Pathology 3

8/13/2019 Pathology 3

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1. Amorphous having no apparent shape or organization

166. are enzymes affected in autosomal dominantdisorders?

no - instead receptors and structural proteins are involved

3. atypia being uncharacteristic or lacking uniformity

5. basophilic readily stainable with basic dyes (usually hematoxylin = blue)

4. being uncharacteristic or lacking uniformity atypia

7. biopsy the removal of a sample of tissue, cells , or fluids from the living body forexamination

115. BOOK QUESTION: A 9-month-old child has a history of recurrentinfections with multiple agents, includingcytomegalovirus, Candida albicans,Staphylococcus aureus, and Staphylococcusepidermidis. A carefuly Fx and pedigreeanalysis show this to be a genetic disorder thatis inherited in an autosomal recessive pattern.

Which of the following laboratory studies islikely to be most useful in estabilshing theunderlying mechanism of immunodeficiency inthis infant?

A) quantitative serum immunoglobulin levlesB) enumeration of B cells in bloodC) enumeration of CD3+ cells in bloodD) tests of neutrophil functionE) adenosine deaminase levels in leukocytes

E) adenosine deaminase levels in leukocytesThis patient is susceptible to bacterial, fungal, and firal infections and mostlikely has sever comined immunodeficiency (SCID). The autosomal recessivepattern of inheritance implicates adenosine deaminase (ADA) deficiency ratherthan mutations in the gamma chain of cytokine receptors. Low ADA levels inthe leukocytes are diagnostic. The other lis ted options are relevant to the workupof primary immunodeficiencies, but they are not specific to SCID. see pp. 234-235

107. BOOK QUESTION: A 10-year old boy who is mentally retarded isable to carry out activities of dai ly living,including feeding and dressing himself. On PEhe has brachycephaly and oblique palpebralfissures with prominent epicanthal folds. A transverse crease is seen on the palm of eachhand. On auscultation of the chest there is agrade III/VI systolic murmur. Which of thefollowing deseases is he most likely to develop

by age 20? A) acute leukemiaB) hepatic cirrhosisC) chronic renal failureD) acute myocardial infarction

E) aortic dissection

A) acute leukemiaThis boy has Down syndrome (trisomy 21), one of the trisomies that can resultin a liveborn infant. Although children with Down syndrome can function fairly

well, they often have many associated congenital anomalies. Among the morecommon is congenital heart disease, including ventricular septal defect. Thereis also a 10-20 fold increased risk of acute leukemia. Virtually all persons withDown syndrome who live to the age of 40 will have evidence of Alzheimerdisease. Hepatic cirrhosis is a feature of galactosemia. Chronic renal failuremay be seen in genetic disorders that produce polycystic kidneys. Myocardialinfarction at a young age suggests familial hypercholesterolemia. Aorticdissection is seen in persons with Marfan syndrome. See pp. 161-162.

PathologyStudy online at quizlet.com/_f6ab5


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106. BOOK QUESTION: A 25-year-old woman amenorrhea has never hadmenarche. On physical examination, she is 145 cm (4ft, 9 in) tall. She has a webbed neck, a broad chest, and

widely spaced nipples. Strong pulses are palpable inthe upper extremities. On an abdominal MR imaging,the ovaries are small, elongated and tubular. Which of following karyotypes is most likely to be present in this

patient? A) 45,X/46,XXB) 46,X,X(fra)C) 47,XXY D) 47,XXXE) 47,XX,+16

A) 45,X/46,XXThe features described are those of class ic Turner syndrome. Persons

who reach adulthood may have mosaic cell lines, with some 45,X cellsand some 46,XX. A female carrier of the fragile X syndrome, X(fra), isless l ikely to manifest the disease than a male, but the number of triplerepeat sequences (CGG) increases in her male offspring. The 47,XXY karyotype occurs in Klinefelter syndrome; affected persons appear asphenotypic males. The "superfemale" karyotype (XXX) leads to mild.

(?) see pp. 165-167

108. BOOK QUESTION: A 25-year-old woman gives birth to a term infant with aright transverse palmar crease, low-set ears, obliquepalpebral fissures, and a heart murmur. The infantsurvives childhood and exhibits only mild mentalretardation. Which of the following chromosomal

abnormalities is most likely to be present in this child? A) haploidy B) monosomy C) mosaicismD) tetraploidy E) triploidy

C) mosaicismThese features are characteristic of trisomy 21, but the child is notseverely affected, which suggests mosaicism. In mosaic persons,greater numbers of potentially normal cells having the properchromosomal complement are present, which may allow infants withabnormalities of chromosome number to survive to term and beyond.

Haploidy is present in gametes. Loss of an autosomal chromosome isdevastating; the only monosomy associated with possible survival toterm is Turner syndrome (monosomy X). Most aneuploid conditions(trisomies and monosomies) lead to fetal demise; fetuses with trisomy 21 are the most likely to survive to term. Triploid fetuses rarely survive

beyond the second trimester and are virtually never liveborn. Likewise,tetraploidy accounts for many firs t-trimester fetal loss es and is notsurvivable. see pp. 161-163

114. BOOK QUESTION: A 26-year-old woman has had bouts of joint pain forthe past 2 years. She also has a rash on the cheeks and

bridge of the nose. On PE there is no joint swelling or

deformity, although generalized lymphadenopathy i spresent. Lab studies indicate anemia, leukopenia, apolyclonal gammopahty, and proteinuria. The serum

ANA test result is positive at a titer of 1:1024 with a rimpattern identified by immunofluorescence. The lightmicroscopic immunofluorescent (with antibody to IgG)appearances of a skin biopsy are shown [dark green

with lighter green border]. Which of the following is the best information to give this patient about her disease? A) blindness is likely to occur within 5 yearsB) avoid exposure to cold environmentsC) joint deformities will eventually occur

D) chronic renal failure is likely to occurE) cardiac valve replacement will eventually berecquired

D) chronic renal failure is likely to occurThis patient has systemic lupus erythematosus (SLE). Many persons

with SLE have glomerulonepthritis and eventually develop renalfailure. Blindness is uncommon in SLE. Raynaud phenomenon is

associated with many autoimmune disease, but it is most troublesomein scleroderma. Although synovial inflammation is common in SLE,

joint deformity is rare. The Libman-Sacks endocarditis associated withSLE tends to be nondeforming and limited and there is minimal valvedamage. It is now uncommon, because of the use of corticosteroidtherapy in the treatment of SLE. see pp. 213-221


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109. BOOK QUESTION: A 37-year-old man who is HIV posi tive has noticedmultiple 0.5-1.2 cm plaque-like, reddish-purple skinlesions on his face, trunk and extremities. Some of thelarger lesions appear to be nodular. These lesionshave appeared over the past 6 months and have slowly enlarged. Molecular analysis of the spindle cells foundin these skin lesions is likely to reveal the genome of

which of the following viruses? A) cytomegalovirusB) Epstein-Barr virusC) adenovirusD) human herpesvirus-8E) HIV-1

D) human herpesvirus-8This patient has AIDS, with Kaposi sarcoma of the skin. Kaposisarcoma is associated with a herpesvirus agent that is sexually transmitted: human herpesvirus 8 (HHV-8), also called Kaposisarcoma herpesvirus. Other herpesviruses are not involved in thepathogenesis of Kaposi sarcoma, although infection with these virusescan occur frequently in persons with AIDS. HIV, although present inthe lymphocytes and monocytes, is not detected in the spindle cells that

proliferate in Kaposi sarcoma. With the exception of the varicella-zoster virus, which is as sociated with dermatomally distributed skin vesiclesknown as shingles, skin lesions are not common manifestations of herpesviruses, which include cytomegalovirus, Epstein-Barr virus, oradenovirus infections. see pp. 246-247

110. BOOK QUESTION: A 45-year-old woman has experienced difficulty inswallowing that has increased in severity over the past

year. She has also experienced malabsorption,demonstrated by a 5-kg weight loss in the past 6months. She reports increasing dyspnea during this

time. On PE, her temp is 36.9C, pulse 66/min, RR 18/min and BP 145/90 mmHg. Echocardiography shows a large pericardial effusion. The Anti Nuclear

Antibody test result is posi tive at 1:512 with anucleolar pattern. Which of the following seriouscomplications of the patient's underlying autoimmunedisease i s most likely to occur?

A) meningitisB) glomerulonephritisC) perforated duodenal ulcerD) adrenal failureE) malignant hypertension

E) malignant hypertensionThis patient has diffuse systemic sclerosis (scleroderma). The smallarteries of the kidney are damaged by a hyperplastic arteriolosclerosisthat can be complicated by very high blood pressure and renal failure.Meningitis and adrenal failure are not typical features of autoimmunediseases. Glomerulonephritis is a more typical complication of systemic

lupus erythematosus. With scleroderma, the gastrointestinal tractundergoes fibrosis, without any tendency to perforation or ulceration.see pp. 223-225

111. BOOK QUESTION: An epidemiologic study is conducted to determinerisk factors for HIV infection. The study documentsthat persons with coexisting sexually transmitteddiseases such as chancroid are more likely to becomeHIV posi tive. It is postulated that an inflamedmucosal surface is an i deal location for thetransmission of HIV during sexual intercourse.

Which of the following cells in these mucosal surfacesis most instrumental in transmitting HIV to CD4+ Tlymphocytes?

A) CD8+ cells

B) natural killer cellsC) dendriti c cellsD) neutrophilsE) plasma cells

C) dendritic cellsThree types of cells can carry HIV: dendritic cells, monocytes, and CD4+T lymphocytes. Mucosal dendritic cells (i.e. Langerhans cells) can bindto the virus and transport it to CD4+ cells in the lymph nodes. Whetherthe virus is internalized by mucosal dendritic cells is not clear.Monocytes and CD4+ T cells express CD4 and the coreceptors (CCR5and CXCR4); therefore, HIV can enter these cells. The other lis ted cellscannot be infected by HIV. s ee pp. 238-243


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116. BOOK QUESTION: An infant born at term develops abdominaldistension in the first week of life. Meconium ileusis diagnosed. The infant has persistent steatorrheaand fails to develop normally. Later in childhood,multiple respiratory tract infections lead to

widespread bronhioectasis . Which of the followinglaboratory findings i s most likely related to this

child's underlying disease? A) decreased serum thyroxine levelB) posi tive HIV serology C) elevated sweat chloride levelD) increased urine homovanillic acid levelE) hyperbilirubinemia

C) elevated sweat chloride levelThe findings are typical of cystic fibrosis, CF. see pp. 465-471

104. BOOK QUESTION: A 38-year-old man has had headaches and nauseafor the past 2 months. Lab findings show hypercalcemia and hypophosphatemia and normalserum albumin. Urine microscopic analysis showsdeposition of calcium salts in the renal tubular

epithelium. Which of the following processes hasmost likely produced this change in the kidney?

A) dystrophic calcificationB) renal tubular atrophy C) autophagocytosisD) metastatic calcificationE) cellular aging

D) metastatic calcificationDeposition of calcium in normal healthy tissues as a result of prolongedhypercalcemia is called metastatic calcification. This process may occur inhyperparathyroidism. Dystrophic calcification refers to calcium depositionin in jured tissues, with normal serum calcium levels. Atrophy decreases cellsize but is not accompanied by calcium deposition. Autophagocytosis yields

more golden-brown l ipofuscin pigment in the cytoplasm, particularly inhepatocytes and myocardial fibers, a process that becomes more apparent

with ag ing. See pp. 38-39.

105. BOOK QUESTION: An experiment introduces a "knockout" genemutation into a cell line. The frequency of cellkaryorrhexis and cytoplasmic fragmentation isincreased, compared with a cell line without the

mutation. Overall survival of the mutant cell line isreduced. Which of the following genes is most likely to be affected by this mutation?

A) BAXB) BCL-2C) C-MYCD) FASE) TP53

B) BCL-2The BCL-2 gene product inhibits cellular apoptosis by binding to Apaf-1.The BAX gene product promotes apoptosis. The C-MYC gene is involved

with oncogenesis. The FAS gene encodes for a cellular receptor for FASligand, which signals apoptosis. TP53 gene activity normally stimulates

apoptosis, but mutation favors cell survival. See pp. 28-29.

90. Can hypertrophy and hyperplasia occur together?Can they both result in an enlarged (hypertrophic)organ?

yes and yes: e.g. the uterus during pregnancy

12. the cause or causes of a disease or abnormalcondition

etiology

24. characterized by swelling and taking up of fluid hydropic

26. a deficiency of oxygen reaching the tissues of the body

hypoxia

32. a deficient supply of blood to a body part ischemia

52. a degenerative condition of a cell nucleus marked by clumping of the chromosomes,hyperchromatism, and shrinking of the nucleus

pyknosis

126. describe the mechanism of cell injury by accumulation of reactive oxygen species

covalent modification of cellular proteins, lipids, nucleic acids


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127. describe the mechanism of cell injury by increased permeability of cellular membranes -

what is the typical result?

may affect plasma membrane, lysosomal membranes, mitochondrialmembranes; typically culminates in necrosis

36. a diseased state or symptom morbidity

44. distinctively characteristic of a particulardisease or condition

pathognomonic

72. do all cells have MHC groups? - no - only nucleated cells- therefore not RBCs

91. does increased demand of the striated musclecells in both the skeletal muscle and the heartlead to hypertrophy or hyperplasia?

hypertrophy only because adult muscle cells have a limited capacity to divide

9. eosinophilic readily stainable with eosin (red)

11. etiology the cause or causes of a disease or abnormal condition

13. excision surgical removal or resection of an entire lesion or diseased portion

15. extrinsic originating or due to causes or factors from or on the outside of a body, organ orpart

38. the form and structure (appearance) of anorganism or any of its parts morphology

56. a group of signs and symptoms that occurtogether and characterize a particularabnormality

syndrome

2. having no apparent shape or oganization amorphous

17. heterogeneous not uniform in structure or composition

21. homeostasis the maintenance of a relatively stable internal physiological conditions underfluctuating environmental conditions

19. homogeneous of uniform structure or composition throughout

61. how do antibodies and immunoglobulins relateto each other?

which cells produce and have them bound ontheir membrane?

- antibodies and immunoglobulins are essentially synonymous- produced by and sometimes attached to B-lymphocytes

82. how do the terms etiology and pathogenesisrelate to each other?

etiology refers to why a disease arises, pathogenesis describes how a diseasedevelops - both are essential for not only understanding a disease but also the

basis for rational treatments

69. how do the terms lymphocytes and leukocytesrelate to each other?

- leukocytes is the name for all of the white blood cells- lymphocytes are one of the several types of WBCs

148. how does immunohistochemistry help indiagnosis and classification of tumors?

- distinct protein expression patterns define different entities- e.g. detection of specific monoclonal antibodies by labeling, and specific

antigens like PSA and estrogen receptors144. how does repair by connective tissue proceed? starts with formation of granulation tissue and culminates in the laying down

of fibrous tissue -- scar formation

151. how to the dendritic cells operate? - capture microbes and foreign antigens --> transport to lymph nodes -->antigen presentation to naive lymphocytes --> recognition --> lymphocyteactivation and proliferation into effector/plasma and memory cells

23. hydropic characterized by swelling and taking up of fluid

25. hypoxia a deficiency of oxygen reaching the tissues of the body

33. iatrogenic induced inadvertently by medical treatment or diagnostic procedures


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139. identify some of the major cell-derivedmediators of inflammation and their effects

- vasoactive amines - histamines, serotonin: main effects are vasodilation andincreased vascular permeability - arachidonic acid metabolites - prostaglandins and leukotrienes: several formsexist and are involved in vascular rxns, leukocyte chemotaxis, and other rnxs of inflammation, antagonized by lipoxins- cytokines: proteins produced by many cell types, usually act at short range;mediate multiple effects, mainly leukocyte recruitment and migration; principalones in acute inflammation are TNF, IL-1, IL-6 and chemokines

- ROS: roles include microbial killing and tissue injury - NO: effects are vasodilation and microbial killing- lysosomal enzymes: roles include microbial killing and tissue injury

27. incidence the rate of occurrence of new cases of a particular disease in a population beingstudied

34. induced inadvertently by medical treatment ordiagnostic procedures

iatrogenic

29. intrinsic originating or due to causes or factors within a body, organ or part

99. is the decreased workload and loss of innervation to a limb following inj ury pathologic or physiologic atrophy?

pathologic atrophy

98. is the loss of hormone stimulation inmenopause pathologic or physiologic atrophy?

physiologic atrophy

31. ischemia a deficient supply of blood to a body part

22. the maintenance of relatively stable internalphysiological conditions under fluctuatingenvironmental conditions

homeostasis

35. morbidity a diseased state or symptom

37. morphology the form and structure (appearance) of an organism or any of its parts

39. mortality the number of deaths in a g iven time or place

94. most forms of pathologic hyperplasia arecaused by excessive hormonal or growth factorstimulation - what are some examples?

- endometrial hyperplasia after a normal menstrual period due to disturbance between estrogen and progesterone- hyperplasia of the breast- skin warts composed of hyperplastic epithelium - growth factors may be from

viral genes or those of infected cells

54. a negative aftereffect sequelae

18. not uniform in structure or composition heterogeneous

113. NOTES QUESTION: Which of the following is classifiable as afunctional abnormality?

A. a large tumor increasing pressure in the

brainB. elevated HR due to excessive secretion of thyroid hormoneC. loss of renal filtration due to amyloiddeposition in the kidney D. rupture of an aneurysm due to a thin, weak

vessel wall.

B. excretion / gain(A, C, D all structural)


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112. NOTES QUESTION: Which of the following is TRUE of free radicals? A. cells with high metabolic requirements are prone to injury B. formed by antioxidantsC. inhibit apoptosisD. predominately cause damage by blocking metabolicpathways

A. cells with high metabolic requirements are prone to injury

40. the number of deaths in a given time or place mortality

20. of uniform structure or composi tion throughout homogeneous

16. originating or due to causes or factors from or on the outside of a body, organ, or part

extrinsic

30. originating or due to causes or factors within a body, organ orpart

intrinsic

41. pathogenesis the steps in development of a disease

43. pathognomonic distinctively characteristic of a particular disease or condition

48. the percentage of a population that is affected with a particulardisease at a given time

prevelance

45. pleomorphism variation in size and shape

47. prevelance the percentage of a population that is affected with aparticular disease at a g iven time

49. prognosis the prospect of survival and recovery from a disease asanticipated from the usual course of that disease or indicated

by special features of the case

50. the prospect of survival and recovery from a disease asanticipated from the usual course of that disease or indicated

by special features of the case

prognosis

51. pyknosis a degenerative condition of a cell nucleus marked by clumping of the chromosomes, hyperchromatism, andshrinking of the nucleus

28. the rate of occurrence of new cases of a particular disease in apopulation being studied

incidence

6. readily stainable with basic dyes (usually hematoxylin = blue) basophil ic

10. readily stainable with eosin (red) eosinophilic

8. the removal of a sample of tissue, cells, or fluids from the living body for examination

biopsy

53. sequelae a negative aftereffect

42. the steps in development of a di sease pathogenesis

14. surgical removal or resection of an entire lesion or diseasedportion

excision

55. syndrome a group of signs and symptoms that occur together andcharacterize a particular abnormality

89. True or False:Hyperplasia is an adaptive response in cells capable of replication, whereas hypertrophy occurs when cells have alimited capacity to divide.

True!


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88. True or False:in pure hypertrophy there are no new cells, jus t bigger cells containing increasedamounts of structural proteins andorganelles

True

46. variation in size and shape pleomorphism

66. what are 3 possible actions of a B-

lymphocyte?

- the antibodies on outside of cell can bind pathogen then engulfs and performs

antigen presentation using MHC II (like macrophage)- [activated by Helper T cell that binds to MHC II]- can proliferate as PLASMA cells to produce free floating antibodies- can proliferate to form long-lasting MEMORY cells

173. what are characteristics of Downsyndrome?

- trisomy 21 or translocation- clincial features: severe mental retardation, flat facial profile, epicanthic folds,cardiac malformations, higher risk of leukemia and infections, prematuredevelopment of Alzheimer disease

117. What are some categories of causes of cellinjury?

- oxygen deprivation / hypoxia- chemical agents- infectious agents- immunologic rxns- genetic factors- nutritional imbalances- physical agents- aging

163. what are some of the common primary (congenital) immune deficiency diseases?

- XLA: failure of B cell maturation, absence of antibodies, caused by mutations in BTK which encodes a tyrosine kinase required for maturation signals from the pre-B celland B cell receptors- selective IgA deficiency - X-SCID: failure of T cell and B cell maturation; mutation in the common gammachain of cytokine receptor leading to failure of IL-7 signaling and defectivelymphopoiesis- autosomal SCID: failure of T cell development, secondary defect in antibody responses; approximately 50% of cases caused by mutation in the gene encoding

ADA, leading to accumulation of toxic metabolites during lymphocyte maturation andproliferation- X-linked hper-IgM syndrome: failure to produce isotype-switched high-affinity antibodies (IgG, IgA, IgE); mutation in gene encoding CD40L- clinical presentation: increased susceptibility to infections in early life

141. what are some of the features of chronicinflammation?

- prolonged host response to persistent stimulus- caused by: microbes that resist elimination; immune responses against self andenvironmental antigens, and some toxic substances (e.g. silica); underlies many important diseases- characterized by persistent inflammation, tissue injury, attempted repair by scarringand immune response- cellular infiltrate consisting of activated macrophages, lymphocytes and plasmacells often with prominent fibrosis- mediated by cytokines produced by macrophages and lymphocytes (notably Tlymphocytes) with a tendency to an amplified and prolonged inflammatory responseowing to bidirectional interactions between these cells

150. what are some of the mechanisms of innate immunity?

- epithelial barriers- phagocytes- NK cells- plasma proteins (e.g . complement)*rxn often manifested as inflammation


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158. what are some of the principal mediatorsin immediate (typeI I) hypersensitivity (allergies)?

what are symptoms of allergies?

- histamine, proteases; prostaglandins and leukotrienes, cytokines- responsible for immediate vascular and smooth muscle rxns and late-phase rxn(inflammation)- from annoying rhinitis to fatal anaphylaxis

142. what are some systemic effects of inflammation?

- fever: cytokines (TNF, IL-1) stimulate production of prostaglandins in hypothalamus- production of acute-phase proteins: C-reactive protein, others; synthesis stimulated by cytokines (IL-6, others) acting on liver cells- leukocytosis: cytokines (CSFs) stimulate production of leukocytosis from precursors inthe bone marrow - in some severe infections, septic shock: fall in BP, disseminated coagulation,metabolic abnormalties; induced by high levels of TNF

83. what are the characteristics externalstress that lead to irreversible injury anddeath of affected cells?

if stress is : severe, persistent, rapid in onset

137. what are the mechanisms by whichleukocytes eliminate microbes? how canthis damage normal tissues?

- leukocytes eliminate microbes and dead cells by phagocytosis- destruction is caused by free radicals (ROS, NO) generated in activated leukocytes andlysosomal enzymes- enzymes and ROS may be released into ECF

71. what are the most common/best antigenpresenting cell? what type of MHC groupdoes it have?

which T cells will read the MHCpresentation?

- dendritic cells- MHC II- Helper T cells with CD4 receptor

147. what are the sampling approaches fordiagnosis of tumors?

excision, biopsy, fine-needle aspiration and cytologic smears

133. what are the steps of inflammatory response (the five Rs)? what are thepossible outcomes due to inflammation?

- 1. recognition of the injurious agent; 2. recruitment of leukocytes, 3. removal of theagent, 4. regulation (control) of the response, 5. resolution (repair)- outcome of acute inflammation is either elimination of the noxious stimulus, followed

by decline of the rxn and repair of the damaged tissue OR - persistent injury resulting in chronic inflammation

135. what are the steps of leukocyterecruitment to sites of inflammation and

what chemical signals promote eachstep?

- leukocytes are recruited (by cytokines from tissue macrophages and other cells) from blood to extravascular tissue where pathogens or damaged tissues are present, thenactivated to perform fxn:- loose attachment to and rolling on endothelium, mediated by selectins- firm attachment to endothelium, mediated by integrins- migration through interendothelial spaces

103. what are the two types of cell death? whatare the mechanisms, morphology androles of each in disease and physiology?

- necrosis is when damage to membranes is ssever, enzymes leak out of lysosomes,enter the cytoplasm, and digest the cell - cellular contents also leak through plasmamembrance into ECF and elicit inflammation- apoptosis is when a cell is deprived of growth factors or the cell's DNA or proteins aredamaged beyond repair and the cell kills itself - characterized by nuclear dissolutionand does not illicit an immune response

130. what are the two types of pathologiccalcification and what are thecharacteristics of each?

- dystrophic calcification: deposition of calcium at sites of cell injury and necrosis- metastatic calcification: deposition of calcium in normal tissues, caused by hyperclcemia (usual ly a consequence of parathyroid hormone excess)

93. what are the two types of physiologichyperplasia and what is an example of each?

- hormonal hyperplasia exemplified by the proliferation of the glandular epithelium of the female breast at puberty and during pregnancy - compensatory hyperplasia in which residual tissue grows after removal or loss of partof an organ, e.g. when part of a liver is resected mitotic activity in the remaining cells

begin as early as 12 hours later eventually restoring the liver to its normal weight


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92. what are the two types of signals driving cardiachypertrophy?in what types of cells doeshypertrophy occur?

- mechanical triggers such as stretch- trophic triggers which are soluble mediators that stimulate cell growth such as growth factors andadrenergic hormones- occurs in tissues incapable of cell division

58. what are the types of WBCsor leukocytes?

- neutrophils- lymphocytes (B&T)- monocytes- eosinophils- basinophils

134. what are the vascular rxnsin acute inflammation?

- vasodilation induced by chemical mediators such as histamine and is the cause of erythema and stasisof blood flow - increased vascular permeability induced by histamine, kinins, and other mediators that produce gaps

between endothelial cells; by direct or leukocyte-induced endothelial injury; and by increased passageof fluids through the endothelium - this increased perm. allows plasma proteins and leukocytes to entersites of infection or tissue damage; fluid leak through blood vessels results in edema

131. what are three routes tocellular aging? what are thecharacteristics of each?

- accumulation of DNA damage (e.g. by free radicals): defective DNA repair mechanisms; conversely DNA repair may be activated by calorie restriction which is known to prolong aging in modelorganisms

- replicative senescence: reduced capacity of cells to divide secondary to progressive shortening of chromosomal ends (telomeres)- other factors: progressive accumulation of metabolic damage; possible roles of growth factors thatpromote aging in simple model organisms

140. what are three types of plasma protein-derivedmediators of inflammationand what is theirmechanism?

- complement proteins: activation of the complement system by microbes or antibodies leads to thegeneration of multiple breakdown products, which are responsible for leukocyte chemotaxis,opsonization and phagocytosis of microbes and other particles and cell killing- coagulation proteins: activated factor XII trigges the clotting, kinin and complement cascades andactivates the fibrinolytic system- kinins: produced by proteolytic cleavage of precursors, this group mediates vascular rxn and pain

136. what cells predominate inthe early inflammatory response and by what arethey later replaced?

- neutrophils predominate in the early inflammatory infil trate- later replaced by macrophages/monocytes

119. what changes in the cellcharacterize necrosis?

- changes in cytoplasm and nuclei- increased eosinophilia (i.e. pink staining from eosin dye) attributable to increased binding o eosin todenatured cytoplasmic proteins and in part to loss of the basophili a that is normally imparted by theRNA in cytoplasm- nuclear changes are all due to breakdown of DNA and chromatin and assume one of three patterns:karyolysis (fading), pyknosis (nuclear shrinkage), karyorrhexis (fragmentation)

95. what distinguishespathologic hyperplasiafrom cancer?

- hyperplastic process is controlled if the signals abate, it disappears- in cancer the growth control mechanisms become dysregulated or ineffective

78. what do mast cells release when stimulated by injury or release of chemokines?

release of histamines

146. what does persis tentstimulation of collagensynthesis in chronicinflammatory diseases leadto?

fibrosis of the tissue

75. what happens when acytotoxic T cell CD8receptor binds to a body cell MHC I?

- cytotoxic T cell releases perforins to kill cells that are infected or bad


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80. what is a general role of the complement system?

- inactive, free-floating proteins that are activated by proteolytic cleavage --> nonspecific defense- CLASSICAL PATHWAY: C4 and C2 each cleaved --> C4b and C2a bind --> together act as enzyme tocleave C3 --> C3a bind to leukocytes --> triggers inflammation --> C3b binds w ith C4b-C2a --> cleaves C5- ALTERNATIVE PATHWAY: spontaneous cleavage of C3 --> C3b binds to microbe surface --> bindsfactors --> cleaves more C3 --> eventually cleaves C5

165. what is amyloidosis? - a disorder characterized by the extracellular deposits of misfolded proteins that aggregate to forminsoluble fibrils- may result in excessive production of proteins prone to misfolding and aggregation; or incompleteproteolytic degradation of extracellular proteins- can be localized or systemic; associated with: deposits of light chain Igs/ monoclonal plasma cellproliferations, chronic inflammatory disease such as RA, Alzheimer disease, dialysis- deposits can cause tissue injury and impair normal function by causing pressure on cells and tissues- do NOT evoke an inflammatory response

57. what is another term for white blood cells?

leukocytes

129. what is apoptosis? how is it characterized? and

what are the twopathways?

- regulated mechanism of cell death that serves to eliminate unwanted and irreparably damaged cells , with the least possible host rxn-characterized by enzymatic degradation of proteins and DNA, initiated by caspases; and by recognitionand removal of dead cells by phagocytes

- mitochondrial/intrinsic pathway is triggered by loss of survival signals, DNA damage and accumulationof misfolded proteins (ER stress); associa ted with leakage of pro-apoptotic proteins from mitochondrialmembrane into the cytoplasm, where they trigger caspase activation; inhibited by anti-apoptotic membersof the Bcl family, which are induced by survival signals including growth factors- death receptor/extrinsic pathway is responsible for elimination of s elf-reactive lymphocytes and damage

by cytotoxic T lymphocytes; is initiated by engagement of death receptors (members of the TNF receptorfamily) by ligands on adjacent cells

152. what is cell-mediatedimmunity?

rxn of T lymphocytes, designed to combat cell-associated microbes, e.g. phagocytosed microbes and thosein cytoplasm and infected cells

170. what is CF? - cystic fibrosis is autosomal recessive disease caused by mutations in the CFTR gene encodingtransmembrane regulator- principal defect is chloride ion transport resulting in high salt conc. in sweat and viscous luminalsecretions in resp. and GI tracts- cardiopulmonary complications constitute the most common cause of death, pulmonary infections arefrequent- pancreatic insufficiency is common along with infertility- liver disease is increasing due to improved survival

174. what is DiGeorgesyndrome?

- deletion of genes at chromosomal locus 22q - gives rise to malformations affecting face, heart, thymusand parathyroids

177. what is fragile Xsyndrome?

- pathologic amplification of trinucleotide repeats of CGG (normally ~29; if 52-200 can expand to 4,000)causing loss-of-function (fragile X) or gain-of-function (Huntington)- most produce neurodegenerative disorders- fragile X from loss of FMR1 gene

- clinical features: mental retardation, macroorchidism, abnormal facial features172. what is galactosemia? - caused by inherited lack of GALT enzyme leading to accumulation of galactose-1-phosphate

- clinical features: jaundice, liver damage, cataracts, neural damage, vomiting and diarrhea, E. coli sepsis

178. what is genomicimprinting? which pai rof diseases result fromimprinting onchromosome 15?

- imprinting involves transcriptional silencing of the paternal or maternal copies of certain genes - oftendue to deletion- Prader-Willi syndrome results from deletion of paternal chromosomal region 15q12 - characterized by mental retardation, short stature, hypotonia, obesity and hypogonadism- Angelman syndrom results from deletion of maternal chromosomal region 15q12 - characterized by mental retardation, ataxia, seizures and inappropriate laughter


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162. what is GVHD? - graft-versus-host disease- when immunologically competent T cells are translanted into recipients who areimmunologically compromised- acute GVHD: days to weeks, epithelial cell necrosis in liver, skin, gut --> jaundice --> bloody diarrhea --> rash- chronic GVHD: may follow acute or occur insidiously, patient develops skin lesionsresembling SS and other symptoms that mick other autoimmune disorders

62. what is hematopoesis? - the development/maturation of B-lymphocytes- intentional shuffling of the variable portion of DNA for antibodies

153. what is humoral immunity? mediated by antibodies and is effective against extracellular microbes, i.e. those in circulationand mucosal lumens

132. what is inflammation? what are themain components of inflammation?

- a defensive host response to foreign invaders and necrotic tissue but it is itself capable of causing tissue damage- vascular rxn and cellular response; both are activated by mediators derived from plasmaproteins and various cells

84. what is ischemia? lack of blood flow

175. what is Klinefelter syndrome? - two or more X chromosomes with on Y chromosome- clinical features: testicular a trophy, s terility, reduced body hair, gynecomastia, eunuchoid

body habitus169. what is Marfan syndrome? - mutation to gene encoding fibrillin FBN1 which is required for structural integrity of

connective tissue- affected tissues include skeleton, eyes, CV system- clinical features: tall stature, long fingers, bilateral subluxation of lens, mitral valveprolapse, aortic aneurysm, aortic dissection

63. what is opsonization? - when antibodies bind to antigen on pathogen and mark them for destruction- can reduce pathogen activity - can cause coagulation

81. what is pathology? the study of disease, it involves the causes of disease and the associated changes at the levelsof cells, tissues, and organs which in turn give rise to the presenting signs and symptoms of the patient

171. what is PKU? - phenylketonuria is autosomal recessive disorder caused by lack of enzyme phenylalaninehydroxylase consequent inability to metabolize phenylalanine- clinical features: severe mental retardation, seizures, decreased pigmentation of skin- females who discontinue treatment can give birth to children with malformations due totransplacental passage of phenylalanine metabolites

159. what is SLE? - systemic lupus erythematosus- systemic autoimmune disease with manifestations including nephritis, skin lesions,arthritis, hematologic and neurologic abnormalities- underlying cause unknown; may include nuclear antigens, inherited susceptibility genes,environmental triggers

160. what is SS? - systemic sclerosis, aka scleroderma- characterized by progressive fibrosis involving the skin, GI tract, and other tissues- fibrosis may be the result of activation of fibroblasts by cytokines produced by T cells,triggers unkown- endothelial injury and microvascular disease are commonly present in the lesions of SScausing chronic ischemia

145. what is the consequence of excessive production of ECM?

can cause keloids in the skin

77. what is the general name forchemicals released by damaged/injured cells?

- chemokines


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123. what is the gross appearance of caseousnecrosis?

- "cheese-like" friable yellow-white appearance- tissue architecture obliterated- distinctive inflammatory border = granuloma

120. what is the gross appearance of coagulativenecrosis?

- tissues have firm texture- eosinophilic anucleate cells persist- characteristic of infarcts in solid organs except brain

124. what is the gross appearance of fat

necrosis?

- leaked activated pancreatic lipases liquefy membranes of fat cells in peritoneum

- released fatty acids combine with calcium to produce grossly visible chalky whiteareas (fat saponification)- occurs during emergency known as acute pancreatitis

125. what is the gross appearance of fibrinoidnecrosis?

- antigen and antibody deposits on walls of arteries- visible by ligh t microscopy - on H&E preparations bright pink appearance cal led fibrinoid

122. what is the gross appearance of gangrenous necrosis?

- limb that has los t blood supply and bacterial infection superimposed --> coagulativenecrosis + liquefactive action = wet gangrene

121. what is the gross appearance of liquefactive necrosis?

- often evoked from hypoxic death of CNS cells- dead cells digested into a liquid viscous mass- if bacterial --> acute inflammation --> material frequently creamy yellow pus

164. what is the HIV life cycle and pathogenesisof AIDS?

- virus entry into cells : requires chemokine receptor CD4, main targets CD4+helper Tcells, macrophages, DCs- viral replication- progression through acute infection of mucosal T cells and DCs, latent infection of cells in lymphoid tissue- mechanisms of immune deficiency: loss of CD4+T cells, defective macrophages andDC fxns , destruction of lymphoid tissue (late)

143. what is the importance of the extracellularmatrix related to tissue repair?

an intact ECM is required for tissue regeneration, and if the ECM is damaged, repaircan be accomplished only by scar formation

118. what is the relationship among cellularfunction, cell death, and morphologicchanges of cell injury?

- cellular function may be lost long before cell death occurs, and the morphologicchanges of cell injury (or death) lag far behind both- e.g. myocardial cells become noncontractile after 1-2 minutes of ischemia althoughthey do not die until 20-30 minutes of ischemia have elapsed - may not appear dead

by electron microscopy for 2-3 hours or by ligh t microscopy for 6-12 hours

86. what is the response of the myocardium topersistent load from hypertension orstenotic valves?

hypertrophy - increased size of the individual cells and ultimately the entire heart - togenerate the required higher contractile force

100. what is the reversible change in which oneadult cell type (epithelial or mesenchymal)is replaced by another adult cell type?

metaplasia

154. what is the role of the CD4+ helper T cells? - help B cells make antibodies, activate macrophages to destroy microbes, s timulaterecruitment of leukocytes- fxns regulated by cytokines- match with MHC II

155. what is the role of the CD8+ cytotoxic Tcells?

- kill cells that express antigens in the cytoplasm that are seen as foreign, e.g. virus-infected and tumor cells- match with MHC I


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70. what is the role of the helper T lymphocytecells?

- has CD4 receptor with variable region (analogous to B cell antibody variable region)- wh en T cell CD4 receptor binds to MHC II becomes activated (no longer naive)- wil l trigger proliferation of identical effector Th cells- and triggers proliferation of identical memory Th cells- releases cytokines to "sound alarm" and trigger immune response (activates cytotoxicT cells)- activates B cells by binding to their MHC II when presenting an antigen thatmatches T cell CD4 receptors

79. what is the sequence of events duringinflammatory response?

- invasion/injury --> chemokine release --> mast cell release of histamine --> vasodilation --> phagocyte first responders are neutrophils --> roll along capillary wall (marginalization) --> squeeze through capillary wall cells (emigration) intointerstitial fluid -->

138. what is the sequence of events in acuteinflammation?

- increased blood flow secondary to arteriolar and capillary bed dilation (erythema and warmth)- increased vascular permeability as a consequence of wither widening of interendothelial cell junctions of the venules or direct endothelial cell injury results inexudate of protein-rich extravascular fluid (edema)- leukocytes, initially neutrophils, adhere to endothelium via adhesion, leave andmigrate to site of injury under influence of chemotactic agents- phagocytosis, killing, and degradation of the offending agent follow - genetic or acquired defects in leukocyte fxn give rise to recurrent infections- outcome may be: removal of exudate and restoration of normal tissue; transition tochronic inflammation; or extensive destruction of tissue resulting in scarring

96. what is the shrinkage in the size of a cell by the combination of decreased proteinsynthesis and increased proteindegradation?

- atrophy - when a sufficient number of cells are involved the entire tissue organdiminishes in size but not dead- decrease in protein synth. because of reduced metabolic activity

87. what is the term for irreversible injury and cell death?

infarction

85. what is the word that describes narrowedheart valves?

stenotic

176. what is Turner syndrome? - partial or complete monosomy of genes on short arm of X chromosome, usually 45,X,less commonly from mosaicism- clinical features: short stature, webbing of the neck, cubitus valgus, CV malformations, amenorrhea, lack of secondary sex characteristics, fibrotic ovaries

64. what kinds of cells perform phagocytosis? - macrophages- dendritic cells- neutrophils

161. what leads to recognition and rejection of organ transplants (allografts)?

- response initiated mostly by host T cells that recognize the foreign HLA antigens of the graft, either directly (on APCs in the graft) or indirectly (after uptake andpresentation by APCs)- hyperacute rejection: pre-formed antidonor antibodies bind to graft endothelium

immediately after transplantation, leading to thrombosis, ischemic damage, and rapidgraft failure- acute cellular rejection: T cells destroy graft parenchyma (and vessels) by cytotoxicity and inflammatory rxns- acute humoral rejection: antibodies damage graft vasculature- chronic rejection: dominated by arteriosclerosis , probably caused by T cell rxn andsecretion of cytokines

97. what might cause atrophy of the brain inan 82 year old with atheroscleroticdisease?

aging (senile a trophy) and reduced blood supply


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128. what results fromcell injury by accumulation of damaged DNA andmisfolded proteins?

triggers apoptosis

65. what type of MHCmolecules are foundon macrophages?

MHC II for antigen presentation

73. what types of MHCgroup do regularcells have?

MHC I

167. what types of proteins areinvolved withautosomal recessivedisorders?

enzyme proteins are frequently involved

74. when a regular cellpresents proteins -

what reads themessage?

- regular cells present proteins on MHC I- read by CYTOTOXIC T cells with CD8 receptors

68. where do B cellsmature?

bone marrow

67. where do T cellsmature?

in the thymus

149. which are the mostefficient antigenpresenting cells?

dendritic cells which are located in epithelia and most tissues

76. which CD receptorsand MHC receptorsmatch up? on whichcells do each occur?

- professional antigen presenting cells (WBCs) have MHC II that bind with helper T cells with CD4- regular body cells have MHC I that bind with cytotoxic T cells with CD8 receptor

60. which cells are mostinvolved in the cell-mediated response?

- T-lymphocytes (helper & cytotoxic)- destroys infected self-cells

59. which cells are mostinvolved in thehumoral response?

- B-lymphocytes (antibody producers)- identify pathogens in plasma as non-self - though can be activated/amplified by helper-T cells

157. which Ig productionis stimulated by allergens?

- IgE- found on mast cells to react to reexposure

156. which moleculeconfers passiveimmunity toneonates?

antibodies passed from mom transplacentally or via breast milk

101. why does metaplasiaoccur? what is anexample of metaplasia?

- a cell type sensitive to a particular stress is replaced by another cell type better able to withstand the adverseenvironment- epithelial metaplasia is exemplified by the squamous change that occurs in the respiratory epithelium of habitual cigarette smokers; the normal ciliated columnar epithelial cells of the trachea and bronchi are focally or widely replaced by stratified squamous epithelial cells ; although the metaplastic squamous epithelium hassurvival advantages, important protective mechanisms are lost such as mucus secretion and ciliary clearance of particulate matter


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168. why females are less likely tohave an X-linked disorder?

- random inactivation of one X chromosome

102. why is epithelial metaplasia adouble-edged sword?

- the rugged stratified squamous epithelium may be able to survive the noxious chemicals incigarette smoke that the more fragile specialized epithelium would not tolerate- but the influences that induce metaplastic change, i f persistent, may predispose to malignanttransformation of the epithelium

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Pathology 3