Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation

Abstract

Synopsis Elevated serum eosinophil levels have been associated with multiple disorders of immune deficiency or immune dysregulation. Although primary immunodeficiency diseases (PIDD) are rare, it is important to consider these in the differential diagnosis of patients with eosinophilia. This review discusses the clinical features, laboratory findings, diagnosis, and genetic basis of disease of several disorders of immune deficiency or dysregulation – all which have documented eosinophilia as part of the syndrome. The article includes autosomal dominant hyper IgE syndrome, DOCK8 deficiency, PGM3 deficiency, ADA-SCID, Omenn syndrome, Wiskott-Aldrich syndrome, Loeys-Dietz syndrome, autoimmune lymphoproliferative syndrome, immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome, Comel-Netherton syndrome, and severe dermatitis, multiple allergies, and metabolic wasting syndrome (SAM).

Impact of atopy on the severity and extrapulmonary manifestations of childhood Mycoplasmapneumoniae pneumonia

“…Interestingly, the serum total IgE levels were also higher in the extrapulmonary group than in the pulmonary group, which was consistent with the findings in the recent reports . The increased IgE levels can be considered a marker of immune dysregulation . These findings may partly explain the relationship between atopy and extrarespiratory manifestations and indicate that an individual with an atopic predisposition could have an additional stimulation driving IgE production during MP infection; as a consequence, this individual might develop some self‐reactive IgEs that promote immune‐mediated diseases and manifestations.…”

“…7,8 The increased IgE levels can be considered a marker of immune dysregulation. 19,20 These findings may partly explain the relationship between atopy and extrarespiratory manifestations and indicate that an individual with an atopic predisposition could have an additional stimulation driving IgE production during MP infection;…”

Human genetic basis of interindividual variability in the course of infection

“…Autoinflammatory phenotypes include a tremendous variety of inflammatory conditions in which there is no evidence for the presence of autoreactive T and B cells (126). Allergic phenotypes have been attributed more recently to certain primary immunodeficiencies (127). The genetic causes of these conditions were discovered by candidate gene approaches from 1985 onward (adenosine deaminase deficiency) (128), by positional cloning from 1986 onward (chronic granulomatous disease) (129), and by next-generation sequencing from 2010 onward (fas-associated via death domain deficiency) (130).…”

Aplicações práticas de uma plataforma multiplex para detecção de IgE específica por componentes alergênicos em doenças alérgicas

“…No entanto, a IgE total, isoladamente, não se apresenta como um marcador fidedigno de atopia. Algumas doenças que cursam com desregulação imunológica com polarização para o perfil Th2 (parasitoses intestinais e cutâneas), bem como algumas imunodeficiências (síndrome de Wiscott-Aldrich, síndrome de Hiper-IgE) também podem cursar com níveis muito elevados de IgE total plasmática 7,8 .…”