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Immunodeficiency

Immunodeficiency

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Immunodeficiency

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  1. Immunodeficiency Paula O’Leary CP4004 Lecture Nov 2010

  2. Immune Defence – the challenges • Microbial Challenges • On us, in us, around us • Variable structure; activity; pathogenecity

  3. Host defence – protective measures • Non-Immunological • Physical • Functional • Chemical • Biological • Immunological • Innate / Non-Specific recognition of non-self • Specific / Adaptive

  4. Immune Defense Overview

  5. Immunodeficiency / Immunocompromised States Primary • Intrinsic abnormality of one or more components of the Immune System • >130 Conditions Characterised • Individually, uncommon, but important to recognise • Range from global, overwhelming immune failure ( SCID) to subtle defects in individual components of function • Opportunistic; Disproportionately Severe; Recurring Infections; Immune dysregulation; Malignancies • 1/ 10,000 live births

  6. Primary Immunodeficiencies – type and causes • SCID / CID – T cells (and B cells) • Il 2 common gR; ADA, AK RAG, CD40Ligand • Antibody deficiencies – B cells • CVID, XLA; Hyper IgM; Specific Antibody Deficiency • Immunodeficiency with Immune Dysregulation • APECED ( AIRE); IPEX; ALPS; Hyper IgE • Innate Defects • Phagocyte (neutropaenia); CGD; LAD • Complement • Tlr defects • Il – 12 / IFN - gamma

  7. Likely Deficiency • B cells – pyogenic infections; resp tract; any age • T cells – broad ranging; opportunistic; disseminated; early onset • Neutrophils – skin; mucosae; bacterial and fungal; deep-seated abcesses • Complement – Neisseria; pyogenic • Cytokine defects – TB; disseminated intracellular bacteria

  8. 8 or more ear infections within 1 year Recurrent, deep skin or organ abscesses 6 1 2 or more serious sinus infections within 1 year Persistent candidiasis after the age of 1 year 7 2 2 or more months on antibiotics without resolution Need for I.V. antibiotic to clear infections 8 3 2 or more pneumonias within 1 year 2 or more deep-seated infections 9 4 Failure to gain weight or grow normally A family history of Primary Immunodeficiency 10 5 The Ten Warning Signs of Primary Immunodeficiency

  9. Likely Deficiency and Investigation • B cells – Igs, Electrophoresis, (BJP), IgG subclasses, Specific Antibodies; Immunisation challenges; Specialised studies • T cells – total lymphocyte numbers, phenotyping; genetics; functional analysis • Neutrophils – numbers; function assays • Complement – CH50; individual components • Cytokine defects – specialised studies - refer

  10. PID - treatments • Immunoglobulin replacement • Bone Marrow Transplantation • Gene-based therapies • Antimicrobial management and prophylaxis • Nutritional Support • Patient Support Groups

  11. Immunodeficiency / Immunocompromised States Secondary • Dysfunction of one or more components of the Immune System arising as a result of another process • Much more common; accumulation of defects leading to clinical immunocompromise • More difficult to measure in the laboratory

  12. Infections • HIV • Pneumocystis jiroveci infection • Mycobacterial Infections • Toxoplasmosis • CMV infection • CNS lymphoma • Kaposi Sarcoma • Tissue Infection – GI; CNS

  13. Haematological Malignancies • Lymphoid • Multiple Myeloma • Chronic Lymhocytic Leukaemia • Lymphoma • Treatments for these disorders

  14. Medical Treatments • Corticosteroid therapy • Widely used in the management of inflammatory and autoimmune diseases; transplant patients • Monocyte; Lymphocyte Inhibition and Prostaglandin Inhibition • Infection more widespread and less clearcut in presentation

  15. Medical Management cont./ • “Crude” Immunosuppressants – AZA; MTX; 6-MP; Cyclophosphamide; Radiotherapy • Bone Marrow Suppression • Neutropaenia; Lymphopaenia • Careful dose calculation and patient monitoring • Targeted cell Immunosuppressants – Cyclosporin, Tacrolimus • Opportunistic Infections; Malignancies • Targetted Biologicals – Rituximab; Infliximab; CAMPath-1H; Netalizumab • Risks specific to interrupted immune component

  16. Medical States • Splenectomy • OPSI; Malaria; Salmonellosis • Immunisation; Antibiotic Prophylaxis • Poor Nutrition; Surgery / Trauma / ICU • Recognition and Correction • Protein Losing States • Renal Disease • Extremes of Age

  17. Care of Immunocompromised Patients • Vigilance and Monitoring • Prophylaxis • Pneumocystis prophylaxis; Immunisation • Limitation of Immune-suppressing Agent • Patient Education • Medical Carer Education