9276869
Description
Flashcards by Marissa Alvarez, updated more than 1 year ago
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Created by Marissa Alvarez
almost 7 years ago
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| Question | Answer |
| CONGENITAL IMMUNODEFICIENCY | PRIMARY (1°) PID -inherited -some genetic cause/ GENETIC BASED -may be treated with stem cell transplantation -relatively RARE |
| ACQUIRED IMMUNODEFICIENCY | SECONDARY (2°) Immunodeficiency -something has been done to you -Pathogenic OR drug INDUCED |
| 10 WARNING SIGNS of PID (1°) | **Almost always present themselves in CHILDREN -family history of PID -MANY recurrent infections -tend to be more resistant to infections |
| MOST COMMON PID (1°) ? RARE? | ANTIBODY DEFICIENCIES RARE = complement deficiencies |
| BACTERIA ASSOCIATED WITH ? | ANTIBODY DEFIENCY (and enteroviruses) |
| FUNGI associated with ? | CELLULAR DEFIENCY (CARD9) |
| PHAGOCYTIC DISORDERS (rare) (1°) CGD? | 1) Chronic Granulomatous Disease (CGD) -Neutrophils (lack of proteins to kill target cells via PMNs) --defect: respiratory burst enzymes |
| PHAGOCYTIC DISORDERS (1°) Congenital Agranulocystosis | Congenital Agranulocystosis -Myeloid Progenitor ----- Neutrophil defect: cannot make granulocytes (lack PMNs) |
| PHAGOCYTIC DISORDERS (1°) Leukocyte Adhesion Deficiency | Leukocyte Adhesion Deficiency *Neutrophils and Monocytes defect: intern beta chain deficiency (issue with how they respond and migrate to site of infection) |
| T Cell Disorders: (much more common) Severe Combined Immunodeficiency (SCID) "Bubble Boy" | -defect in LYMPHOID PROGENITOR CELL (step one) -Lack T, B, and/or NK cells -thymus does NOT develop (myeloid and erythroid are normal) -LETHAL (~100%) --susceptible to nearly ALL infections **LIVE ATTENUATED VACCINES: like MMR can cause disease due to lack of T cells |
| T Cell Disorders: Severe Combined Immunodeficiency (SCID) Continued. . . RAG 1/2 Infants IL2-R defect | RAG1/RAG/2 Recombinase Activating Gene: shared by both T and B cells, required for TCR and Ig gene rearrangement Infants: passively protected by maternal antibodies for first 6-8 months IL2-R defect (in common gamma chain): LOSE the ability to SENSE that the cytokine is there, but can still make IL2 -accounts for most of ALL SCID types |
| T cell disorders: DiGeorge Syndrome | -deletion of chromosome 22 -abnormal migration and development of T cells |
| T CELL disorders BARE-Lymphocye Syndrome (BLS) | -protein that T and B cells use to communicate is MISSING |
| T CELL Disorders Wiskott-Aldrich Syndorme (WAS) | -x-linked -abnormal function of T and B cells -abnormal bleeding |
| KEY CONCEPT: Lymphocyte Phenotype and Molecular Defect?? | NOT a good correlation or connection between genotype : phenotype !! |
| B CELL Disorders: Bruton's X-linked A-g-globulinemia | tiny tonsils defect: absence of Bruton's tyrosine kinase COMPLETE LACK of B CELLS Ig < 10% normal -infection after 5 months (after maternal protection wanes) *Treatment = IV immunoglobulin (IgG) |
| B CELL disorders: Selective IgA Deficiency | MOST COMMON Immunodeficiency -some cases = AR (autosomal recessive) & do not get selected out most CLINICALLY NORMAL IgA < 5% = allergy, GI, SLE (recurrent and chronic infections) **Defect: in the switch to IgA ->Transfusion runs: can have anti-IgA in IgA deficient patients to cause anaphylactic res (think IgA is non-self) |
| B CELL Disorders Common Variable Immune Deficiency (CVID) | AD & x-linked LATE ONSET (after 10 yrs) NO specific Antibody production NO response to VACCINES -low IgG, low IgA, low IgM (decrease in ALL types) -->anti B cell autoantibodies UNIQUE: bimodal distribution in age of onset (in childhood and in adulthood) *Treatment: IV IgG |
| SECONDARY (2°) Immunodeficiencies | EXAMPLE: HIV b/c of the cells it targets = CD4+T helper Cells (mediate everything -- downstream effects) -other examples: treatments for cancer, graft rejection, removal of spleen *pathogen OR drug induced* |
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