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Immunoglobulins (Ig) or antibodies are glycoproteins produced by plasma cells. B cells are instructed by specific immunogens, for example, bacterial proteins, to differentiate into plasma cells, which are protein-making cells that participate in humoral immune responses against bacteria, viruses, fungi, parasites, cellular antigens, chemicals, and synthetic substances. The immunogen or antigen reacts with a B-cell receptor (BCR) on the cell surface of B lymphocytes. A signal is produced that directs the activation of transcription factors to stimulate the synthesis of antibodies, which are highly specific for the immunogen that stimulated the B cell. Furthermore, one clone of a B cell makes an immunoglobulin (specificity). Besides, the immune system remembers the antigens that caused a previous reaction (memory) due to the development of memory B cells. These are intermediate, differentiated B cells that can quickly become plasma cells. In addition, circulating antibodies recognize antigens in tissue fluids and serum. This activity describes the physiology and pathophysiology of immunoglobulins. Objectives: Describe the function of immunoglobulins. Outline disorders associated with immunoglobulin deficiencies. Summarize the presentation of patients with immunoglobulin deficiency. Explain the importance of improving care coordination amongst the interprofessional team to enhance care delivery for patients with immunoglobulin deficiency. Access free multiple choice questions on this topic.
Immunoglobulins (Ig) or antibodies are glycoproteins produced by plasma cells. B cells are instructed by specific immunogens, for example, bacterial proteins, to differentiate into plasma cells. Plasma cells are protein-making cells participating in humoral immune responses against bacteria, viruses, fungi, parasites, cellular antigens, chemicals, and synthetic substances.[1] Immunoglobulins constitute about 20% of the protein in plasma. The immunogen or antigen reacts with a B-cell receptor (BCR) on the cell surface of B lymphocytes. A signal is produced that directs the activation of transcription factors to stimulate the synthesis of antibodies, which are highly specific for the immunogen that stimulated the B cell. Furthermore, one clone of a B cell makes an immunoglobulin (specificity). The immune system remembers the antigens that caused a previous reaction (memory) due to the development of memory B cells. These are intermediate, differentiated B cells that can quickly become plasma cells. Circulating antibodies recognize antigens in tissue fluids and serum. The following are five types of immunoglobulins in humans: IgM IgG IgA IgE IgD[2]
Managing patients with immunoglobulin deficiencies involves an interprofessional team that includes nurses, clinicians, and pharmacists. Many of these patients are prone to infections and opportunistic organisms; hence surveillance and close monitoring of the patient is vital. Immunologists, transplant specialists, geneticists, and hematologists from NIH collaborate in an interprofessional manner for the Blood and Immune Deficiency–Cellular Therapy Program (BID–CTP). These clinicians deliver state-of-the-art and experimental care to patients with rare blood and immune system diseases through an integrated and interprofessional approach to evaluating, treating, and monitoring patients. Similarly, PIDTC (Primary Immune Deficiency Treatment Consortium) is a network of 33 centers in North America that explore the management of severe primary immunodeficiency diseases (PID). PIDTC evaluates the natural history of patients treated for Severe Combined Immunodeficiency (SCID), Wiskott-Aldrich Syndrome, and Chronic Granulomatous Disease through retrospective, prospective and cross-sectional studies. In addition, the PIDTC has interprofessional collaborative partnerships with European and international colleagues and works with patient advocacy groups to promote community awareness.[30]
Clinical nurse specialists (CNS) interventions and other healthcare professionals can reduce the risk of adverse events associated with immunoglobulin therapy. Providing adequate hydration before IVIG administration can reduce the risk of developing an acute kidney injury. Nursing interventions for post-infusion headaches are to decrease the infusion rate, and administering premedication with NSAIDs /acetaminophen can be helpful.[31] If there is a concern regarding the development of aseptic meningitis, the concerned clinician should be contacted immediately, and switching to subcutaneous immunoglobulin (SCIg) is required.[32]
Hypotension and anaphylaxis are known complications of immunoglobulin therapy. Hence, healthcare providers should monitor vital signs, shortness of breath, and urticaria. Transfusion-related acute lung injury (TRALI) can occur within six hours of transfusion. Monitor for clinical signs and symptoms such as hypotension, vomiting, dyspnea, and hypoxemia, as TRALI requires immediate workup, cessation of infusion, and supportive treatment with close monitoring.[14]