IMMUNOLOGY COURSE OUTLINE

Immunodeficiency Diseases

Few minds wear out; more rust out
Christian Nestell Bovee

Readings

Updated 04/10/00

Who am I? When investigating children who lacked a thymus, I observed that they had normal numbers of lymphocytes and immunoglobulin levels but that they lacked a cell mediated response. This syndrome now bears my name.

Tables

Prevalance of Immunodeficiency Diseases

Phagocytic defeciences

Deficiencies

Combined Deficiences

RESOURCES

PowerPoint Animations

PowerPoint Slide Shows

Immunological Diseases Web Site: This is an extensive meta site hosted by the Karolinska Institute Library and Information Center covering immunologic diseases.
URL --> http://www.mic.ki.se/Diseases/c20.html

ASSIGNED READING

Chapter 21: pp 507-521

OUTLINE/SUMMARY

Phagocytic Deficiencies
- Reduced Neutrophils
- Defective phagocytes
- adherence defects
- chemotactic defectgs
- killing defects
Humoral Deficiencies
- X-linked agammaglobulinemia
- X-linked hyper-IgM Syndrome
- Common variable hypogammaglobulinemia
- Selective immunoglobulin deficiences
Cell mediated Deficiences
- DiGeorge sysndrome
- Nude mice
Combined Immunodeficiences
- Reticular dysgenesis
- Bare lymphocyte syndrome
- SCID
- Wiskott-Aldrich Syndrome
Complement Deficiences

PERFORMANCE OBJECTIVES

DEFINE THE FOLLOWING KEY TERMS:

immunodeficiency [507]	Wiskott-Aldrich syndrome (WAS)[519]
neutropenia[508]	congenital agranulocytosis [508]
granulocytopenia[508]	leukocyte-adhesion deficiency [508]
agranulocytosis[508]	lazy leukocyte syndrome [510]
selective Iga Deficiency [ 514]	chronic granulomatous disease (CGD)[510],
nude mice [515]	X linked agammaglobulinemia [511],
reticular dysgenesis [516	X-linked hyper IgM (XHM) syndrome [512]
bare lymphocyte syndrome [516]	common variable hypogammaglobulinemia(CVH) [513
X linked SCID [518]	DiGeorge Syndrome (Congential thymic aplasia) [515]
	severe combined immunodeficiency disease (SCID)[517]

ON COMPLETION OF THIS SECTION THE STUDENT SHOULD BE ABLE TO:

construct a table comparing four different
phagocytic deficiency diseases [510]
Construct a table comparing 4 different humoral deficiences;
- X linked agammaglobulinemia [511],
- X-linked hyper IgM (XHM) syndrome [512],
- common variable hypogammaglobulinemia(CVH) [513],
- selective Iga Deficiency [514]
Draw a diagram of hematopoiesis and indicate congential defects that impair the immune response for
- phagocytic deficiences,
- humoral deficiences
- cell mediated deficiciences
- combined immunodeficiencies
Discuss the impact of T cell deficiencies
Construct a table of the combined immunodeficiences that shows the disease, the immune system deficiency and the possible mechanism [517]
Discuss the use of CB-17 SCID mice as a model system

SHORT ANSWER QUESTIONS

How does immunodificiency differ from immunotolerance?