PRIMARY AND SECONDARY IMMUNODEFICENCY

1. PRIMARY AND SECONDARY IMMUNODEFICENCY NWABUEZE ADAOBI PATRICIA WINDSOR UNIVERSITY MAY, 2014

2. Definition and overveiw • Immunodeficiency is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. • Most cases of immunodeficiency are acquired ("secondary") but some people are born with defects in their immune system, aka primary immunodeficiency.

3. TYPES BY AFFECTED COMPONENT SECONDARY PRIMARY

4. Pathogenesis • Primary Immunodeficiency is also known as congenital immunodeficiencies. Many of these disorders are hereditary and are autosomal recessive or X-linked. Primary disorders include: • X-linked agammaglobulinemia (XLA); mutation occurs at the Bruton's tyrosine kinase (Btk) gene via BCR • severe combined immunodeficiency (SCID disorders) aka alymphocytosis • common variable immunodeficiency

5. secondary • Secondary disorders happen when the body is attacked by an outside source, such as a toxic chemical or an infection,Severe burns and radiation, malnutrition, agingand particular medications such as (chemotherapy, immunosuppressive drugs after organ transplants, glucocorticoids). Secondary disorders include: • AIDS • cancers of the immune system, such as leukemia, lymphoma • immune complex diseases, such as viral hepatitis • multiple myeloma

7. pathophysiology They can be broadly classified into six groups based on the part of the immune system that's affected: • B cell (antibody) deficiencies • T cell deficiencies • Combination B and T cell deficiencies • Defective phagocytes • Complement deficiencies • Unknown (idiopathic)

8. Clinical features • increased susceptibility to infections. • infections that are more frequent, longer lasting or harder to treat than are the infections of someone with a normal immune system. • Increased chances of opportunistic infections. • Frequent and recurrent pneumonia, bronchitis, sinus infections, ear infections, meningitis or skin infections • Blood infections

9. Clinical presentation • Patients with immunodeficiency often look ill on presentation, with pale skin and a distended abdomen. Various skin manifestations may be apparent, such as rashes and eczema. • The eyes may be inflamed and infected. • Signs of chronic disease, such as scarred eardrums, encrusted nostrils and postnasal drip may be evident. • There may be a chronic cough. • Hepatomegaly and splenomegaly may be detected in the abdomen. • In infants, crusting around the anus may be a sign of chronic diarrhoea. Delayed development or ataxia may be present too.

10. Clinical presentation • Inflammation and infection of internal organs • Blood disorders, such as low platelet counts or anemia • Digestive problems, such as cramping, loss of appetite, nausea and diarrhea • Delayed growth and development • Autoimmune disorders, such as lupus, rheumatoid arthritis or type 1 diabetes

11. Prognosis Primary • Most primary immunodeficiencies are genetic and lifelong. Many patients have a normal lifespan, especially if the condition is diagnosed early and infections treated regularly.The prognosis in other conditions such as severe combined immunodeficiency is less optimistic. Patients suffer from chronic illness and require intensive treatment. Secondary • This depends on the underlying cause. Many conditions secondary to acute disease resolve when the underlying pathology is treated.

12. Investigation Screening tests can be done in primary care. These should include: • FBC, IgG, IgM and IgA levels and tests to confirm the presence and type of any infection. • An elevated ESR may indicate chronic. • Appropriate microbiological swabs should be taken. • assays of lymphocyte response, antibody response to immunisation of diphtheria, tetanus and pneumococcal polysaccharides, phagocytosis assay and quantitation of individual complement components

13. diagnosis • Blood test • Prenatal testing • Identifying disease • Bone marrow examination (remember multiple myeloma)

14. management • Antibiotic treatment; Some people need to take antibiotics long term to prevent infections from occurring and to prevent permanent damage to the lungs and ears. • Asymptomatic treatment; Example; such as ibuprofen for pain and fever, decongestants for sinus congestion, and expectorants to help clear your airways of mucus.

15. Management Because primary immune disorders are caused by genetic defects, there's no way to prevent them. But steps to prevent infections include: • Practice good hygiene • Dental care and check up • Eat right • Avoid exposure • Take medications and ask your doctor about vaccinations

16. Treatment • Treatments for primary immunodeficiency involve preventing and treating infections, boosting the immune system, and treating the underlying cause of the immune problem. • The treatment of immunodeficiencies also depends on the nature of the defect, and may involve antibody infusions, long-term antibiotics and (in some cases) stem cell transplantation.

17. Treatment Treatment to BOOST the immune system • Immunoglobulin therapy; (gamma globulin therapy) suitable for people who have an antibody deficiency could be injected IV or inserted underneath the skin (subcutaneous infusion). • Gamma interferon therapy; Gamma interferon is a man-made (synthetic) substance given as an injection in the thigh or arm three times a week. • Growth factors; Such as granulocyte-macrophage colony-stimulating factor (Leukine) and granulocyte colony-stimulating factor (Neupogen, Neulasta) help increase the levels of immune-strengthening white blood cells. Treatment to CURE would include stem cell transportation: Stem cells can be harvested through bone marrow, or they can be obtained from the placenta at birth (cord blood banking).

18. Secondary type is known that the current epidemics of AIDs and tuberculosis have caused global increases in the condition. Secondary immunodeficiency is common in people who are hospitalised for: • Lymphoreticular malignancy. • Drugs - particularly cytotoxic drugs and immunosuppressants. • Viruses, eg HIV. • Malnutrition - the most common cause worldwide. • Metabolic disorders, eg renal disease requiring peritoneal dialysis. • Trauma or major surgery. • Protein loss - for example, due to nephrotic syndrome.

19. High yield • Secondary immune deficiencies or acquired deficiencies, more frequent than primary immune deficiencies • Immunocompromised; Having an immune system that has been impaired by disease or treatment. • immunosuppressive Having the capability to suppress the immune system, capable of immunosuppression. • Immunodeficiency A depletion in the body's natural immune system, or in some component of it. • secondary infection; are especially nosocomial infection

20. Reference • Microbiology: A Systems Approach by Marjorie Kelly Cowan • Brock Biology of Microorganisms by Michael T. Madigan, John M. Martinko, David Stahl, David P. Clark • Microbiology: Principles and Explorationsby Jacquelyn G. Black • Foundations in Microbiology by Kathleen Park Talaro, Barry Chess • Prescott's Microbiology by Joanne Willey, Linda Sherwood, Chris Woolverton • Wikitionary, wikimedia, wikipedia

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