Clinical approach to high immunoglobulin E levels and hyperimmunoglobulinemia E syndromes
Current Approaches to Allergic Diseases, NOVA Publications , ss.89-118, 2024
- Yayın Türü: Kitapta Bölüm / Araştırma Kitabı
- Basım Tarihi: 2024
- Yayınevi: NOVA Publications
- Sayfa Sayıları: ss.89-118
- Anahtar Kelimeler: Epsilon-chains, Hyper IgE syndromes, Hypersensitivity, Immunodeficiency, Immunoglobulin E
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Immunoglobulin (Ig)E has a crucial role, particularly in atopic diseases and in host defense against parasitic infections. Due to its high affinity binding to receptors, IgE has the ability to generate a rapid biological response that can impact various systems in the body. From a clinical viewpoint, there is a wide differential diagnosis of elevated IgE including atopic diseases, parasitic infestations, primary immunodeficiencies, viral, bacterial, and fungal infections, autoimmune disorders, neoplastic, cutaneous, chronic liver and kidney diseases. Hyper IgE syndromes marked by a triad of atopic dermatitis, recurrent skin, and lung infections, and elevated IgE levels stand out among these diseases in terms of the importance of early diagnosis and treatment. While autosomal dominant Hyper IgE Syndrome (Signal transducer and activator of transcription 3 deficiency) serves as the prototype for these disorders, the past decade has witnessed the identification of several other genetically characterized immunodeficiency disorders, expanding the spectrum covered under the umbrella of Hyper IgE Syndromes. It appears that Hyper IgE Syndrome is not a singular disease but rather a cluster of related disorders with a diverse range of underlying genetic defects. Ultimately, Hyper IgE Syndrome can be identified by closely monitoring the clinical progression, often characterized by the onset and recurrence of distinctive infectious episodes or the presence of non-immunological manifestations. The use of genetic testing aids in forecasting the clinical trajectory and identifying the necessity for treatments such as hematopoietic stem cell transplantation. In this overview, we will briefly examine these conditions, explore their molecular bases, emphasize notable clinical and laboratory features, and discuss available therapeutic choices. Despite their similar phenotypes, it is essential to note that the treatment approaches for these syndromes vary.