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115 Cards in this Set
- Front
- Back
- 3rd side (hint)
autophagic granules often suggest?
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these are intracytoplasmic vacuoles containing debris from degraded organelles; suggest ATROPHY
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squamous metaplasia assoc'd with?
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chronic irritation, vit A deficiency
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myeloid metaplasia =?
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extramedullary hematopoiesis
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phosphofructokinase activity in cell injury?
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increased which leads to increased glycolysis (catalyzes F6P-->F1,6BP?), accumulation of lactate. Acidic pH -->reversible clumping of nuclear chromatin
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what are myelin figures?
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reversible morphologic signf of late stage hypoxic cell injury; whorl-like structures, probably from damaged membranes
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which cells are most susceptible to hypoxic injury?
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neurons, esp cerebella Purkinje cells and hippocampal neurons
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proliferation and hypertrophy of hepatocyte sER--classic ultrastructural markers of?
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barbiturate intoxication
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what happens to liver when exposed to CCl4?
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chemical cell injury; CCl4 processed by P450s to CCl3 radical which--> lipid peroxidation and damage of cell membrane, disaggregation of ribosomes--> fatty change (b/c can't make proteins necessary for dealing with fat)
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what nuclear changes seen in coagulative necrosis?
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pyknosis (chromatin clumping); karyorrhexis (chromatin fragmentation); karyolysis (fading of chromatin); disappearance of stainable nuclei
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what are Councilman bodies?
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apoptotic hepatocytes seen in viral hepatitis
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role of p53 in apoptosis?
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facilitates apoptosis by decreasing transcription of blc-2 (antiapoptotic) and increasing bax
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what is Argyria?
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silver poisoning. May lead to permanent gray discoloration of skin and conjunctivae
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hemosiderosis vs. hemochromatosis
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hemosiderosis--accumulation of ferritin aggregates that stain but NO tissue/organ damage; hemochromatosis--more extensive, WITH organ/tissue DAMAGE
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hereditary hemochromatosis genetics?
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Hfe gene on chromosome 6 mutation (get triad of micronodular cirrhosis, DM, skin pigmentation)
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"brown atrophy" refers to?
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lipofuscin accumulation and organ atrophy (common in old age?) (N.B. this is NOT "brown tumor"--osteitis fibrosa cystica??)
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None
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milk-alkali syndrome
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nephrocalcinosis and renal stones due to milk + antacid self treatment for peptic ulcer
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what is dystrophic calcification?
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(different from metastatic calcification) occurs in previously damaged tissue, NOT due to hypercalcemia
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leukocyte ROLLING mediated by?
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selectins
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leukocyte TRANSMIGRATION mediated by?
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PECAM-1
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C5a, LTB4, HETE, kallikrein, bacterial products are all?
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chemotactic factors for PMNs (among other things, probably)
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None
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most important opsonins?
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IgG, C3b
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what stimualte release of His from basophils and platelets?
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Ag binding to membrane-bound IgE; anaphylatoxins (C3a, C5a); heat, cold; IL-1
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what is endothelial PGI2?
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powerful vasoDILATOR and inhibitor of platelet aggregation (epoprostenol is PGI2 analog, vasodilator for pulm HTN)
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None
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products of lypoxygenase pathway of arachidonic acid metabolism?
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HDETES (5-HDETE-->HETE, a potent PMN chemotacter)
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membrane attack complex composed of which complementies?
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C5b-C9
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What happens to catalase+ organisms in Chronica Granulomatous dz?
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ingested but not killed b/c of NADPH oxidase deficiency which results in no H202 substrate for MPO. Catalase negatives don't get rid of own H202 so there the host NADPH oxidase can still function
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None
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how is chediak-higashi syndrome characterized functionally? Morphologically?
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functionally--abnl microtubule formation-->impaired chemotaxis and migration of PMNs; morph--lg cytoplasmic granules, melanosome also affected (albinism!)
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None
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Leukocyte Adhesion Deficiency type 1
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deficiency of beta 2 integrins
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Leukocyte Adhesion Deficiency type 2
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fucosyltransferase gene mutation (fucosyltransferase needed for synthesis of Sialyl Lewis X PMNS…i think this is aka CD15s...interacts with selectins on endothelium?)
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None
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hemorrhagic infarcts usually occur where?
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in lung and GIT, wherever redundant arterial blood supply
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how is PTT affected in Antiphospholipid Ab Syndrome?
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PROLONGED which is weird for a thrombotic D/O. probably artifact from interaction of Ab with test reagent
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what are lines of Zahn?
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alternating layers of dark gray platelets and lighter fibrin; in mature arterial thrombi
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None
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"mural thrombus"=
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thrombus that adheres to one wall of heart chamber or major artery
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branch of carotid artery most commonly involved in thromboembolism?
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middle cerebral artery
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specific gravity of transudate?
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<1.012
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7 morphologic manifestations of shock
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1. acute tubular necrosis (kidney); 2. centrilobular necrosis (liver). 3. fatty change (heart or liver); 4. areas of necrosis (brain); 5 .patchy mucosal hemorrhages (colon); 6. depletion of lipid (adrenal cortex); 7. pulmonary edema
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most common cause of aneuploidy?
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meiotic nondisjunction
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what is a Roberstonian translocation
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long arms (q) or 2 acrocentric chromosomes are joined with common centromere and the short arms (p) are lost
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familial form of Down's?
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parental meiotic translocation; 3-5% of cases; no relation to maternal age
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what are Brushfield spots?
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small white spots on periphery of iris in Down syndrome
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complications of Down syndrome?
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congenital heart dz; acute leukemia (esp lymphoblastic); increased susceptibility to infection; Alzheimers (middle age)
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maternal screening for Down syndrome?
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alpha fetoprotein is LOW (marker for liver and germ cell cancers, btw); hCG is HIGH; unconjugated estriol is LOW
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Cri du chat
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5p del; severe mental retardation, microcephaly, catlike cry, hypertelorism
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DiGeorge syndrome
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microdeletion 22q11--> CATCH 22 (Cardiac abnl, Abnl facies, T cell deficity/Thymic hypoplasia, Cleft palate, Hypocalcemia (b/c dec'd PTH)..all due to microdeletion of 22!)
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Edward's syndrome
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trisomy 18 (nondisjunction); microcephaly, micrognathia, rocker-bottom feet, congenital heart dz
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name the dz: microcephaly, rocker-bottom feet, micognathia, congenital heart dz
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Edward's syndrome (trisomy 18)
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Name the dz: microcephaly, microphthalmia, cleft lip/palate, polydactyly, congenital heart dz
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Patau syndrome (trisomy 13)
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Prader Willi syndrome and Angelman syndrome--examples of what?
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genomic imprinting. Same del but if from dad-->Prader Willi (uncontrolled appetite!); if from mom-->Angelman ("happy puppet")
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what are Lisch nodules?
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pigmented iris hamartomas seen in von Recklinghausen dz (NF)
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what is the specific mutation in von Recklinghausen dz?
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mutation in NF1 gene which codes for GAP--GTPase activating protein
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increased incidence of what other tumors seen in von Recklinghausen dz?
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pheochromocytoma, Wilms tumor, rhabdomyosarcoma, leukemia
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Glial nodules and distorted neurons in cerebral cortex characterize?
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tuberous sclerosis
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tuberous sclerosis also assoc'd with what tumors?
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rhabdomyomas of heart, renal angiolipomyomas
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von Hippel Lindau dz assoc'd with what cancer?
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renal cell carcinoma; also: hemangioblastomas, adnemoas, and cysts of liver, kidney, pancreas
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Tay-Sachs--what's the enzyme deficiency and what accumulates?
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hexosaminidase A--> accumulation of GM2 ganglioside
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cherry red spot especially assoc'd with what lysosomal storage dz?
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Tay-Sachs (hexosaminidase A def); less commonly, in Niemann-Pick dz
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glucocerebrosidase def and accumulation of glucocerebroside =?
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Gaucher dz (get Gaucher cells--enlarged histocytes with wrinkled tissue paper cytoplasm..where's the food analogy??)
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variants of Gaucher dz?
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(def of glucocerebrosidase); type 1--80%, normal lifespan possible, no CNS; type 2--infantile, death <1yr, CNS only; type 3--juvie, less severe than type 2, CNS and viscera
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Hurley syndrome
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AR, mucopolysaccharidosis; alpha-L-iduronidase def-->accumulation of heparin sulfate and dermatan sulafate; death by 10 y/o
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None
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deficiency in von Gierke's dz?
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glucose 6 phosphatase
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deficiency in Pompe's dz?
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alpha1,4 glucosidase-->accumulation of glycogen, esp in liver, heart, sk mm; death <3 y/o
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None
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deficiency in Cori dz?
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amylo1,6glucosidase (debranching enzyme)-->variable accumulation of glycogen leads to stunted growth, hepatomegaly, hypoglycemia
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McArdle syndrome
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muscle phosphorylase def--> accumulation of glycogen in mm; get painful msucle cramps and weakness post-exercise
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infantile cataracts characteristic of what carb metabolism d/o?
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galactosemia
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name the dz: failure to thrive, infantile cataracts, mental retardation, cirrhosis?
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galactosemia (classic)
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name the dz: mental deterioration, musty body odor in blond haired blue eyed kids
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PKU (phenylalanine hydroxylase mutation)
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name the dz: dark urine, ochronosis (dark pigmentation of fibrous tissues and cartilage)
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alkaptonuria (homogentisic oxidase def.) AR
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None
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defect in Maple Syrup urine dz?
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defect in any of the proteins in the branched chain alpha ketoacid dehydrogenase complex
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urine in maple syrup dz?
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smells like maple syrup (duh); high levels of keto acids of LEUCINE, ISOLEUCINE, VALINE
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chromosomal abnormality in cystic fibrosis?
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CFTR mutation on csome 7
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what organism is common cause of death in CF?
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Pseudomonas aeruginosa
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what X-linked d/o has characteristic angiokeratomas?
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Fabry dz (alpha-galactosidase A def-->ceramide trihexoside accumulation)
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normal ratio of T cells?
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2:1 CD4+:CD8+
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Hot T-Bone stEAk
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fever--IL-1; T cell stim--IL-2; bone marrown stim--IL-3; IgE production stim--IL-4; IgA production stim--IL-5
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classic vs alternate pathway
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classic--initiated by reaction with Ag-Ab complex, leads to MAC; alternate--initiated directly by nonimmunologic stimuli (e.g. invader), leads to MAC
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what is MHC?
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major histocompatibility complex. Region on csome 6 where genes coding for HLA Ag are
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class I vs class II HLA?
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I--on almost all human cells, imp in graft rejection; II--mostly on immunocompetent cells
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warm Ab AIHA, hemolytic dz of newborn, Goodpasture's--all examples of what kind of hypersensitivity reaction?
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type II (cytotoxic)
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C1 esterase inhibitor deficiency--> ?
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hereditary angioedema; type 1 hypersensitivity
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What happens in type III hypersensitivity reaction?
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Ab binds Ag (NOT intrinsic part of target cells). Aggregates deposit in vessel walls, etc. complement binds--> PMNs come and cause damage. Hageman factor also activated-->intrinsic pathway coag--> thrombosis and activation of kinins --> vasodilation, edema
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None
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serum sickness--what kind of hypersensitivity reaction?
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type III
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Arthus reaction?
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LOCALIZED immune complex dep (type III). Occurs when Ag introduced--via injection or organ transplant-- in presence of preformed Abs
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name the type of transplant rejections: T cell mediated, days-months
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ACUTE rejection
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chronic rejection primarly due to?
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Ab-mediated vascular damage
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underlying problem in X-linked agammaglobulinemia of Bruton?
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mutation in tyrosine kinase gene--> block in maturation of pre B cells to B cells
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Name the dz: absence of plasma cells in tissue, absent of poorly defined germinal centers
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X-linked agammaglobulinemia
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Name the dz: recurrent viral and fungal infections, tetany
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DiGeorge syndrome (CATCH-22)
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ADA deficiency--> ?
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accumulation of deoxyadenosine, deoxy-ATP which are toxic to lymphocytes. Seen in ~50% of AR SCID
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Clinical manifestations of SCID
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severe infections (of all sorts); high incidence of malignancies; failure to thrive; GVHD after blood 'fusions
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Wiskott-Aldrich syndrome
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X-linked d/o with eczema, thrombocytopenia, recurrent infections
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Significance of gp120?
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cell surface protein of HIV virion, has binding sites for CD4 (not just on T cells)
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Are AIDS pts hyPER or hyPOgammaglobulinemic?
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paradoxically, hyPER b/c even though can't make specific Abs, have polyclonal B cell proliferation
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significance of dsDNA in lupus?
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ANA react with dsDNA--> very specific for lupus, shows characteristic "rim" pattern on IF; also identifies pt at risk for glomerulonephritis
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None
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CREST syndrome
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limited scleroderma; Calcinosis, Raynaud phenom, Esophageual dysfxn, Sclerodactyly, Telangiectasias; get anticentromeres, pulm HTN
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None
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secondary amyloidosis (reactive systemic amlyoidosis)
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AA protein dep (from SSA); chronic tissue destruction--> SSA; usually involves parenchymous organs, esp kidneys)
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Familial Mediterranean fever
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AR, episodic fever and polyserositisl; AA amyloid depositon
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amylin=?
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amyloid thought to be derived from glucagon or insulin; seen in type II DM in islet cells
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carcinomas are malignant tumors what what origin?
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EPITHELIAL (sq cell, tranisitional cell, adeno cell)
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what is desmoplasia?
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tumor-induced proliferation of non-neoplastic fibrous connective tissue, assoc'd with adenocarcinoma
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origin of sarcomas?
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mesenchymal
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what is a choristoma?
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small non-neoplastic area of NORMAL tissue misplaced within another organ
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None
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What is Trousseau phenomenon?
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migratory thrombophlebitis assoc'd with underyling carcinoma--usually of lung or pancreas
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None
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CEA is tumor marker for?
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carcinomas of COLON, pancrease, stomach, lung, breast
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alpha fetoprotein is marker for?
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(LOW in Downs); hepatocellular carcinoma, nonseminomatous germ cell tumors of the nuts; also increased in fetal anencephaly and other NTDs
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radium watch-dial workers tend to get what kind of cancer?
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osteosarcoma
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None
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uranium miners tend to get what kind of cancer?
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lung cancer
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How is ras an oncogene?
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ras codes for p21. mutated--> aberrant p21 that can bind and be activated by GTP but can't be inactivated. (mutated in ~25-30% of malignancies!)
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I say "t(8;14)", you say?
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Burkitt's lymphoma (inc'd c-myc)
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I say, t(14;18), you say?
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Follicular lymphoma (inc'd bcl-2)
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I say, t(9;22), you say?
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CML, Phl csome, bcr-abl, Gleevec\
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I say, t(15;17), you say?
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APL, tx: retinoic acids (do NOT give regular chemo b/c of tumor lysis syndrome and release of Auer rod material!)
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None
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what is a double minute?
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small free chromosome-like body resulting from extensive gene amplification
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2 examples of gene amplification in cancer?
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neuroblastoma (n-myc amp); breast cancer--her2neu
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5 cancer suppressor genes
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Rb, p53, WT-1,2 (Wilms tumor), APC, BRCA-1
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MEN IIa
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medullary thyroid cancer, bilateral pheochromocytomas, hyperparathyroidism (hyperplasia or tumor); ret mutation
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None
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