a variety of causes. The diagnosis is most often made on a clinical basis taking into consideration a complete history and physical. In this case report we describe a patient with elevated IgE levels and our discovery upon further investigation after noticing some unique findings on his physical examination. The differential diagnosis of extremely elev...

Bronchial asthma was classified by the pathophysiology and by the mechanism of onset of the disease. Forty asthmatics who had serum IgE levels lower than 200 IU/ml were evaluated by two classification methods. 1. In asthma classified by a score based on clinical findings and examinations, the characteristics of the findings and examination results were compared among three asthma types, i.e., I...