Multiprofessional Critical Care Review: Pediatric 2024 (MCCRCP24ON)-Primary Acquired Immunodeficiencies

Primary Acquired Immunodeficiencies

Pdf Summary

The presentation, delivered by Dr. Asya Agulnik from St. Jude Children’s Research Hospital, focuses on the components, signs, symptoms, and treatment of primary and acquired immunodeficiencies. Dr. Agulnik set out to educate on the innate and adaptive immune systems, the characteristics of primary immunodeficiencies, and strategies for managing these conditions.<br /><br />The immune system comprises barriers (skin, mucociliary), non-cellular components (complement, acute phase reactants, immunoglobulins), and cellular defenses split into innate and adaptive immunity. Innate immunity involves non-specific defenses active from birth and includes cells like neutrophils and macrophages, whereas adaptive immunity involves T and B lymphocytes and develops memory for specific pathogens.<br /><br />Primary immunodeficiencies show features such as recurrent and severe infections, granulomas, autoimmune diseases, and a family history of immunodeficiencies. Specific cellular defects like those in neutrophils and lymphocytes lead to a range of syndromes such as Kostmann Syndrome, Chronic Granulomatous Disease (CGD), Severe Combined Immunodeficiency (SCID), and Wiskott-Aldrich Syndrome, each with distinct symptoms, diagnostic markers, and treatments (like G-CSF, IVIG, and HSCT).<br /><br />Lymphocytes can exhibit both quantitative and qualitative defects, leading to disorders like DiGeorge Syndrome, Bruton’s agammaglobulinemia, and Hyper IgM Syndrome. These conditions generally require specific treatments like HSCT, IVIG, or antibiotic prophylaxis.<br /><br />Natural Killer (NK) cell defects, such as those seen in familial hemophagocytic lymphohistiocytosis (HLH), lead to severe hyperinflammation and require treatments ranging from corticosteroids to more aggressive therapies like HSCT.<br /><br />Non-cellular elements such as complement proteins and immunoglobulins can also be deficient, leading to increased susceptibility to infections with encapsulated organisms. Conditions such as asplenia and secondary (acquired) immunodeficiencies from factors like HIV/AIDS or chemotherapy further impact immune function, increasing infection risks.<br /><br />Understanding these immunodeficiencies is crucial for appropriate diagnosis, infection risk management, and implementation of necessary treatments to improve patient outcomes.

Keywords

immunodeficiencies

innate immunity

adaptive immunity

primary immunodeficiencies

treatment

immune system

NK cell defects

complement proteins

HSCT

IVIG

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