Chapter 19 Immunodeficiency Questions What is immunodeficiency? How can immunodeficiency occur? What clinical problems can immunodeficiency create? Immunodeficiency refers to a state in which the immune system does not work with full efficiency, leading primarily to an increased risk of infections. Since the immune system also detects and destroys some tumour cells, patients with immunodeficiency may also be at increased risk of neoplasia. For example, patients on long-term immunosuppression following a solid organ transplant such as a kidney are at increased risk of developing cutaneous squamous cell carcinoma and Epstein–Barr virus-driven lymphoproliferative disorder (a virally triggered proliferation of immature lymphoid cells that behaves in a similar way to lymphoma). Causes of immunodeficiency There are many reasons why immunodeficiency may occur. Some of these are congenital (primary) and, therefore, affect neonates and children while others may be acquired (secondary) during childhood or adulthood. Primary causes: common variable immunodeficiency severe combined immunodeficiency chronic granulomatous disease specific immunoglobulin deficiencies. Secondary causes: malnutrition serious systemic disease: cancer, renal disease, conditions resulting in bone marrow destruction or interference with the normal function of marrow (e.g. leukaemia) iatrogenic: chemotherapy, post-transplant anti-rejection drugs, immunosuppressive drugs for chronic inflammatory conditions (e.g. connective tissue diseases, inflammatory bowel disease) infective: HIV infection splenectomy: traumatic or secondary, for example to sickle cell disease. Iatrogenic causes of immunodeficiency are important and relatively common, especially with the increasing range of chemotherapeutic drugs available for neoplasia, immunosuppressive therapies for chronic inflammatory diseases, and increasing numbers of organ transplants. Primary immunodeficiency Primary immunodeficiencies vary in clinical severity from conditions that, if untreated, are incompatible with life (e.g. severe combined immunodeficiency) to conditions that may not require specific treatment (e.g. common variable immunodeficiency). They may predominantly affect one component of the immune system (e.g. hypogammaglobulinaemia, in which one or more class of antibody may be affected, or chronic granulomatous disease, in which the ability to kill phagocytosed microorganisms such as bacteria is impaired) or may affect the functioning of both the humoral and cellular arms of the immune system (e.g. severe combined immunodeficiency). However, almost all forms will be associated with increased risk of infections. Accurate diagnosis of an immunodeficiency syndrome usually requires detailed specialist testing of the components of the immune system. However, some are associated with characteristic changes within tissue biopsies. For example, common variable immunodeficiency is often associated with the presence of Giardia lamblia parasites on duodenal biopsy. The more serious forms of primary immunodeficiency may be treatable by bone marrow transplantation. Less severe types (e.g. hypogammaglobulinaemia) may only be diagnosed as a result of specific investigations performed to investigate recurrent infections such as pneumonia.< div class='tao-gold-member'> Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: lung disease big picture heart disease disease and congenital heart disease and epidemiology of neoplasia Stay updated, free articles. Join our Telegram channel Join Tags: The Flesh and Bones of Pathology Jul 3, 2016 | Posted by admin in MUSCULOSKELETAL MEDICINE | Comments Off on Immunodeficiency Full access? Get Clinical Tree
Chapter 19 Immunodeficiency Questions What is immunodeficiency? How can immunodeficiency occur? What clinical problems can immunodeficiency create? Immunodeficiency refers to a state in which the immune system does not work with full efficiency, leading primarily to an increased risk of infections. Since the immune system also detects and destroys some tumour cells, patients with immunodeficiency may also be at increased risk of neoplasia. For example, patients on long-term immunosuppression following a solid organ transplant such as a kidney are at increased risk of developing cutaneous squamous cell carcinoma and Epstein–Barr virus-driven lymphoproliferative disorder (a virally triggered proliferation of immature lymphoid cells that behaves in a similar way to lymphoma). Causes of immunodeficiency There are many reasons why immunodeficiency may occur. Some of these are congenital (primary) and, therefore, affect neonates and children while others may be acquired (secondary) during childhood or adulthood. Primary causes: common variable immunodeficiency severe combined immunodeficiency chronic granulomatous disease specific immunoglobulin deficiencies. Secondary causes: malnutrition serious systemic disease: cancer, renal disease, conditions resulting in bone marrow destruction or interference with the normal function of marrow (e.g. leukaemia) iatrogenic: chemotherapy, post-transplant anti-rejection drugs, immunosuppressive drugs for chronic inflammatory conditions (e.g. connective tissue diseases, inflammatory bowel disease) infective: HIV infection splenectomy: traumatic or secondary, for example to sickle cell disease. Iatrogenic causes of immunodeficiency are important and relatively common, especially with the increasing range of chemotherapeutic drugs available for neoplasia, immunosuppressive therapies for chronic inflammatory diseases, and increasing numbers of organ transplants. Primary immunodeficiency Primary immunodeficiencies vary in clinical severity from conditions that, if untreated, are incompatible with life (e.g. severe combined immunodeficiency) to conditions that may not require specific treatment (e.g. common variable immunodeficiency). They may predominantly affect one component of the immune system (e.g. hypogammaglobulinaemia, in which one or more class of antibody may be affected, or chronic granulomatous disease, in which the ability to kill phagocytosed microorganisms such as bacteria is impaired) or may affect the functioning of both the humoral and cellular arms of the immune system (e.g. severe combined immunodeficiency). However, almost all forms will be associated with increased risk of infections. Accurate diagnosis of an immunodeficiency syndrome usually requires detailed specialist testing of the components of the immune system. However, some are associated with characteristic changes within tissue biopsies. For example, common variable immunodeficiency is often associated with the presence of Giardia lamblia parasites on duodenal biopsy. The more serious forms of primary immunodeficiency may be treatable by bone marrow transplantation. Less severe types (e.g. hypogammaglobulinaemia) may only be diagnosed as a result of specific investigations performed to investigate recurrent infections such as pneumonia.< div class='tao-gold-member'> Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: lung disease big picture heart disease disease and congenital heart disease and epidemiology of neoplasia Stay updated, free articles. Join our Telegram channel Join
