1 / 17

Immunodeficiency

Immunodeficiency. Paula O’Leary CP4004 Lecture Nov 2010. Immune Defence – the challenges. Microbial Challenges On us, in us, around us Variable structure; activity; pathogenecity. Host defence – protective measures. Non-Immunological Physical Functional Chemical Biological

arden
Download Presentation

Immunodeficiency

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  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

More Related