Immune deficiencies
21 important questions on Immune deficiencies
Why are immuno deficiencies more common in males?
When do we speak of a real indication of immunodeficiency in children?
Indication for ID when:
- >10 otitis media a year, >2 pneumonia a year.
- >2 severe (life threathening) infections per lifetime.
- Family history of ID
- Infections caused by unusual organisms (or when response to an organism is unusual).
What are the symptoms of phagocytic disorders en what kind of infections are most common?
Common infections are: mycobacteria, aspergilles, candida, catalase positive microorganisms.
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Which phagocytic diseases do you know, what are the characteristics and how do they cause immunodeficiency?
- Chronic Granulomatous Disease: 70% xlinked, defect in NADPH oxidase, no ROS formation for microbe killing and protease activation, susceptible for catalasepositive bacteria.
- Leukocyte Adhesion Defect: leukocytes cant enter the tissue; recurrent skin infections, severe periodontal disease (tooth loss), delayed woond healing, leukocytosis (elevated leukocyte levels), leading to candidiasis (schimmelinfectie mond)
Which gene defects can cause Leukocyte Adhesion Deficiency?
- LADI: mutation in beta chain of integrin LFA1; reduced binding of mononuclear cells to the endothelial cells: no adhesion (and no extravasation).
- LADII: absence of sugar ligands for selectine beacause of defect in fucosetransporter gene: no roling.
- LADIII: activation of integrins is defective (activation and 3d transition does not occur): no adhesion.
Summary the development of a naive bcel to a IgM plasma cell
Affinity maturation by somatic hyper mutation in the lymph nodes (also tcell dependent_ "perfect" plasmacells migrate from the lymphnode and are able to produce specific antibodies.
Explain the difference between the primary and secundary (bcell) respons
Secondary respons: memory IgG cells can directly be activated by antigen; + SHM due to tcell interaction = a lot of IgG with high affinity.
Explain what primary B cell deficiencies are and appoint the clinical signs
- Disorders characterized by reduction and/or change in composition or absence of serum Ig (antibodies).
- Clinical signs are; recurrent sepsis and bacterial infections with encapsulated (kapsel) bacteria (opsonisation is defect!), bronchiectasia (widening of breathing tubes due to damage caused by inections) leading to cough, chronic gastroenteritis (darm en maagontstekingen), failure to thrive (groei/ontwikkeling verstoring).
What categories of primary bcell deficiencies do you know?
- Defect in early bcell development
- Class switch recombination defects
- Common variable immunodeficiencies
What is the underlying mechanism of early b cell development defects? What is an example of a early bcel development disease?
X linked agammaglubulinaemia: mutation in Btk results in a pre-BCR signalling defect, leading to failure to produce mature b lymphocytes and Ig.
What is the underlying mechanism of class switch and somatic hypermutation? What is an example of a disease?
X-linked hyper IgM syndrome (HIGM1): mutation in CDL40 ligand on tcells, leading to 1) only IgM, 2) no class switch 3) no somatic hypermutations.
What is the mechanism of cell mediated defects?
X-linked SCID: deficiency in common gammachain.; present in IL receptors; defective cytokine signaling (tcells, bcells and nk cells).
Treatment of bcell, tcell and phagocytic defects:
What is the definition of secondary immunodeficiencies and what kind of infections are caused by secondarty immunodeficiencies?
- Defects in Ig/complement/phagocytic cells leads to pyogenic bacterial infections.
- Defects in cell mediated immunity leads to viral, fungi and intracellular microorganism infections. (tcells viral/fungi, bcells bacterial)
What are causes of secundary immunodeficiency?
The immune system is supressed due to...
- Infection (HIV, measles)
- Iatrogenic (immune blockers and stem cell transplantation)
- Malignancy (cancer)
- Biochemical/nutritional disorders (diabetic, renal insufficiency)
- Other conditions (trauma, stress)
What is the difference between immune deficiency and auto immune disease?
Immune def: system works too little
What are the three mechanisms immune deficiency?
- Killing of cells (low numbers)
- Disrupted function (recognition)
- Disrupted localization of cells
Explain how aging influences immune deficiencies
Children: developing memory
Eldery: frail immunosenecense; sensitivity for disease increase and vaccination loses effect.
The neonatal immune system is in a continues state of tolerance (less cytokines, only maternal Ig, less tcell activity, less complement).
- Probably due to a combination of immaturity and active suppression.
- This tolerance is there to prevent immunopathology upon contact with new pathogens.
- Nucleated erythrocytes are only demonstrated in neonates and actively suppres the immune system
Explain how immunosupressive drugs can cause immune deficiencies. When are those drugs used?
- Glycocorticoids: Inhibit inflammation.
- Calcineurine (small molecule): Hinders tcel activation
- Inhibitors of nucleotide syntheses: no proliferation (like chemo).
- Antimetabolites: these are analogs for nucleotides; also no proliferation
- Fingolimod: hinders localization of immune cells
- Protein drugs: depleting antibodies, nondepleting antibodies, intravenous Ig
Explain the mechanism of multiple sclerosis. What is possible as treatment?
What is a risk of this treatment?
Fingolimod: Tcell does not express S1P1receptor anymor; this treatment ensures that this receptor cant be recycled but is downregulated in proteasomes --> Tcell cant leave the lymph nodes.
Other option: Alemtuzumab; inhibits active tcells.
Risk of fingolimod: immunodeficieny!: reactivation of the normally latent virus "JC" in the brain, because Tcells cant reach the brain anymore.
Explain how bone marrow supression (secondary to chemo, due toradiotherapy or due to infiltration of malignant cells into the bone marrow) leads to immune deficiency. Which deficiency?
Dus beenmerg remmen kan door chemo of door de kankercellen zelf...
Stopping chemo if infection is severe; quick reconstiitution of neutrophils.
Reconstitution of tcells is slower; so log after cancer treatment higher risk on viral and fungi infections.
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