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Flashcards in Pathoma Deck (285)
1

Describe the difference between hypertrophy and hyperplasia

Hypertrophy is the increase in gene activation, protein synthesis, and production of organelles.

Hyperplasia is the production of new cells from stem cells. Permanent cells cannot undergo hyperplasia. (Cardiac, Skeletal muscle, nerves)

2

How are cells decreased in size?

Ubiquitin proleosome degradation.

3

MetaplasiA

stress causes a change in cell type.
Barretts esophagusnon keratinized squamous to columnar in response to stomach acid.

4

In addition to cell stress, which vitamin deficiency can lead to metaplasia?

Vitamin A deficiency
Important in differentiation of specialized epithelial structures such as the eye.

5

Aplasia

Failure of cell production during enibryogenesis.

6

Describe the difference between slowly developing cell injury and acute injury in regard to ischemia.

Slow developing ischemia results in atrophy. Think renal artery atherosclerosis.

Acute ischemia results in cell injury (renal artery embolus)

7

Hypoxia

Low oxygen in the tissue.
Impairs ATP production leading to cellular injury.

8

Hypoxemia

Low Oxygen in the blood.
Normally it is 60 mm Hg. and 90% sat.

Think of high altitude.
Diffusion defects
V/Q mismatches
Decreased carrying capacity.

9

Cherry red appearance of the skin.

Carbon monoxide poisoning.

10

Iron in the blood is oxidized to Fe3+

Methemoglobinemia.
Seen with sulfa/nitrate drugs in new borns.

Treat with intravenous methylene blue.

11

Cyanosis with chocolate colored blood.

Methemeglobin
Treat with methylene blue.

12

What is the hallmark of reversible cellular injury?

Cellular swelling with loss of microvilli and membrane blebbing.
Think initial hypoxia.

13

What is the hallmark of irreversible cellular injuries?

Membrane damage.
Cytoslic enzyme leakage (cardiac troponin)

Cytochrome C leaking into cytosol from mitochondria activating apoptosis.

14

What is the morphologic hallmark of cell death?

Loss of the nucleus.
Nuclear condensation (pyknosis)
Fragmentation
Dissolution

Two mechanisms of cell death are necrosis and apoptosis.

15

Necrotic apoptosis

Necrotic tissue remains firm.
Characteristic of infarction of all organs except the brain (liquefactive)

16

Liquefactive necrosis

Liquid necrosis via cell lysis.

Brain lysis from microglial cells.

Abscesses from neutrophil enzymes.

Pancreatitis from pancrease enzymes.

17

Gangrenous necrosis

Coagulative necrosis
following ischemia of lower limb and GI.

Can be wet when there is an infection leading to liquefactive.

18

Caseous necrosis

Soft, cotage cheese like.
Combination of coagulative and liquefactive.

Very related to fungal infections and TB.

19

Fat necrosis

Chalky white necrotic adipose tissue due to calcium deposit.

Characteristic of trauma to fat. Trauma to breast etc.

20

Difference between dystrophic calcification and metatastic calcification.

Dystrophic occurs when there is necrosis and calcium binds to it.

Metatastic is due to excessive serum calcium levels leading to calcium deposits in normal tissue.

21

Fibrinoid necrosis

Necrotic damage to blood vessel wall.

Leads to exudate and bright staining of vessel wall.

Think hypertension and vasculitis.

22

How does the cytoplasm change with apoptosis?

Becomes eosinophilic (pink) and shrinks.

Proteins break down and the nucleus condenses.

Capsases then induce cellular injury and DNA damage.

23

What is the intrinsic mitochindrial apoptotic pathway?

Cellular injury, DNA damage or loss of hormonal stimulation leads to activation of Bc12.

Cytochrome c leaks out of inner mitochondrial matrix and activates capsases.

24

What is the extrinsic mitochondrial apoptotic pathway?

FAS ligand binds FAS death receptor on target cell activting capsases.

Tumor necrosis factor TNF can also bind leading to capsase activity.

25

How do CD8 cells trigger apoptosis

Perforins secreted
Granzyme enters pores from perforins and activate capsases.

26

how do free radicals cause cellular injury?

Peroxidation of lipids and oxidation of DNA/proteins.

27

What are the main antioxidants?

Glutathione
Vitamins A, C, and E.

28

Describe reperfusion injuries

return of blood to ischemia tissus results in the production of o2 derived free radicals that damage tissue.

Myocardial tissue will continue forming cardiac enzymes following reprofusion.

29

What are the common characteristics of amyloid?

Beta pleated sheets
Congo red staining
Apple-green birefringence under polarized light.

30

How will amyloid effect the kidneys?

Nephrotic syndrome
The kidney is the most common organ involved in amyloidosis.

31

What role does amylin have in type I diabetes?

Aniylin derived from insulin deposits into the beta islets of the pancrease.

32

Describe the role of amylin in alzhimers disease.

Amyloid deposits in the substantia nigrae (basal ganglia) in the midbrain.

33

What happens when a TLR is activated on a macrophage?

Leads to the upregulation of NF-kB which is a nuclear transcription factor that leads to production of immune mediators.

34

What role do prostaglandins have in inflammation?

Increase vascular permeability and pain.

35

What role do leukotriens have in inflammation?

These attract neutrophils
Mediate vasoconstriction
Bronchospasm
Increase vascular permeability.

36

What do mast cells release?

Histmine immediately leading to vasodilation and increased vascular permeability. (IgE)

Leukotriens are released as well in the delayed response.

37

Classic pathway of compliment

IgG or IgM mediated

38

Alternative pathway of complement

Microbrial products directly activate complement.

39

Mannose- binding lectin pathway

MBL binds to mannose on pathogen activating complement.

40

What do all three compliment pathways produce?

All three will produce C3 convertase that activate C5 .

C5 combines with 6-9 to form the MAC (attack complex)

41

Describe C3a and C5a

C3a and C5a both trigger mast cell histamine release.

C5a further chemoattracts neutrophils.

42

Hageman factor

Factor 12 relased by the liver that is activated by tissue collagen.

When activate starts coagulation and fibrinolytic systems, complement, and the kinin system.

43

Cardinal signs of inflammation

Rubor
Calor
Dolor
tumor
Loss of function

44

How is fever mediated?

Pyrogens cause macrophages to release IL-1 and TNF which increase COX activity in hypothalamus.

Raises the temp set point.

45

What allows neutrophils to roll?

Selectins
P-selectin = histamine
E selectin = TNF and IL-1

46

What allows neutrophils to perform adhesion?

ICAM and VCAM interacting with integrins.

47

Pt shows increased bacterial infections that lack pus, had a delayed separation from the umbilical cord at birth and increased circulating neutrophils.

Likely a neutrophil adhesion mutation in the integrins.

48

What enzyme allows for oxidative bursts?

NADPH interacting with O2

49

Chronic granulomatous disease?

Characterized by poor O2 dependent killing due to a deficiency in the NADPH oxidase defect.

Heavily suceptible to catalase positive critters!

Can test with the nitro blue tetrazolium test.

50

What are the antiinflamatory cytokines produced?

IL-10 and TGF

51

Which cytokine by macrophages recruits more neutrophils?

IL-8

52

Describe CD4 cell activation

APC cells present on MHCII

B7 on APC binds CD28 on CD4 cells.

53

What do TH1 cells secrete

CD4 cells that secrete IL-2 which is a Tcell growth factor and CD8 T cell activator.

Also secrete IFN gamma that activates macrophages.

54

What do TH2 cells secrete

CD4 cells that secrete IL-4 for B cel switch to IgG and IgE.

IL-5 which is for eosinophils and plasma cell maturation.

And IL-10 that inhibits TH1 phenotype.

55

what is the second activation factor for CD8 cells?

First is MHC1 interaction.

Second is IL-2 from CD4 TH1 cells.

then they are good to go NAVY SEAL.

56

What do all naive B cells have?

All have IgM and IgD

57

How do T helper clls interact with B cells?

T helper cells bind B cells via CD40 receptor binding the CD40L on the T helper cell.

In addition, the T helper cell secretes IL-4 and IL-5 to mediate B-cell isotype switching and plasma cell maturation.

58

what makes sure that granulomas around TB remain intact?

IFN-gamma from TH1 cells.

Converts macrophages to epitheliod histiocytes and giant cells that wall off the infection.

59

What is Digeorge syndrome?

Developmental failure of the third and fourth pharyngeal pouches.
22q11 microdeletion presenting with lack of thymus, hypocalcemia, and abnormalities of the heart, great vessels, and face.

60

What is Severe combined immunodeficiency?
SCID

Defective cell mediated and humoral deficiency.

Cytokine receptor defects
Adenosine deaminase deficiency
MHC II deficiency


Need to bubble baby this person and use stem cell transplant.

61

What is X-linked agamma globulinemia?

complete lack of immunoglobulin due to error in B-cell maturation.

Mutated tyrosine kinase.

62

What happens if there is an IgA deficiency?

Most common immunoglobulin deficiency.

Results in increased risk for mucosal infections especially viral.

63

What is Wiskott Aldrich syndrome?

Thrombocytopenia, eczema and recurrent infections do to a MUTATION IN THE WASP GENE X-LINKED.

Defective humoral and cellular immunity.

64

If one were deficient in C5-C9 compliment what are they at increase risk for infection of?

Neisseria infections!

65

what is seen when there is a C1 inhibitor deficiency?

Hereditary angiodema.

Beware of ACE inhibtors in these patients as edema of lips and skin can occur.

66

What is T cell negative selection.

The removal of self reactive T cells. Occurs in the thymus or bone marrow or become anergic.

67

What is Libman Sacks endocarditis?

Classic finding in lupus patients.

Small sterile deposits on both sides of the mitral valve.

68

What are lupus patients have mutation in?

Characteried by antinuclear antibody ANA (sensitive but not specific) and anti dsDNA antibodies (highly specific)

69

Which three drugs are common agents of drug induced lupus?

Hydralazine
Procainamide (Class 1A)
Isoniazid (RIPE therapy)

Cause lupus through antihistone antibodies.

70

What is Sjorgren syndrome?

Autoimmune destruction of the lacrimal and salivary glands. Characterized by ANA antibodies.

Type IV mediated (lymphocye mediated Delayed response)

Look for dry eyes (keratoconjuctivitis), dry mouth (xerostomia) and recurrent dental carries in older women.

71

Which lymphoma are Sjorgren syndrome patients at risk for?

B cell lymphoma.

72

What is scleroderma?

Autoimmune tissue damage with activation of fibroblasts and deposition of collagen.

Can be difuse or localized and almost any organ can be involved although the esophagus is most common.

Characterized by ANA and anti-DNA topoisomerase (Scl-70) antibody.

Prototype for localized is CREST syndrome with calcinosis, anti centromere antibodis, raynauds, esophageal dysmotility etc.

73

Describe the difference between labile, stable and permanent tissues.

Labile tissues possess stem cells and can reheal readily. (Small and large bowel, skin, bone marrow)

Stable tissue are quiescent tissues that can regenerate when needed (ex: Liver regeneration after hemisection)

Permanent tissue cannot regernate (myocardium, skeletal muscle, and neurons)

74

Which type of collagen is immediately layed down following an injury?

Type III collagen.

Later replaced with the high tensile strength type I collagent.

in order to remove type III collagen zinc is required as a cofactor.

75

What is the major mediator of tissue regeneration and repair?

TGI-alpha which is an epithelial and fibroblast growth factor.

76

what is primary and secondary intention healing?

Primary is when the wound edges are brought together via suturing.

Secondary intention involves the edges not being approximated. Granulation tissue fills the defect and myofibroblasts contract the wound forming a scar.

77

Why is vitamin C essential in scar formation?

It is needed to hydroxylate proline and lysin procollagen residues to allow for collagen cross linking.

Copper plays a role in cross linking as well.

Collegenase requires zinc to remove type III collagen after Type I tensile is layed down.

78

what collagen type is in excess during keloid formation?

Type III collagen excess.

79

Most common cause of cancer mortality in adults

Lung
Prostate/breast
Colorectal

80

what are the high risk HPV strains?

16,18,31,33

81

How does non ionizing radiation cause cell problems?

Sun rays
results in pyridine dimers in DNA normally excised from restriction endonuclease.

82

Describe the function of Ras

When bound to GTP it sends growth signals to the nucleus.

It normally inactivates itself.
Thus if its constantly on = increased growth factors.

83

What is the purpose of cyclins?

Cyclins and cyclin dependent kinases form a complex which phosphorylates proteins that drive the cell through the cell cycle.

84

what are the two tumor supression genes?

P53 and Rb (retinoblastoma) act to supress the risk of tumor supression.

85

Which stage of the cell cycle does p53 regulate?

Stops G1 from entering the S phase.

If there is DNA damage p53 stops the cells cycle and upregulates repair enzymes.
If it cannot be repaired p53 will induce apoptosis through the BAX pathway

86

C-myc mutation

Burkitt lymphoma

87

N-myc mutation

Neuroblastoma

88

L-myc mutation

Lung carcinoma
Transcription factor.

89

Describe how p53 can cause apoptosis.

p53 will upregulate the BAX

BAX disrupts Bcl12 allowing protein C to leak out of the mitochondria

90

What cell cycle stage is regulated by Rb

G1 to S phase by holding onto E2F transcription factor.

When its phosphorylated by cyclin D/ Cyclin dependent kinase 4 it will release E2F allowin cell cycle progression.

91

Which type of lymphoma is Bcl2 overexpressd?

Follicular lymphoma.

Thus bcl2 cannot be released from the mitochondria inhibiting the release of cytochrome c apoptosis factor.

92

What role does telomerase play in cell cancers?

Telomerase is generally upregulated preservin the telomeres.

FGF and VEGF are upregulated as well allowing for angiogenesis.

93

What occurs when E-Cadherins are down regulated in cancer?

These normally keep cells conjoined adjacently.

when down regulated metastasis can occur.

94

What type of collagen holds cells down to the baement membrane?

Type 4

95

What is lematrogenous cancer spread?

Common to sarcomas.
Invades the blood supply.

96

Which carcinoma is characterized by seeding of body cavities?

Ovarian.

Makes sense as it involves the peritoneum.

97

what is the most important prognostic marker for cancer patients?

Staging is key.
Far more important than grading.
T - Tumor size/depth
N - Tumor spread
M - Metastasis (important)

98

What is the difference between primary and secondary hemostasis?

Primary forms a platelet plug between the platelets and the vascular wall.

Secondary hemostasis stabilizes the platelet plug and is mediated by the hemostasis cascade.

99

Describe the steps of primary hemostasis.

Transient vasoconstriction via endothelin.

Platelets hook onto vWF sing GP1b.

Platelets release ADP promoting GPIIb/IIa binding and thromboxane via COX is released promoting aggregation and the primary plug.

100

what are the most common clinical signs of primary hemostasis disorders?

Mucosal and skin bleeding.

Look for petechiae and echymoses as well as purpura.

101

What are normal platelet ammounts?

150,000-400,000

<50,000 leads to symptoms.

102

What is immune thrombocytopenic purpura?

Autoimmune production of IgG against platelet antigens GPIIb/IIIa.

Most common form of thrombocytopenia in adults and children.

103

what is microangiopathic hemolytic anemia?

RBC are sheared as they cross microthrombi from platelets resulting in anemia with schistocytes.

104

What is Bernard Soulier syndrome?

Degect in GPfb thus platelet adhesion is impaired.

Look for a blood smear with thrombocytopenia and enlarged platelets.

105

Describe what would happen if there was a deficiency in GPIIB/GPIIIa

Platelet aggregation would be impaired.

106

How do NSAIDS disrupt platelet aggregation.

Inhibit the formation of thromboxane from COX.

107

What factor is required for the extrinsic clotting pathway vs the intrinsic?

Extrinsic requires factor 12

Intrinsic requires factor 7

108

What does PT measure?

Factor 7 and common factors
2,5, 10, and fibrinogen.

109

What does PTT measure?

Measures intrinsic factors
12,11,9,8

and common factors 1, 5, 10 and fibringen.

110

What is hemophilia A?

Genetic factor 8 deficiency.

It is X linked recessive mostly in males.

Look for deep tissue joint and post surgical bleeding.

111

How would PT and PTT be effected in someone with hemophilia A?

This is lack of Factor 8 clotting

Thus the extrinsic PTT would be prolonged with a normal PT.

112

What is seen in Von Willebrand disease?

Genetic vWF deficiency.
Most common inherited coagulation disorder

Impaired platelet binding, mild mucosal and skin bleeding.

Increase PTT bleeding time and a normal PT.

Keep in mind that VWF carries factor 8.

113

What is the treatment for von willebrand disease?

Desmopressin (ADH analog) which increases vWF release from Weibel palade bodies of the endothelial cells.

114

Which factors require vitamin K?

Factors 2, 7, 9, and 10 as well as proteins C and S.

Very common in newbords due to a lack of GI colonization by bacteria that normally synthesize vitamin K. Thus prophylatic vitamin K injections are norm.

115

What is disseminated intravascular coaulation?

Pathological activation of the coagulation cascade.

Leads to widespread microthrombi resulting in ischemia and infarction.

Lab values show decreased platelet count as well as increased PT and PTT times.

D-dimer will be extremely high as well.

116

What is the best screening test for DIC?

D-dimer.

Derived from splitting of cross linked fibrin; D-dimer is NOT produced from splitting of fibrinogen.

117

What does tissue plasminogen activator tPA do

Converts plasminogen to plasmin.

Plasmin cleaves fibrin and serum fibrinogen destroying coagulation factors and blocking platelet aggregation.

118

Patient comes in with increased PT and PTT, platelets however are in normal range.

D-dimer is in the normal range.

This is excessive tpa produced lysing all clots.

Treatment is aminocaproic acid.

119

Aminocaproid acid

Drug used to block the activation of plasminogen in the circumstance of excessive clot busting.

120

Lines of Zahn consist of?

These are deeply related to deep venous thrombosis.

Include layers of platelets/fibrin and RBC attached to vessel walls.

This is highly indicative of thrombosis.

121

What does protein C inactivate?

Factors 5 and 8

122

What is elevated in vitamin B12 and folate deficiency?

Homocysteine becomes elevated this can lead to increased rsk for thrombosis.

123

What is factor 5 Leiden?

Mutated form of factor V that lacks the cleavage site for deactivation by proteins C and S.

124

why is birth control use related to hypercoagubility?

Estrogen induces increased production of coagulation factors, therby increasing the risk for thrombosis.

125

When are gas emboli seen?

Seen when nitrogen gas percipitates out of the blood in divers.

126

What is the Bends?

Gas embolus causing joint and muscle pain in divers.

127

Where do systemic emboli usually originate

The left heart
Travel down systemic circulation to occlude flow to organs most commonly in the lower extremities.

128

What is mean corpuscular volume?
MCV

Microcytic MCV < 80
Normocytic 80-100
Macrocytic >100

Just think of what scores on an exam would be acceptable.

129

Why does microcytic anemia occur?

Decreased production of hemoglobin or protoporphyrin.

Bone marrow problems in hemoglobin division.

130

What is the most common type of anemia?

Microcytic due to lack of iron.

131

What is the function of ferroportin?

Enterocytes transport iron across the cell membrane into blood via ferroportin.

132

What is the function of transferrin?

Transports iron in the blood and delivers it to the liver and bone marrow for storage.

133

What is the role of ferretin?

Stored intracellular iron.
Stored within macrophages to prevent free radical formation.

134

What does total iron binding capacity measure?

Meaures the transferrin molecules in the blood.

Typically you want it to be low meaning there isnt a capacitance for more iron.

135

What parasite can cause iron deficiency in the elderly?

Hook worm!
Think ancylostoma and americanus.

136

What is normocytic anemia?

A stage of iron deficiency where the bone marow is making normal but low levels o RBC.

137

Iron deficiency anemia with an esophageal web and atrophic glossitis.

Pt will present with anemia and dysphagia as well as a BEEFY-RED TONGUE

Pluminer Vinson syndrome.

Treat with supplemental iron.

138

Hepcidin can be produced under what situations?

Can be produced under stages of chronic disease.

Hepcidin sequesters iron in storage sites by limiting iron transfer from macrophages to erythroid precursors and supressing erythopoieten.

139

How does hepcidin sequester free iron under circumstances of infection?

Decrease free iron stores by binding inside macrophages.

And decreases erythroid precursors by supressing erythropoieten.

140

What is sideroblastic anemia?

Anemia due to decreased protoporphyrin synthesis in the liver.

141

Describe the synthesis of protoporphyrin.

Aminolevulinic acid synthetase (ALAS) converse succinyl CoA to aminolevulinic acid (ALA) using vitamin B6!!!!!!!!!
(Rate limiting step)

Aminolevulinic acid dehydrogenase (ALAD) converts ALA to porphobilinogen.

Additional rxns convert porphobilinogen to protoporphyrin.

Ferrochelatase ataches protoporphyrin to iron to make heme.

142

What is necessary in the rate limiting step to form protoporphyrin?

Vitamin B6 is absolutely necessary.
Aminolevulinic acid synthetase converts succinyl CoA to aminolevulinic acid (ALA) using B6 (folate) as a co factor.

Without it you would end up with sideroblastic anemia.

143

Iron laden mitochondria showing iron rings in the mitochondria.

A sign of sideroblastic anemia due to the inability to use iron in the formation of hemoglobin.

144

what are the three aquired forms of sideroblastic anemia?

Alcoholism - Mitochondrial poisoning.

Lead poisoning - Inhibits ALAD and ferrochelatase stopping perphorin synthesis.

Vitamin B6 (folate) deficiency Cofactor for ALAS
(isoniazid treatment for tuberculosis can cause this)

145

What is thalassemia?

An anemia that is caused by decreased synthesis of the globin chains of hemoglobin.

146

Crew cut appearance of skull on X-ray with hypersegmented neutrophils and macrocytic anemia.

S Thalasmia major
Extremely severe form of thalassemia.

147

What is the most common cause of Macrocytic anemia?

MCV > 100
Most commonly due to folate or vitamin B12 deficiency leading to megaloblastic anemia.

148

Why do folate and vitamin B12 deficiencies lead to macrocytic anemia?

They lead to macrocytic/megaloblastic anemia because both are necessary for the formation of pyradines.

Folate circulates as THF and and donates a methyl group to B12. B12 is then able to donate a methyl group to homocystein producing methionine.

149

How does folate and vitamin B12 deficiency affect neutrophils?

Impaired division of granulocytic precursors leads to hypersegmented neutrophils.

150

What dietary food stuffs has folate?

folate can be absoarbed through vegetable and fruits and is taken up in the jejunum.

the body stores are minimal and so deficiencis develop within months.

151

How is vitamin B12 absoarbed in the gut?

Absoarbed by animal derived proteins.

Amylase from saliva liberate vitamin B12 that is then bound to an r binder and carried through teh stomach.

Pancreatic proteases in the duodenum detach B12 from the R binder and it then binds intrinsic factor in the small bowl for reabsorption in the ILEUM.

Vitamin B12 has years of storage in the liver and is less likely to be deficient.

152

What type of anemia will present if there is a lack of B12

Pernicious anemia.

Remember that folate deficiencies will cause megaloblastic anemia.

And that folate and B12 are dependent on eachother for DNA synthesis through the THF/ Homocysteine pathway.

153

In fatty acid synthesis, Vitamin B12 is a cofactor for what mechanism?

Converts methylmalonic acid to succinyl CoA.

Deficiencies wil then lead to increased levels of methylmalonic acid that impairs spinal cord myelinization leading to loss of vibration sense and spastic paresis.

154

Describe how B12 deficiency causes spastic paralysis and loss of vibratory sensation.

Vitamin B12 is used to convert methylmalonic acid to succinyl-CoA in fatty acid synthesis.

thus without it methylmalonic acid builds up in the system and causes nerve damage to the dorsal column and the lateral cortical spinal tract causing spastic paralysis and loss of vibratory sensation.


Note that this is with B12 deficiency, not folate. Makes for good differential.

155

What is extravascular hemolysis?

RBC destruction by the reticuloendothelial system (macrophages of the spleen, liver, and lymph nodes.)

156

What is intravascular hemolysis

RBC within the vessels due to hemoglobinemia, hemoglobinuria and hemosiderinuria.

157

What is hereditary spherocytosis

A type of normocytic anmia with predominant extravascular hemolysis.

Due to inherited defect of RBC cytoskeleton

158

Why do hereditary spherocytosis lead to anemia?

Spherocytes are less able to maneuver through the spleen and macrphages consume them leading to anemia.

treatment is splenectomy to stop the anemia, howell jolly bodies and spherulocytes will continue to exist.

159

What is the amino acid change in sickle cell anemia?

Autosomall recessive switch of glutamic acid with an abnormal valine that is hydrophobic.

HBS polymers aggregate when deoxygenated.

HBF is protective at birth.

160

what effect can hydroxy urea treatment at birth have?

Can increase HbF concentrations in the blood.

161

What are the microscopic changes seen with sickle cell anemia?

Target cells
and
Sickle cells.

162

What is the amino acid swap in hemoglobin C patients?

Normal glutamic acid is replaced by a lysine.

163

what is paroxysmal nocturnal hemoglobinuria?

Aquired defect in myeloid stem cells resulting in absent GP1 (holds decay accelerating factor inhibited) rendering the cell to destruction by compliment.

the hemolysis thn occurs episodically often during sleep at night. Look for intravascular hemolysis.

164

How can one test for paroxysmal nocturnal hemoglobinuria?

Sucrose test
Confirmatory test is acidifies serum test or flow cytometry to detect the lack of CD55 (DAF) on RBC.

165

What is glucose -6 -phosphate dehydrogenase deficiency?

X linked recessive
Reduced G6PD leads to cells being suceptible to oxidative stress.

Be careful with fava beans in these people.

166

What RBC type will be seen histologically following oxidative stress in G6PD deficient patients?

Heinz bodies.

Primaquine, sulfa drugs, and dapsone can all cause oxidative stress that would be dangerous in these patients.

167

What are heinz bodies?

Cells that have had a bite taken out of them by macrophages in the spleen.

Another name for it is bite cell.

Leads to intravascular hemolysis.

168

Which antibody tends to bind RBC in the heat?

Cold?

IgG in the heat (central body)

IgM in the cold (extremities) Think mycoplasm pneumo.

169

Direct coombs test

detects antibodies bound to a RBC.
Anti-IgG is added to the patients RBC.

170

Indirect coombs test

Confirms the presence of antibodies in a patients serum.

171

What is microangiopathic hemolytic anemia?

Intravascular hemolysis that occurs as RBC pass through the circulation.

172

If a patient has anemia as well as a low reticulocyte count what should you be considering?

At this point one should be considering the possibility of bone marrow corruption.

Think EPO destruction/kidney damage, bone marrow damage, or microcytic/macrocytic causes

173

What is aplastic anemia?

Damage to the hematopoietic stem cells resulting in pancytopenia

Which is anemia, thrombocytopenia, and leukopenia.

A bone biopsy will reveal fatty empty marrow.

174

What is leukopenia

Below normal WBC count
<5K

175

What is leukocytosis?

A high WBC count >10K

176

What do myeloblasts produce?

Neutrophils
Basophis
Eosinophils.

177

What type of lymphoma can cause eosinophilia?

Hodgkins lymphoma

178

What type of leukemia can lead to basophilia?

Myeloid leukemia.

179

The monospot test can screen for....

EBV
Usually positive within 1 week after infection.

180

EBV patients are at risk for forming a rash to what drug?

ampicillin.

181

Describe what markers state a patient has acute lymphoblastic leukemia

Seen most commonly in children and down syndrome.

TdT and DNA polymerase staining can be a sign.

182

Philadelphia chromososme

t9:22 seen most commonly in adults with acute lymphoblastic leukemia.

183

What staining can differentiate acute myeloid leukemia?

Myeloblasts
Crystal agregates of MPO seen as Auer rods.

184

What happens in chronic lymphocytic leukemia?

Neoplastic proliferation of naive B cells co-expressing CD5 and CD20.

Most common leukemia overall.

185

What will be seen in a blood smear with chronic lymphocytic leukemia?

Increased lymphocytes and smudge cells.

Look for hypogammaglobinemia as well. Most common cause of death in CLL.

186

Hairy cell leikemia

Neoplastic proliferation of mature B cells characterized by hairy cytoplasmic prosses.

Will be positive fo tartate resistant acid phosphatase TRAP.

187

Which type of leukemia will have a dry ta on bone marrow testing?

Hairy cell leukemia.

188

Describe chronic myeloid leukemia

Neoplastic proliferation of mature myeloid cells.

Driven by philadelphia chromosome 9;22

Which generates a BCR-ABL fusion protein with increased tyrosine kinase activity.

189

First line treatment for myeloid leukemia

Imatinib blocking tyrosine kinase activity.

190

What is polycythemia vera?

Neoplastic proliferation of mature myeloid cells especially RBC's

associated with AK2 kinase mutation.

LOOK FOR ITCHING AFTER BATHING.

TREAT WITH PHLEBOTOMY AND SECOND LINE HYDROXYUREA.

191

What is essential thrombocythemia?

Associated with JAK2 kinase mutation
Neoplastic proliferation of mature myeloid cells especialy platelets.

192

which part of the lymph node tends to enlarge with viral infections?

Paracortex T cell region

193

Which part of the lymph node tends to enlarge with rheumatoid arthritis and early stages of HIV infection?

The follicular region B cell.

194

Describe non-hodgkins lymphoma

Found in 60% of patients with malignant lymphoid cells.

Will arise as painless lymphadenopathy late in adulthood.

195

Describe hodgkins lymphoma

Look for the owl eye reed sternberg cells.

Will find in young adults

196

Which type of lymphoma is heavily associated wth inflammatory states such as hashimoto thyroidits, sjorgren syndrome and H. pylori gastritis?

Marginal zon lymphoma.

Gastric Maltoma is an example.

197

What is Burkitt lymphoma?

Neoplastic proliferation of intermediate sized B cells associated with EBV.

African form usually involves the jaw.

DRIVEN BY C-MYC GENE translocation t8;14 onto chromosome 8

198

Systemic hypertension

140/90 mm Hg

199

what is atherosclerosis

Intimal plaque obstructing blood flow

Nectotic lipid core in large/medium sized arteries.

Starts with fatty streaks early in life and progresses to atherosclerotic plaque.

200

what is arteriosclerosis?

Narrowing of small arterioles

Can be hyaline or hyperplastic types.

Hyaline is with long standing benign hypertension or diabetes.

Hyperplastic arteriosclerosis involvs thickening of vessel wall by hyperplasia of smooth muscle.

201

which part of the aortic wall is dislodged in aortic dissection?

Intimal tear with dissection of blood through the media of the aortic wall.

Think margans synrome and ehlers danlos syndrome.

202

What is a hemangioma?

Benign tumor comprised of blood vessels.

commonly present at birth and regresses during childhood.

203

What is an aniosarcoma?

Malignant proliferation of endothelial cells

Highly agressive.

204

what is kaposi sarcoma

Low grade malignant proliferation of endothelial cells associated with HHV8

205

Stable angina

Chest pain that arises with exertion or emotional stress.

due to atherosclerosis of the coronary arteries > 70%.

Likely ST depression due to subendocardial ischemia.

206

Unstable angina

Chest pain at rest look for ST depressions. and partially occluded coronary artery due to embolism.

Releived by nitro.

207

Prinzmetal angina

ST elevation due to full coronary artery vasospasm leading to transmural ischemia.

Releived by nitro or calcium channel blockers.

208

What is the most sensitive marker for MI?

Troponin I Gold standard
Rise 2-4 hours post and peak at 24 returning to normal by 7-10 days.

CK-MB is best for testing for reinfarcation as levels start over after 72 vhours.

209

What usually causes sudden cardiac death?

Usually due to fatal ventricular arrhythmia.

Mitral valve prolapse, cardiomyopathy and cocaine.

210

Presents with hypertension in the upper extremities and hypotension in the lowerextremities.

Coarcation of the aorta.
Look for notching of the ribs due to engorged intercostal arteries.

Associated with turner syndrome.

211

What is the most common cause of rhinitis?

Inflammation of the nasal mucosa
Adenovirus is the most common cause.

212

Most common cause of acute epiglottits?

Inflammation most commonly caused by H influenzae type b

213

Most common cause of laryngotracheobronchitis (croup)

Parainfluenza virus.

214

what type of HPV can cause laryngeal papiloma?

HPV6
HPV 11

215

What is the most common cause of secondary pneumonia superimposed on COPD?

Haemophilus influenzae

216

Most common form of pneumonia in Cystic fibrosis patients?

Pseudomonas

217

Most common cause of atypical pneumoniae in anfants?

Resiratory synctial virus (RSV)

218

Atypical pneumonia with high fever (Q fever) seen in farmers and vetrinarians?

Coxiella burnetti

219

Blue bloaters

Chronic bronchitis with excessive mucus plugs trapping carbon dioxide.

220

Alpha 1 anti trypsin is a rare cause of....

Emphysema
Results in severe panacinar emphysema in the lower lobes.


"Pink Puffer"

221

Pink puffer

Emphysema

222

What type of respiratory disorder is asthma?

Asthma is a obstructive dsorder

Stimulates TH2 cells that secrete IL-4 IgE and IL-5 IgA

223

Bronchiectasis

Permanent dilation of bronchioles and bronchi leading to loss of airway tone and air trapping.

Kartagener syndrome is a defect in dynein arm disrupting ciliary elevator.

224

What is sarcoidoisis?

Systemic disease characterized by noncaseating granulomas in multiple organs CLASSICALLY IN AFRICAN AMERICAN FEMALES.

Leads to a restrictive lung disease.

225

Neonatal respiratory distress syndrome

Seen when the type II pneumocytes arent making enough surfactant.

amniotic fluid lecithin to sphingoyelin ratio used for screening.

226

describe the two most common forms of lung carcinoma

Small cell carcinoma not amendable to resection.

Non small cell carcinoma treated with resection. Can include adenocarcinomas, sqamous cell carcinoma, and large cell carcinoma/carcinoid tumor.

227

Horners syndrome triad

Droopy eyelid - ptosis
Miosis (pinpoint)
Anhidrosis

Due to loss of the sympathetic chain.

228

In a pneumo thorax, the trachea is pushed to which side?

Pushed to the side of the collapse.

229

Difference between oral leukoplakia and a candida infection?

Candida can be scrapd off leukoplakia cannot.

230

What is mallory weissyndrome?

Logitduinal laceration of mucosa at the gastroesophageal junction caused by severe vomitting seen in alcoholism and bulmia.

231

Where does the distal esophageal vein normally drain into?

Drains into the portal vein via the left gastric vein.

Varices can occur when there is portal hypertension.

232

What is aclasia?

Disordered esophageal motility with the inability to relax the lower esophageal sphincter.

Loss of myenteric plxus.

T.Cruzi can cause this.

"Bird beak sign with barium swallow"

233

What is the most common type of cancer that barretts esophagus can become?

Adenocarcinoma.

234

What lymph nodes do esophageal carcinoma spread to when in the upper 1/3 middle 1/3 and lower 1/3

Upper 1/3 is to the cervical nodes
Middle 1/3 is to the mediastinal or tracheobronchial nodes

Lower 1/3 is to the celiac and gastric nodes.

235

Projectile nonbilious vomiting, visible peristalsis and olive like mass in the abdomen.

Pyloric stenosis!

Treatment is myotomy.

236

Most common site for an H. pylori infection?

The antrum.

Look for possible MALT lymphoma.

237

Therapy for H. pyori infections

clarithromycin
omeprazole
ampicillin

238

Duadenal ulcer

Almost always due to H. pylori.

Look for hypertrophy of the brunners glands.

239

Signet ring cells that diffusely infiltrate the gastric wall.

This is a type of gastric carcinoma.

Shows with thickening of the stomach wall.

Can involve the left supraclavicular lymph node (VIRCHOW node)

240

What is virchows node?

Left supraclavicular lymph node.

241

Celiac disease

Immune mediated damage of small bowel villi due to gluten exposure.

gliadin interacts with MHC II mediating cellular damage. Mostly IgA mediated against endomysium.

242

Tropical sprue

Damage to the small bowel villi due to an unknown organism leading to malabsorption.

243

Whipple disease

Systemic tissue damage characterized by macrophages loaded with T. whippeli organisms.

Clasically in the small bowel lamina propria.

244

Abeta lipoproteinemia

Lack B-48 apolipoprotein and thus cannot form chylomicrons.

VLDL and LDL will be absent.

245

Carcinoid tumor.

Malignant tumor that secretes serotonin
5-HIAA serotonin biproduct will be abundant in the urine.

Excess serotonin can cause carcinoid heart disease as well.

246

Inflammatory bowel disease

Chronic relapsing.inflammation of the bowel.

Cassically in young women/

247

Hirschsprung disease

Constant relaxation/peristalsis of the rectum and distal sigmoid colon associated with down syndrome.

Due to congenital failure of ganlion cells to migrate to intestins.

248

Which layers of intestine can the myenteric plexus be located between?

Located between the inner circular and outer longitudina muscle layers.

Gives motility.

249

Where can the submucosal /meissners plexus be located?

Found in the submucosa and regulates bood flow, secretions and absorption.

250

Irritable bowel syndrome

Relapsing abdominal pain with bloating, flatulence, and change in bowel habits.

Related to disturbed intestinal motility. No other identifiable changes.

251

Familial adenomatous polyposis

Autosomal dominant disorder characterized by numerous adenomatous coloninc polyps due to mutated APC gene.

Colon and rectum normally removed prophylacticly.

252

Peutz jeghers syndrome

Hamartomatous polyps in the GI tract and mucocutaneous hyperpigmentation on lips, oral mucosa, genital skin.

253

how does left sided colon carcinoma usually appear

Napkin ring appearance with decreased stool caliber, lower left quadrant pain and bloody stool.

254

How does right sided colonic carcinoma usually appear?

Grows as a raised lesion presenting with iron deficiency and vague pain.

255

Describe TNM stagins

T = Depth of the tumor
N = Spread to regional lymph nodes
M = Metastasis distant spread.

256

What serum tumor marker is great for assessing treatment response to colonic cancer?

CEA

257

What is annular pancreas?

Developmental malformation in which the pancreas forms around the duodenum.

258

Epigastric abdominal pain that radiates to the back with nausea and vomitting should have you thinking of...?

Pancreatitis.

Look for elevated serum lipase and amylase levels although lipase levels will be more specific.

259

What are the most common causes of chronic pancreatitis in adults and children?

Most commonly due to alcohol in adults

Cystic fibrosis in children.

However it can be idiopathic.

Look for fat soluble vitamin deficiencies.

260

which section of the pancrease does cancer most commonly arise?

Most commonly arise in the head of the pancreas.

261

What is trousseau's sign?

Migratory thrombophlebitis
Presinting with swelling erythema and tenderness in the extremetie.

Common to pancreatic carcinoma.

262

Whipple procedure

Removal of head and neck of pancrease.

Proximal duodenum

Gallbladder.

Used to treat pancreatic carcinoma.

263

What are the most common type of gallstones in the west?

Cholesterol stones.

264

Which liver fluke can cause biliary stones?

Clonorchis sinesis the chinese liver fluke

Treat with praziquantel.

265

Pt presents with exceedingly high levels of unconjugated billirubin that overwhelm the liver and cause jaundice

Begin thinking paravascular hemolysis or ineffective erylhropoieses.

Pt will present with very dark urine.

266

Giberl syndrome

Mildly low UGT activity in the liver leading to unconjugated jaundice during times of stress.

267

Crigler-Najjar syndrome

Absence of liver UGT enzyme leading to inability to conjugate billiruben.

Often fatal.

268

Dubin Johnson syndrome

Deficiency of bilirubin canalicular transport protein leading to a dark liver.

269

Hepatitis D depends on what for infection?

Hepatitis B infection to allow for a super infection.

270

In hepatitis B infections, what is the first serologic marker to ris?

HBsAG

271

In a chronic hepatitis B infection, what antigens/antibodies wil be present?

HBeAg indicates infectivity.

IgG should be present.

272

What is the most common cause of liver disease in the west?

Alcohol.

Will progress from a fatty liver to alcoholic hepatitis to cirrhosis.

Acelaldehyde (metabolite of alcohol mediates the damage). Leads to hepatic swelling and formation of mallory bodies.

AST > ALT for alcohol damage

ALT > AST for viral damage.

273

How could one differntiate fatty liver in obesity vs alcoholism?

ALT will be elevated in obesity

AST will be elevated in alcoholism.

274

What is the classic triad of hemochromatosis?

Cirrhosis
Secondary diabetes mellitus
Bronzing of the skin.

Use prussian blue stainin to look for lipofuscin and iron deposits.

275

Wilsons disease

Lack of copper transport into the bile.

Copper builds up in the hepatocytes leaks into the serum and deposits into tissues.

Cause Kayser-Fleisher rings in the cornea.

276

Reyes syndrome

Fulminan liver failure and encephalopathy in children with viral illness who take aspirin.

Likely mitochondrial damage of hepatocytes.

277

What structure does the horseshoe kidney wrap itself around?

Abnormally wrapped around the inferior mesenteric artery root as it descends from the pelvis to the abdomen.

278

Renal angenesis

Absent kidney formation

279

Polycystic kidney disease

Inhrited defect leads to bilaterally enlarged kidneys with cysts in the renal cortex and medulla.

280

Prerenal azotemia

Decreased blood flow to the kidneys.

Look for BUN elevation

281

Postrenal azotemia

Obstruction of urinary tract in ureters decreases GFR.

During early stages, BUN elevates in the blood and tubular function remains intact.

With long standing obstruction tubular damage occurs and sodium reabsorption/ ability to concentrate urine greatly decreases.

Urine osmolarity < 500 m OSM

282

Acute tubular necrosis

Injury and necrosis of the tubular epithelial cels.

look for necrotic cells plugging tubules decreasing GFR and causing brown casts to be seen in the urine.

283

What are the most common nephrotoxic agents?

Aminoglycosides
Heavy metals
Myoglobinuria.

284

Acture interstitial nephritis

Drug induced hypersensitivity involving the intersititium of the tubules.

Results in acute renal failure.

285

Renal papillary necrosis

Necrosis of the papillae

Caused by analgesic abuse long term phenacetin or aspirin use.

Diabetes
Sickle cell
Severe or acute pyelonephritis.

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