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Immnunodeficiency
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- 3. whatisimmunodeficiency Immunodeficiency, also known asimmunocompromising, is a state in which the Immune system’s ability to fight infectious disease and cancer is compromised or entirely absent As a result, unusual bacterial, viral, or fungal infections or other cancers may develop
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- 5. Classificationofimmunodeficiency • Abnormalities ofB and T cells Specific • Abnormalities if non- specific components Non- specific
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- 8. Primary Immunodeficiency These disordersare usually present at birth and are genetic disorders that are usually hereditary. They become evident infancy or childhood. They can be caused by mutation of any gene involved in immune system development
- 9. 1. Humoral immunity 2.Cell mediated immunity 3. Combined immunodeficiency 4. Phagocytosis DeficienciesofDefence
- 10. 1. X- linkedAgammaglobulinemia 2. Transient hypogammaglobulinemia of infancy 3. Common variable immunodeficiency Humoraldeficiencies
- 11. X- linked Agammaglobulinemia • Describedby Bruton • It is only seen in male. The disease is not observable till 6 months of age. • Recurrent serious of infection with pyogenic bacteria . • Characterized by extremely low IgG levels and by the absence of other immunoglobulin classes. • X-LA is caused by a defect in Bruton’s tyrosine kinase (Btk), which is required for signal transduction through the BCR, resulting in arrest in the b cell development. • Treatment done by maintaining adequate amount of immunoglobulin. Done by administrating 300mg per kg of gamma globulin per kg of body weight.
- 12. Transient hypogammaglobulinemia of infancy • Thisdisease is caused due to an abnormal delay in the initiation of IgG synthesis in infants. • Maternal IgG is solely catabolised in the new born and reaches a level of 200 mg per 100 ml by second month • Treatment – done with gamma globulin
- 13. Common variable immunodeficiency (CVIDS) •This is also known as late onset hypogammaglobulinemia. As it usually manifest by the age of 15-35 years of age. • It is characterised by recurrent pyogenic infection and autoimmune disease. • Caused due to increased suppressor T cell and diminished helper T Cell activity. • Treatment - administration of gamma globulin intravenously and intramuscular
- 14. • Thymic Hypoplasia •Chronic mucocutaneous candidosis Cellularimmunodefiencies
- 15. Thymic Hypoplasia • Alsocalled DiGeorge syndrome. • It is a development defect involving the endodermal derivatives of pharyangeal pouches • It leads to aplasia or hypoalpasia of the thymus. • The immunodeficiency primarily involves cell mediated immunity. • The thymus dependent areas of the lymph nodes and spleen are depleted of lymphocytes. Circulating T cells are reduced in numbed • Treatment – vitamin D and C supplements, transplantation of thymus.
- 16. Chronic mucocutaneous candidosis •It caused due to an abnormal response to Candida albicans. • symptoms involve severe chronic candidosis of the mucosa, skin and nails. • Caused due to malfunctioning of T cells. • Intracellular killing if candida is defective . Treatment • Antifungal drug is applied to skin
- 17. 1. Ataxia telangiectasia 2.Wiskot- Aldrich syndrome Combinedimmunodefiencies
- 18. Ataxia telangiectasia • Itis a hereditary condition transmitted autosomal recessive mode. • Combined immunodeficiency is associated with cerebral ataxia and abnormal chromosome. • Usually noticed in infancy. • The majority of patients lack serum and secretory IgA and some posses antibody to IgA. • CMI is also defective resulting in delayed hypersensitivity and graft rejection. Treatment – antibiotics and immunoglobins
- 19. Wiskot- Aldrich syndrome •This is an X- linked disease characterised by eczema and recurrent infection. • Caused due to mutation in the X chromosome. • CMI undergoes progressive deterioration association with cellular depletion of the thymus. Treatment Stem cell transplantation Antibiotics
- 20. Causes maybe intrinsicor extrinsic defects. Intrinsic defects maybe due enzyme deficiency. Extrinsic defect maybe due to complement of factors promoting phagocytosis. Results in increase susceptibility to infections examples are 1. Chediak- Higashi syndrome 2. Tuftsin deficiency Phagocytosis
- 21. Chediak- Higashi syndrome •It is a genetic disorder • Decreased pigmentation of skin, eyes and hair. • The inclusion maybe the result of autophagocytic activity. • The leukocytes posses diminished phagocytic activity. • Pyogenic infections
- 22. Tuftsin Deficiency Tuftsin isa leukokinin capable of stimulating phagocytosis. It is a small tetrapepetide. Symptoms – local and systemic bacterial infection. Acquired tuftsin deficiency can occur in granulocyte leukemia, when blood neutrophils failed to show stimulation with synthetic tuftsin or with the serum leukokinin
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- 24. SecondaryImmunodeficiency These disorders generally developlater in life. They are more common than primary immunodeficiency. Example - Hypogammaglobulinemia, agent- induced immunodeficiency
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- 26. 1. Antibiotics 2. Immuneglobulin 3. Stem cell transplantation 4. Transplantation of thymus Treatment
- 27. References Overview of immunodeficiency disorders, James Fernanadez,MSD Manual, 2021 Ananthanarayan and Paniker’s textbook of Microbiology ,10th edition, 2017 pages 173-177 Kuby IMMUNOLOGY, OWEN, Punt, Stranford, 7th edition 2013 pages- 593-606 Immunodeficiency, Angel A. Justiz Vaillant; Ahmad Qurie., Statpearls,2020
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