BIOM30002 Lecture Notes - Lecture 1: Severe Combined Immunodeficiency, Primary Immunodeficiency, Common Variable Immunodeficiency
12 Jun 2018
School
Department
Course
Professor
Topic 1 – B cell and Diseases
L1 B lymphocytes biology 1
Summary
- Major steps in Ab gene rearrangement and B cell development
- Major steps of B cell differentiation in an immune response
- Molecular basis of at least one primary immunodeficiency disorder and their therapeutic
options
- Strategies for generating mAb, modifying then for therapeutic use and disease in which they
are beneficial
- Disease arising from errors in normal B cell behaviour are a “cost” that is balanced by the
“benefit” of an adaptive immune system to the organism and the population
Classification of PID
- Primary Immunodeficiencies (PID)
1. Antibody Deficiencies
Absent B cells
X-linked agammaglobulinaemia (XLA)
Common variable immunodeficiency (CVID)
Hyper IgM syndromes (HIGM)
2. Combined immunodeficiencies (CID)
Severe combined immunodeficiency (SCID) – defects in T cells and B cells
Treatment
- Depends
1. Replace the whole IS
Severe, eg. SCID, XLA
Haematopoietic stem cell transplantation (HSCT)
Gene therapy
With some Ab, eg. HIGM, XLA CVID
Immunosuppression
Graft vs host disease
2. Replace Ab intravenous IF (IVIG) subcutaneous IG (SCIG)
Document Summary
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