Term
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Definition
•Anaphylactic type reaction •Mediated by IgE and mast cells/basophils •Hay fever, atopic dermatitis, bronchial asthma, anaphylactic shock
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•Cytotoxic antibody-mediated reaction •Mediated by cytotoxic antibodies (IgG/IgM) that react with extrinsic or intrinsic antigens in cells •Hemolytic anemia, Goodpasture's Syndrome, Graves' Dz, Myasthenia Gravis
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Definition
•Immune complex-mediated reaction •Mediated by immune complexes that are formed between antigens and appropriate antibodies •SLE, post-strep glomerulonephritis, polyarteritis nodosa
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Definition
•Cell-mediated or delayed-type reaction •Mediated by T-lymphocytes and macrophages, which typically aggregate at the site of injury to form granulomas •Reactions to tumors, sarcoidosis, contact dermatitis
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The following are all examples of what kind of reactions? -Addison's Dz -Scleroderma -Type I DM -Sjogren's Syndrome -Graves' Dz -SLE -MS -Myesthenia Gravis -Polymyositis -Membranous glomerulonephritis |
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The following are _____ d/o's that impair the immune system. -X-linked agammaglobulinemia (XLA) -Severe combined immunodeficiency dz (SCID)
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Definition
•Rare •Sex linked recessive •Total absence of immunoglobulins and B cells •Usually dx'ed due to recurring infections |
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Definition
•No immune response (both cell- and antibody-mediated responses are missing)
•Can be caused by problems on several genes (X-linked, ADA mutation- autosomal recessive)
•"Boy in the bubble"
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Definition
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•HIV = ____virus with what 3 components |
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Definition
•Retrovirus •RNA molecules (genetic material) •Reverse transcriptase (enzyme; transcribes HIV RNA into a DNA molecule which is inserted into a human chromosome) •A protein coat (encloses other 2 compartments)
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| Approx. ____ people worldwide have HIV/AIDS |
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Definition
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Definition
Yolk sac (during fetal dvlpmt) → Fetal liver → Fetal spleen → Bones (transition = shortly before birth) |
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| Hb consists of ___ heme groups and ___ globins |
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Definition
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Definition
Iron Vit. B12 Vit. B6 Folic acid |
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| Adult Hb contains ____ subunits each being composed of _________ |
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Definition
•4 (2 alpha, 2 beta) •Polypeptide chain (globin) and a prosthetic group (heme), which is an iron containing pigment that combines with oxygen to give the molecules its oxygen transporting ability
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Term
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Definition
2 alpha or alpha-like chain (Chr 16) + 2 beta or beta like chains (Chr 11)
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Definition
•Chr 16 •2 genetically identical copes of alpha globin
•Zeta •Psuedogenes (genes that are closely related to functional genes elsewhere but have undergone a mutation which makes them non-functionl)
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Definition
•Chr 11
•Beta globin gene
•4 beta-like genes -2 genetically NON-identical gamma genes -Delta -Epsilon
•1 pseudogene |
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Definition
Fetus = gamma + alpha Adult = beta + alpha
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| Common most single-gene dz in humans |
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Definition
| Hemoglobinopathies (genetic disorders of human hemoglobins) |
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Definition
•Very 1st genetic d/o cloned •Autosomal recessive
•Single amino acid substitution causing beta globin mutation •Possible treatment = hydroxyurea (anticancer drug) which reactivates gamma genes making fetal Hb reappear in RBC's
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Term
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Definition
Decreased synthesis of 1 of globins → Alpha: beta imbalance |
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Definition
•Most common cause = deletion of 1 or both alpha genes •In the absence of the alpha chains the beta globin cluster forms homotetrameric Hb's which are incapable of releasing oxygen to tissues in normal conditions
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Definition
•→precipitation of the excess normal alpha chains to form a Heinz body in the RBC → damage to the RBC's membrane •Most common cause = single-base pair substitution that results in proteins that have reduced, altered, or no activity
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Definition
•In regions of the world where beta thalassemia is common •Individuals are compound heterozygotes for 2 different beta thalassemia alleles •Characterized by severe anemia and the need for life-long medical management
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Definition
•Carries of 1 beta thalassemia allele •Clinically well with hypochromic, microcytic RBC's
•May have a slight anemia that can be misdiagnosed initially as iron deficiency
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Definition
•Impairs the production of beta globin alone •Almost every type of mutation known to reduce the synthesis of a beta globin mRNA or protein has been ID'ed as a cause
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Definition
Caused by large deletions (beta globin gene + 1 or more of the other genes (or LCR) in the beta globin cluster) -Deletion of LCR → remainder of genes go unexpressed |
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| Hereditary persistance of fetal Hb is caused by: |
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Definition
| Deletions within the beta globin cluster |
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Definition
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Definition
•Increase in WBC's •Malignant leukocytic d/o's: -Neoplasm arising in bone marrow (where WBC are formed & differentiate) → leukemias or plasma cell dyscrasias -Neoplasms arising in lymphoid organs (where WBC's travel to in order to mature and dvlp) → lymphomas
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Definition
•Acute myelogenous (AML) = occurs in all age groups •Chronic myelogenous (CML) = dz of adulthood •Acute lymphocytic (ALL) = most common in children <5 yrs •Chronic lymphocytic (CLL) = a dz of older ppl
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Definition
•Non-Hodgkin's = adults > children •Hodgkin's = affects all ages, but with peaks at 25 and 55 yrs |
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| Bleeding d/o's occur as a result of defects that are: |
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Definition
Vessel wall related Platelet related Clotting factor related |
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Definition
X-linked recessive d/o of coagulation caused by mutation in the F8 gene (→ deficiency or dysfunction of clotting factor VIII)
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Definition
•X-linked recessive d/o of coagulation caused by mutation in the F9 gene (→ deficiency or dysfunction of clotting factor IX)
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