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fatal 6 t cell deficiency often causes secondary disorders such as acquired immune deficiency syndrome aids 7 granulocyte deficiency including decreased numbers of granulocytes called as granulocytopenia or if absent agranulocytosis such as of neutrophil granulocytes termed neutropenia granulocyte deficiencies also include decreased function of individual granulocytes such as in chronic granulomatous disease asplenia where there is no function of the spleen complement deficiency is where the function of the complement system is deficient in reality immunodeficiency often affects multiple components with notable examples including severe combined immunodeficiency primary and acquired immune deficiency syndrome secondary comparison of immunodeficiencies by affected component affected components main causes 8 main pathogens of resultant infections 8 humoral immune deficiency b cell deficiency b cells plasma cells or antibodies primary humoral multiple myeloma chronic lymphoid leukemia aids streptococcus pneumoniae hemophilus influenzae pneumocystis jirovecii giardia intestinalis cryptosporidium parvum t cell deficiency t cells marrow and other transplantation aids cancer chemotherapy lymphoma glucocorticoid therapy intracellular pathogens including herpes simplex virus mycobacterium listeria 9 and intracellular fungal infections 8 neutropenia neutrophil granulocytes chemotherapy bone marrow transplantation dysfunction such as chronic granulomatous disease enterobacteriaceae oral streptococci pseudomonas aeruginosa enterococcus species candida species aspergillus species asplenia spleen splenectomy trauma sickle cell anemia polysaccharide encapsulated bacteria 10 particularly streptococcus pneumoniae 10 haemophilus influenzae 10 neisseria meningitidis 10 plasmodium species babesia species complement deficiency complement system congenital deficiencies neisseria species streptococcus pneumoniae primary or secondary edit the distinction between primary and secondary immunodeficiencies is based on whether the cause originates in the immune system itself or insufficiency of a supporting component or an external factor primary immunodeficiency edit main article primary immunodeficiency several rare diseases increase susceptibility to infections from childhood onward primary immunodeficiency is also known as congenital immunodeficiency 11 many of these disorders are hereditary and are autosomal recessive or x linked there are over 95 recognised primary immunodeficiency syndromes they are generally grouped by the part of the immune system that is malfunctioning such as lymphocytes or granulocytes 12 the treatment of primary immunodeficiencies depends on the nature of the defect it may involve antibody infusions long term antibiotics and in some cases stem cell transplantation the characteristics of lacking and or impaired antibody functions can be related to illnesses such as x linked agammaglobulinemia and common variable immune deficiency 13 secondary immunodeficiencies edit further information immunosuppression secondary immunodeficiencies also known as acquired immunodeficiencies can result from various immunosuppressive agents for example malnutrition aging particular medications e g chemotherapy disease modifying antirheumatic drugs immunosuppressive drugs after organ transplants glucocorticoids and environmental toxins like mercury and other heavy metals pesticides and petrochemicals like styrene dichlorobenzene xylene and ethylphenol for medications the term immunosuppression generally refers to both beneficial and potential adverse effects of decreasing the immune system s functions the term immunodeficiency generally refers solely to the adverse effect of increased risk of infection many specific diseases directly or indirectly cause immunosuppression this includes many types of cancer particularly those of the bone marrow and blood cells leukemia lymphoma multiple myeloma and certain chronic infections immunodeficiency is also the hallmark of acquired immunodeficiency syndrome aids 11 caused by the human immunodeficiency virus hiv hiv directly infects a small number of t helper cells and also impairs other immune system responses indirectly various hormonal and metabolic disorders can also result in immune deficiency including anemia hypothyroidism and hyperglycemia smoking tobacco heavy alcohol use and substance use also depress the immune response heavy schedules of training and competition in athletes increases their risk of immune deficiencies 14 causes edit the cause of immunodeficiency varies depending on the nature of the disorder the cause can be either genetic or acquired by malnutrition and poor sanitary conditions 15 16 only for some genetic causes the exact genes are known 17 immunodeficiency and autoimmunity edit this section needs more citations please help improve this section by adding citations to reliable sources unsourced material may be challenged and removed november 2016 learn how and when to remove this message several immunodeficiency syndromes present clinical and laboratory characteristics of autoimmunity the decreased ability of the immune system to clear infections in these patients may be responsible for causing autoimmunity through perpetual immune system activation 18 one example is common variable immunodeficiency cvid where multiple autoimmune diseases are seen e g inflammatory bowel disease autoimmune thrombocytopenia and autoimmune thyroid disease familial hemophagocytic lymphohistiocytosis an autosomal recessive primary immunodeficiency is another example low blood levels of red blood cells white blood cells and platelets rashes lymph node enlargement and enlargement of the liver and spleen are commonly seen in these patients the presence of multiple uncleared viral infections due to a lack of perforin is thought to be responsible in addition to chronic and or recurrent infections many autoimmune diseases including arthritis autoimmune hemolytic anemia scleroderma and type 1 diabetes are also seen in x linked agammaglobulinemia xla recurrent bacterial and fungal infections and chronic inflammation of the gut and lungs are seen in chronic granulomatous disease cgd cgd is caused by a decreased production of nicotinamide adenine dinucleotide phosphate nadph oxidase by neutrophils hypomorphic rag mutations are seen in patients with midline granulomatous disease an autoimmune disorder commonly occurring in patients with granulomatosis with polyangiitis and nk t cell lymphomas wiskott aldrich syndrome was patients also present with eczema autoimmune manifestations recurrent bacterial infections and lymphoma in autoimmune polyendocrinopathy candidiasis ectodermal dystrophy apeced autoimmunity and infections coexist organ specific autoimmune manifestations e g hypoparathyroidism and adrenocortical failure and chronic mucocutaneous candidiasis finally iga deficiency is sometimes associated with developing autoimmune and atopic phenomena antibody vulnerability period in children edit the period following birth is critical for the development of a child s immune system initially a newborn relies heavily on passive immunity transferred from the mother primarily through the placenta and breastfeeding as breastfeeding frequency declines immune protection gradually wanes making the child more vulnerable and increasingly reliant on their developing immune system this transitional phase known as the antibody vulnerability period lasts until approximately three to four years of age during which the child s immune system matures and becomes fully functional 19 to combat pathogens babies need to develop their own specific antibodies recognizing these antigens and these types of antibodies are known as immunoglobulins immunoglobulin g igg is one of them babies are unable to make their own igg antibodies at birth and rely on maternal transfer of igg via the placenta during the third trimester other types of immunoglobulins iga igm ige and igd do not cross the placenta it is believed that igg is important in protecting babies against infections 20 naturally bioactive immunoglobulin g is found in breast milk which plays a significant role in early life during the vulnerable period the y shaped structure of immunoglobulin g allows it to effectively identify and combat pathogens providing antibody like protection to the child 21 research indicates that maintaining adequate igg levels during early childhood may help mitigate the risks associated with this immune vulnerability this supplementation can offer a protective boost enhancing the infant s ability to fend off infections and other health threats during the critical years when their immune system is still developing the importance of this period underscores the need for targeted nutritional interventions to support overall immune health in young children classes of immunoglobulins igs edit the immune system produces several classes of immunoglobulins ig such as iga igd ige igg and igm each class helps protect the body from infection in a different way see below 22 immunoglobulin subclasses and their properties edit immunoglobulin subclasses subclass structure antigen binding sites crosses placenta total antibody in serum fc binds to functions igm pentamer 10 no 6 complement main antibody of primary responses best at fixing complement monomer form serves as b cell receptor igg monomer 2 yes 80 phagocytes main blood antibody of secondary responses neutralize toxins opsonization all antibodies are y shaped only some are dimers siga or pentamers igm the y shaped structure has sites that effectively identify and bind pathogens able to suppress more than 99 of the antibody response against the bound antigen 23 iga dimer 4 no 13 none secreted into mucus tears saliva and colostrum ige monomer 2 no 0 002 mast cells and basophils antibody of allergy and antiparasitic activity igd monomer 2 no 1 none b cell receptor treatment edit available treatment falls into two modalities treating infections and boosting the immune system prevention of pneumocystis pneumonia with trimethoprim sulfamethoxazole is useful in those who are immunocompromised 24 in the early 1950s immunoglobulin ig was used by doctors to treat patients with primary immunodeficiency through intramuscular injection ig replacement therapy infusions can be either subcutaneous or intravenously administered resulting in higher ig levels for about three to four weeks although this varies with each patient 13 prognosis edit prognosis depends greatly on the nature and severity of the condition some deficiencies cause early mortality before age one others with or even without treatment are lifelong conditions that cause little mortality or morbidity newer stem cell transplant technologies may lead to gene based treatments of debilitating and fatal genetic immune deficiencies the prognosis of acquired immune deficiencies depends on avoiding or treating the causative agent or condition like aids see also edit acquired immune deficiency syndrome aids immune disorder autoimmune disease immune response to self proteins allergy immune response to harmless non self proteins histamine immunosenescence age associated immune deficiency steroids commonly administered drugs like prednisone that suppress the immune system human genetic enhancement immune system immunology references edit chinen j shearer wt february 2010 secondary immunodeficiencies including hiv infection the journal of allergy and clinical immunology 125 2 suppl 2 s195 203 doi 10 1016 j jaci 2009 08 040 pmc 6151868 pmid 20042227 primary immunodeficiency mayo clinic 30 january 2020 retrieved 13 may 2020 meidani mohsen naeini alireza emami rostami mojtaba sherkat roya tayeri katayoun march 2014 immunocompromised patients review of the most common infections happened in 446 hospitalized patients journal of research in medical sciences 19 suppl 1 s71 s73pmc 4078380 pmc 4078380 pmid 25002900 lee ainsley ryan yan bin wong shi yin chai louis yi ann lee soo chin lee matilda xinwei muthiah mark dhinesh tay sen hee teo chong boon tan benjamin kye jyn chan yiong huak sundar raghav soon yu yang 2 march 2022 efficacy of covid 19 vaccines in immunocompromised patients systematic review and meta analysis bmj 376 e068632 doi 10 1136 bmj 2021 068632 pmc 8889026 pmid 35236664 zbinden delphine manuel oriol february 2014 influenza vaccination in immunocompromised patients efficacy and safety immunotherapy 6 2 131 139 doi 10 2217 imt 13 171 pmid 24491087 greenberg s immunodeficiency university of toronto archived from the original on 10 july 2013 schwartz ra 2019 10 22 jyonouchi h ed t cell disorders medscape 1 2 3 if not otherwise specified in boxes then reference for entries is page 432 chapter 22 table 22 1 in jones j bannister ba gillespie sh 2006 infection microbiology and management wiley blackwell isbn 978 1 4051 2665 6 page 435 in jones j bannister ba gillespie sh 2006 infection microbiology and management wiley blackwell isbn 978 1 4051 2665 6 1 2 3 4 brigden ml february 2001 detection education and management of the asplenic or hyposplenic patient american family physician 63 3 499 506 508 pmid 11272299 1 2 basic immunology functions and disorders of the immune system 3rd ed 2011 rosen fs cooper md wedgwood rj august 1995 the primary immunodeficiencies the new england journal of medicine 333 7 431 40 doi 10 1056 nejm199508173330707 pmid 7616993 s2cid 39699189 1 2 immune deficiency foundation primaryimmune org retrieved 2017 04 17 gleeson michael nieman david c pedersen bente k january 2004 exercise nutrition and immune function journal of sports sciences 22 1 115 125 doi 10 1080 0264041031000140590 pmid 14971437 s2cid 84378380 nutrition and immunity the nutrition source harvard t h chan school of public health may 2020 retrieved 8 november 2020 bourke cd berkley ja prendergast aj 2016 immune dysfunction as a cause and consequence of malnutrition trends in immunology 37 6 386 398 doi 10 1016 j it 2016 04 003 pmc 4889773 pmid 27237815 charles a janeway jr travers paul walport mark shlomchik mark j 2001 inherited immunodeficiency diseases immunobiology garland science grammatikos ap tsokos gc february 2012 immunodeficiency and autoimmunity lessons from systemic lupus erythematosus trends in molecular medicine 18 2 101 8 doi 10 1016 j molmed 2011 10 005 pmc 3278563 pmid 22177735 langel stephanie n blasi maria permar sallie r 2022 05 11 maternal immune protection against infectious diseases cell host microbe 30 5 660 674 doi 10 1016 j chom 2022 04 007 issn 1931 3128 pmid 35550669 transient hypogammaglobulinemia infancy immune deficiency foundation primaryimmune org retrieved 2025 01 09 langel stephanie n blasi maria permar sallie r 2022 05 11 maternal immune protection against infectious diseases cell host microbe 30 5 660 674 doi 10 1016 j chom 2022 04 007 issn 1931 3128 pmid 35550669 aryal sagar 2015 05 01 antibody structure classes and functions microbiology ...