Immunodeficiency and Immunotherapy

Module details

Title

Immunodeficiency and Immunotherapy

Type

Specialist

Module code

SLS137

Credits

10

Requirement

Compulsory

Aim of this module

This module will provide the trainee with knowledge and understanding of the causes of immunodeficiency. They will understand the clinical presentation and investigation of a range of immunodeficient conditions and the principles and practice of immunotherapy. They will become familiar with methods and strategies to investigate immunodeficiency and gain experience of the interpretation of patient results in a variety of clinical settings.

Competencies

#	Learning outcome	Competency	Action
# 1	Learning outcome 1	Competency Select the appropriate immunological assay to investigate whether the immune system of the patient is immunocompetent or immunodeficient. To include all of the following: immunoglobulins and subclasses neutrophil function tests lymphocyte function tests specific antibody responses to vaccine antigens complement activity cytokine production molecular assays to define immunodeficiencies.	Action View
# 2	Learning outcome 2	Competency Perform the appropriate investigation of immunodeficiency to include all specified. To include all of the following: immunoglobulins and subclasses neutrophil function tests lymphocyte function tests specific antibody responses to vaccine antigens complement activity cytokine production molecular assays to define immunodeficiencies.	Action View
# 3	Learning outcome 3	Competency Interpret immunological data in the light of clinical details on patients with primary immunodeficiency, including: deficiencies of innate immunity B lymphocyte deficiencies T lymphocyte deficiencies combined T and B cell defects.	Action View
# 4	Learning outcome 3	Competency Interpret immunological data in light of clinical details on patients with secondary immunodeficiency, including all of the following: iatrogenic neoplasia infection.	Action View
# 5	Learning outcome 3	Competency Interpret immunological data in light of clinical details.	Action View
# 6	Learning outcome 4	Competency Apply the appropriate immunotherapeutic strategies/treatment regimens for patients with a range of primary and secondary immunodeficiencies.	Action View
# 7	Learning outcome 3,4	Competency Interpret, assess the effectiveness of and report results from patients receiving a range of immunotherapies, including at least three of the following: antibodies as immunosuppressive agents immunosuppressive drugs physical methods of immunosuppression cytokines and anti-cytokines intravenous immunoglobulins immunisation against infection novel approaches to autoimmunity, i.e. infliximab and rituximab stem cell therapies, specifically for immunodeficiencies.	Action View
# 8	Learning outcome 5	Competency Identify relevant clinical investigations from other disciplines that will impact on the interpretation of the immunological investigation.	Action View

Assessments

You must complete:

• 2 case-based discussion(s)
• 2 of the following DOPS/ OCEs:

Perform analysis of a patients serum to determine levels of immunoglobulins IgG IgM IgA	DOPS
Perform the flow cytometric assay to determine the number of CD4 T cells	DOPS
Perform the assay to determine the level of anti tetanus Or anti pneumococcus Or anti Hib IgG antibodies in a patient serum sample	DOPS
Perform the assay to determine the C3 and C4 levels in a patient sample	DOPS
Perform a lymphocyte proliferation assay	DOPS
Perform a neutrophil functional assay	DOPS
Perform the C100 or C50 assay to see if the classical pathway of complement is working in a patient	DOPS
Perform the AP50 AP100 assay to see if the alternative pathway of complement is working in a patient	DOPS
Perform TCR V beta analysis	DOPS
Take a history where the patient is suspected of having an immunodeficiency disorder	OCE
Describe to a patient with CVID XLA other antibody deficiency why you need to take EDTA blood to examine the B and T cells or measure immunoglboulins	OCE
Observe and then report to the clinician on taking blood for immunoglobulin measurement or for B and T cells from a patient	OCE
Discuss with a patient undergoing intravenous subcutaneous immunoglobulin therapy about this treatment and how it is helping them	OCE
Attend an MDT meeting of clinical immunologists and present the results of patient testing	OCE
Attend an outpatient clinic within any discipline gastro rheumatology dermatology renal etc where the patient may have an immunological dysfunction as part of their disease	OCE
Obtain a patient history from a normal volunteer or typical patient referred to your service and present the findings to a colleague or peer	OCE

Learning outcomes

1. Select immunology tests for the diagnosis and management of immunodeficiency and monitoring of immunotherapy.
2. Perform clinical and laboratory investigation of immunodeficiency.
3. Interpret and report results of investigation of immunodeficiency in the correct clinical context.
4. Apply immunotherapeutic strategies for inpatients with a range of primary and secondary immunodeficiencies.
5. Work in partnership with other clinical specialisms in the investigation of immunodeficiency and immunotherapy.

Learning outcomes

1. Discuss the clinical implications of immunodeficiency and the primary and secondary causes of immunodeficiency.
2. Explain the role of the humoral and cellular components of the immune system in immunodeficiency.
3. Describe the design, operation and performance of laboratory tests and assays used to investigate and define immunodeficiency.
4. Explain the principles of immunotherapy.
5. Describe and monitor the impact of immunotherapeutic treatments.
6. Discuss and justify appropriate immunotherapeutic strategies/treatment regimens for patients with a range of primary and secondary immunodeficiencies.
7. Describe the partnership between the clinical immunology laboratory and other clinical specialisms in the investigation of immunodeficiency and immunotherapy and patient care.

Indicative content

• Assessing immune function (T lymphocytes; B lymphocytes; phagocytes; complement)
• Deficiencies of innate immunity (phagocytic cell defects; leukocyte adhesion defects; complement system defects)
• B lymphocyte deficiencies (X-linked agammaglobulinaemias; selective IgA deficiency; IgG subclass deficiency; common variable immunodeficiency; transient hypogammaglobulinaemia of infancy; selective specific antibody deficiencies)
• T lymphocyte deficiencies (Di George syndrome; Ommen’s syndrome; bare lymphocyte syndrome; X-linked hyper IgM syndrome; severe T cell deficiencies [X-linked recessive form; adenosine deaminase (ADA) deficiency; purine nucleoside phosphorylase (PNP) deficiency])
• Combined T and B cell defects
  • Severe combined immunodeficiency (SCID) (autosomal recessive SCID; T cell receptor immunodeficiency; MHC Class II deficiency; IL-2 production defect)
  • Wiskott-Aldrich syndrome
• Secondary immunodeficiencies (iatrogenic; neoplasia; infection)
• Cytokine defects
• Human immunodeficiency virus (HIV) and AIDS
  • Pathogenesis of HIV infection
  • Epidemiology, prevalence and modes of transmission
  • Laboratory abnormalities in HIV infection
  • Management of HIV infection (drug therapies; vaccines)
• Immunotherapy
  • Antibodies as immunosuppressive agents (plasmapheresis and plasma exchange; monoclonal antibody therapy; generation of antibodies; ‘magic bullet’ therapy)
  • Immunosuppressive drugs (corticosteroids; cyclosporin and tacrolimus; other anti-inflammatory agents)
  • Other immunosuppressive agents (X-irradiation; ultraviolet light)
  • Cytokines and anti-cytokines (interleukin-1; interleukin-2; interferons; tumour necrosis factors [TNF]; Th1/Th2 balance)
  • Immune modulation by intravenous immunoglobulins
  • Immune potentiation (hormones; cytokine therapy; gene therapy)
  • Other uses of monoclonal antibodies
  • Stress and the immune system (psycho-neuro-endocrino-immune pathway)
  • Immunisation against infection (adjuvants; routine immunisations; travel immunisations; passive immunisation; new vaccines)
  • Cancer immunotherapy
  • Novel approaches to autoimmune disease (T cell vaccines; oral tolerance)
  • Other approaches (lymphocyte vaccination; blocking T cell-adenomatous polyposis coli [APC] interactions; gene repair; patient specific amplification of cytotoxic cells; stem cell therapies)

Activities

• Attend outpatient clinics where patients with a range of primary and secondary immunodeficiencies are treated and reflect on the efficacy of each treatment strategy, the side effects and effect on quality of life, and prepare some case reviews to describe your learning.
• Participate in multidisciplinary review meetings where immunology results are presented as part of the clinical care of patients who have an immunodeficiency and/or are undergoing immunotherapy and critically evaluate how a partnership approach between Clinical Immunology and other clinical specialisms benefits patients who are undergoing investigations of the immune system.
• Attend clinics where patients are referred for the investigation of immunodeficiency and/or are undergoing immunotherapy and review and generate your own report from the immunological investigations undertaken.
• Critically appraise the internal quality control and external quality assessment performance of three different methods used in the investigation of immunodeficiency.
• Contribute to Immunology teaching programme.