Cohen lectures Flashcards
What is the normal range of WBCs?
1100-5500/uL (20-50% of total WBCs)
What should you do to blood before giving it to an immunodeficient patient?
Irradiate it
What is the major immunologic deficiency in DiGeorge Syndrome?
Absence of a thymus so unable to make mature T-lymphocytes.
What are other physiological characteristics of DiGeorge syndrome? (Hint: CATCH-22)
C-Cardiovascular abnormalities A-Abnormal face T-Thymus absent C-Clefts H-Hypocalcemia/hypoparathyroid 22-Abnormality on the 22 chromosome
What is the major immunologic deficiency in SCID?
A bone marrow precursor fails to produce functioning B and T cells.
What are the 3 genetic causes of SCID discussed in class?
1.) X1 - X linked recessive in the gamma chain that forms a part of the IL-2R structure deficiency or mutation which prevents proper maturation 2.) Adenosine deaminase deficiency leading to excess adenosine deaminase 3.) A mutation that affects V(D)J recombination
What is the major immunologic deficiency in X-linked (Bruton) hypogammaglobulinemia?
Lack of mature B cells because B cell precursors lank a kinase that allows them to make antibody.
What is the major immunologic deficiency in Common Variable Immunodeficiency?
Cyclic trouble making specific antibodies
What is the major immunologic deficiency in Hyper IgM syndrome?
Inability to class-switch from IgM to IgG
What is the most common immunodeficiency?
Selective IgA deficiency
What are the two most common characteristics of selective IgA deficiency?
Chronic allergies and GI problems
What infections are most likely to be seen in patients with Brutons?
Infections associated with mucous membranes (pneumonia and chronic diarrhea, enteroviruses). Polio from the oral polio vaccine
What infections are T cell deficiencies commonly associated with?
Severe infections with intracellular pathogens, especially Candida albicans and pneumocystis jirovecii
What infections are B cell deficiencies commonly associated with?
Infections with extracellular pathogens such as stap aureus, H. influenzae, and strep pneumonia.
Which syndromes will have increased susceptibility to intracellular pathogens?
SCID, DiGeorge
Which syndromes will have increased susceptibility to extracellular pathogens?
Brutons, Common Variable Immunodeficiency, Hyper IgM syndrome, Selective IgA deficiency
What is wrong with nude mice?
No thymus
What are two treatments for ADA deficient SCID patients?
1.) ADA given as a drug 2.) Bone marrow transplant 3.) Gene therapy
What is the risk of gene therapy discussed in class?
May insert in the wrong place and promote the development of cancer
What is the treatment for DiGeorge syndrome?
Thymus transplant - from fetal thymus or thymic stromal cells
What is the risk of tissue transplant in immunodeficient patients?
Graft verus host disease
What are the initial tests done to determine B cell deficiency?
Serum protein electrophoresis, quatitiative IgG IgM IgA levels, specific Abs prior to immunization, presence of ABO isohemagglutins
What are the advanced tests done to determine B cell deficiency?
Ab response to novel Ags, sequence specific genes
What are the initial tests done to determine T cell deficiency?
Skin test with recall Ab panel, total lymphocytes count
What are the advanced tests done to determine T cell deficiency?
CD3 CD4 CD8 counts. Mitogen response, MLR, cytokine measurements
What are the initial tests done to determine phagocytes deficiency?
WBC count, differential, morphology, NTB test and oxidative burst
What are the advanced tests done to determine phagocytes deficiency?
Assay for phagocytosis, genetics
What are the initial tests done to determine complement deficiency?
CH50, Assay for C1inh
What are the advanced tests done to determine complement deficiency?
Individual complement component levels
How can commercial gamma globulin be given?
IVIG (more common) or SCIG
What is in commercial gamma globulin?
Mostly (99%) mixed IgG from multiple donors
Name the viruses given by Cohen that are immunosuppressive in humans?
HIV, measles, mono, CMV
How is tissue damaged in a Type II autoimmune reaction?
Antibody mediated IgG, IgM, IgA
Name two diseases that result from ?stimulatory hypersensitivity.?
Graves Disease - hyperthyroidism resulting from Ab to TSH (called long-acting thyroid stimulator). Inappropriate tachycardia - Ab to beta-adrenergic receptor. Both stimulate the action of the cells they target.
T or F: ?Lumpy-bumpy? immunofluorescence pattern is characteristic of Type II immunopathology.
False. Type III
T or F: ?Linear? immunofluorescence pattern is characteristic of Type II immunopathology.
TRUE
What is Dressler syndrome?
Post-cardiac injury syndrome. When a person makes autoantibody to heart tissue post-surgery. Resolves over time with anti-inflammatories.
What is Goodpasture syndrome?
Development of autoantibodies to lung and kidney basement membranes, but injury more commonly seen only in the kidney unless the person is a smoker.
T or F: Goodpasture syndrome is a type III immunopathology.
False. Type II, antibodies are directed against basement membranes, not just trapped inside them.
T or F: Autoimmune thrombocytopenia purpura is due to complement mediated destruction.
TRUE
What is rheumatic heart disease? rheumatic fever?
Autoimmune condition acquired after repeat streptococcus infection. Antibody against streptococcus cross reacts with laminin in the heart. R fever is more widespread to skin and CNS.
What is the most common autoimmune disease?
Rheumatoid arthritis
What is rheumatic factor?
IgM anti-IgG. Discovered in RA patient?s serum, although might not actually cause the disease.
What does the Aire gene do?
The Aire gene up regulates expression of many body genes to weed out self-reactive T cells. In some families, there is a mutation of the alpha chain of the T cell that prevents it from reacting to Aire. This results in release of auto reactive T cells.
T or F: A forbidden clone is when you make a clone of yourself for sexual purposes.
False, but if only it was true! The emergence of a clone that escapes clonal abortion.
T or F: Cross-reaction is what happens to vampires when they enter a church.
False. It is when an antibody reacts to both foreign antibody and self.
T or F: Illicit help is when your dad and the maid play games in the bedroom when mommy isn?t home.
False. It is when a B-cell ingests both self and foreign peptide and when activated by a Tfh makes antibodies to self peptide.
What is passive antibody?
Antibody from another person that reacts to the donor?s cells (ex. hemolytic disease of the newborn).
What is sequestered antigen?
When an antigen that is normally sequestered safe in the tissue is exposed.
Resection of which organ can improve myasthenia gravis in some patients?
Thymus
