Flashcards in Pathology - L1 - Immunologic Diseases Deck (48)
tx of X-linked Agammaglobulinemia?
replacement therapy w/ human Ig
- failure of development of 3rd and 4th phraryngeal arches
- absence of thymus, as well as parathyroids
- facial structures will also be underdeveloped
Severe combined immunodeficiency?
- X-linked, associated w/ failure in lymphocyte (esp T-lymphocyte) development
- autosomal recessive form associated w/ deficiency of adenosine deaminase
Isolated IgA deficiency?
typically asymptomatic; compromised mucosal defenses; respiratory, GI, urogenital infections
- angioedema--repaid swelling under dermis
2. complement deficency
HIV primarily targets what?
the immune system and CNS
how is HIV transmitted?
sexual contact, parenteral exposure to blood, maternal/fetal
what is the target cell of HIV?
cells w/ CD4 receptors (primarily T-helper lymphocyte)
Viral RNA is permanently integrated into host DNA via... ?
What is the half-life of HIV?
half-life of 2 days; rapid turnover of HIV
what are the 3 things HIV virus can do?
1. remain silent
2. cause cell death
3. disrupt normal cell function
what is the clinical course of HIV infection?
- acute, self-limited response (similar to infectious mononucleosis)
- 8-10 year asymptomatic stage
- symptomatic stage (AIDS-related complex)
- endpoint of HIV deficiency
- 5 yrs after infection- 18% have AIDS
- 13 yrs after infection - 65% have AIDS, 20% have symptoms, & 15% have no symptoms
AIDS-related complex (ARC) clinical features?
- fever, wt loss, diarrhea, candidiasis, herpes zoster, hairy, leukoplakia
- increased infections: cytomegalovirus, herpes simplex, toxoplasmosis
- pulmonary - pneumocystis carinii oneumonia (presenting sign in 50%)
- AIDS-dementia complex
Type 1 hypersensitivity
allergy/anaphylaxis; IgE mediated
- IgE binds to mast cells, releasing HISTAMINE and other vasoactive amines
Type II hypersensitivity
- complement dependent (transfusion rxns, erythroblastosis fetalis)
- Ab-dependent cell-mediated cytotoxicity (ADCC)
- Ab-mediated cellular dysfunction (myasthenia gravis, Grave's disease)
Type III hypersensitivity
- immune complex-mediated (SERUM SICKNESS, glomerulonephritis)
- Ag + Ab + Complement - Recruitment of neutrophils, resulting in tissue destruction