Exam 1 Flashcards

Question

Angina pectoris

Answer 1

"chest pain" in response to reaching the point of critical stenosis and the vessels are not able to respond in times of increased demand FANCY ANSWER: paroxysmal and usually recurrent attacks of substernal or precordial chest discomfort caused by transient myocardial ischemia that is insuﬃcient to induce myocyte necrosis

Answer 2

"Chest pain with exertion" A stable plaque that’s 70-80% occluded, so, when your heart requires, because of the activity, more oxygen delivered – increased contractility – your vessel cannot respond to that by delivering increased amount of blood and oxygen causing pain

Answer 3

"Chest pain at rest" You’re probably well above the 70% mark, approaching 80-90%. Even at base line contractility of the heart, the coronary arteries are not able to deliver as much oxygen as necessary. plaque disruption can then result in thrombosis and vasoconstriction without total occlusion, so you can develop a thrombus in that area as a response to plaque disruption. A person can go from unstable to stable

Answer 4

Plaques erode or rupture when they are unable to withstand mechanical stresses generated by vascular shear forces Plaque rupture results in the release of the necrotic lipid core, and rapid recruitment of platelets Plaque rupture is typically promptly followed by partial or complete vascular thrombosis

Answer 5

heart attack Death of cardiac muscle due to prolonged and severe ischemia The incidence of MI strongly correlates with genetic and behavioral predispositions to atherosclerosis

Answer 6

Ischemic heart disease

Answer 7

45% occur in people younger than 65 10% of MIs occur in people younger than 40 Blacks and white are equally aﬀected In middle age, men have a higher relative risk Women are generally protected during reproductive years Postmenopausal decline in estrogen production is usually associated with accelerated coronary artery disease

Answer 8

Coronary artery atheromatous plaque undergoes an acute change When exposed to subendothelial collagen and necrotic plaque contents, platelets adhere, become activated, and aggregate they are going to vasospasm and constrict further in response to these local mediators (so instead of dilating, they constrict). Tissue factor activates the coagulation cascade, adding the bulk of the thrombus Occlusion can occur within minutes Obstruction diminishes the blood flow to the region of the myocardium. Within seconds the oxygen will be depleted and without oxygen, aerobic metabolism will stop Noxious metabolites (lactate) accumulate Myocardial contractility ceases Ultrastructural changes occur in the myocyte Myoﬁbrillar relaxation, glycogen depletion, mitochondrial swelling (Reversible!!) In about 20 – 30 minutes, myocyte necrosis begins 2 – 3 hours of half thickness 6 hours for transmural (Irreversible!!)

Answer 9

``` Within 30 minutes Relaxation of myoﬁbrils Glycogen loss Mitochondrial swelling (Reversible injury) ``` 30 minutes – 4 hours Sarcolemmal disruption and mitochondrial densities No gross ﬁndings Subtle waviness of mycoytes at the border of the infarct 4 – 12 hours Beginning to have dark mottling Early coagulation necrosis, edema, and hemorrhage ``` 12 – 24 hours Dark mottling Ongoing coagulative necrosis Pyknosis of nuclei Hypereosinophilia of myocytes Contraction band necrosis Early neutrophilic inﬁltrate ``` 1 – 3 days Mottling with yellow-tan infarct center Coagulation necrosis with loss of nuclei and striations Brisk neutrophilic inﬁltrate ``` 3 – 7 days Hyperemic border with central yellow-tan softening Disintegration of dead myoﬁbers Dying neutrophils Macrophages at infarct border ``` ``` 7 – 10 days peak of macrophages maximally yellow-tan & soft depressed tissue at the site of the infarct brisk phagocytosis Granulation tissue at the margins ``` 10 – 14 days Red-gray depressed borders Well established granulation tissue with new blood vessels and early collagen deposition 2 – 8 weeks Grey-white scar from border to center Collagen deposition Decreased cellularity > 2 months Complete scar Dense collagen

Answer 10

3 – 7 days macrophages are chomping up on the dead myocytes and the nuclear debris.

Answer 11

So the earliest change that we find is sort of this wavy, maybe hyper-eosinophilic area - these are the earlier microscopic changes that we see. Next step in the process is the recruitment of neutrophils – this occurs in the 12-24 hour mark As we progress, neutrophils will be replaced by macrophages Macrophages[replaced] by smooth muscle cells & fibroblast depositing collagen Ultimately you’re left with fibrosis in that area if you survive & the development of a collagenous scar (bottom right)

Answer 12

most of the apex, the anterior wall of the LV, and the anterior 2/3 of the ventricular septum – The dominant artery perfuses the posterior 1/3 occlusions account for 40 – 50% of myocardial infarcts

Answer 13

the entire right ventricular free wall and the posterobasal wall of the LV occlusions account for 30 – 40% of myocardial infarcts

Answer 14

the lateral wall of the LV occlusions account for 15 – 20% of myocardial infarcts

Answer 15

Caused by occlusion of a vessel with full thickness necrosis of the myocardium Usually caused by chronic coronary atherosclerosis, acute plaque changes, and thrombosis

Answer 16

Partial thickness, although that can occur in complete occlusions The subendocardial zone is normally the least perfused region and is most vulnerable to disruptions in ﬂow May result from severe, prolonged reduction in systemic blood pressure in individuals with otherwise non-critical stenosis

Answer 17

Typically seen with pathology involving the small intramural vessels Microembolization, vasculitis, vasospasms like someone who uses cocaine

Answer 18

Typically present with prolonged chest pain described as crushing, stabbing, or squeezing Often associated with a rapid weak pulse, diaphoresis, and nausea and vomiting 25% may be entirely asymptomatic – Diabetics

Answer 19

diagnosed by clinical symptoms, laboratory tests, and characteristic EKG changes Laboratory diagnosis exploits blood levels of proteins that leak out of irreversibly damaged myocytes – Troponins, CK-MB, lactate Troponins rise later than all the other proteins but STAY HIGH LONGER after an MI***

Answer 20

``` Morphine to relieve pain Reperfusion Antiplatelet agents Anticoagulation Nitrates for induce vasodilation Beta blockers to decrease myocardial oxygen demand Antiarrhythmics to manage arrythmias Angiotensin converting enzyme to limit ventricular dilation Oxygen supplementation ```

Answer 21

3 – 5 days after an MI are most at risk you can end up with rupture of the left ventricular free wall which leads to hemopericardium or blood within the pericardial sac.. That blood will compress the heart, reduce its ability to contract & that leads to condition called cardiac tamponade. You can also develop ruptures of the ventricular septum if that’s the area that was infarcted.. This can develop a function ventricular septal defect & a left to right shunt & all of the complications that occur because of that. The papillary muscles in subendocardial ischemia or transmural.. can rupture which can lead to an onset of severe mitral regurgitation

Answer 22

progressive congestive heart failure as the result of accumulated ischemic myocardial damage usually appears after an infarction due to the functional decompensation of the hypertrophied, noninfarcted myocardium

Answer 23

Left ventricular hypertrophy Cardiac dilation Cardiomegaly Heart failure

Answer 24

most commonly stenosis, insufficiency, or some combination of both. Valvular stenosis is a failure of the valve to open completely, which is going to impede FORWARD flow Valvular Insufficiency results from a failure of a valve to CLOSE completely, so this leads to regurgitation or REVERSED flow, so pressure overload in the chamber prior to

Answer 25

Primary: defects & generation of the valvular tissue ITSELF Secondary or functional insufficiency: often happens because of DILATION of one of the CHAMBERS of the heart, so dilation of the left ventricle may prevent the proper closure of an otherwise normal valve

Answer 26

1. Aortic stenosis 2. Aortic insufficiency 3. Mitral stenosis 4. Mitral insufficiency So left sided heart problems are the ones that are the most common, most frequently encountered.

Answer 27

Calciﬁcation and sclerosis of anatomically normal or congenitally bicuspid aortic valves Consequence of recurrent chronic injury due to factors similar to atherosclerosis in other areas Hyperlipidemia Hypertension Inﬂammation Chronic progressive injury leads to valvular degeneration and incites deposits of hydroxyapatite (same calcium found in bone) Obstruction to the left ventricular outflow tract leads to gradual narrowing of the valve oriﬁce and an increasing pressure gradient across the valve Left ventricular pressures rise Left ventricular hypertrophy Systolic and diastolic dysfunction occur Angina, congestive heart failure, and death TXT: Valve replacement

Answer 28

Dilation of the ascending aorta secondary to hypertension or aging

Answer 29

Rheumatic heart disease Rheumatic fever is an acute, immunologically mediated, multisystem inﬂammatory disease classically occurring a few weeks following group A streptococcal pharyngitis Results from a host immune response to GAS antigens that cross react with host proteins Antibodies against streptococcal M proteins that cross react with cardiac self antigens Leads to complement activation, cytokine production, and T-cell/macrophage activation

Answer 30

Myxomatous degeneration Marked thickening of the spongiosa layer with deposition of myxomatous material Attenuation of the collagenous ﬁbrosa layer Secondary changes Fibrous thickening of the leaﬂets Fibrous thickening of the left ventricular endocardial surface Thrombi on the atrial surface of the leaﬂets

Answer 31

degenerative changes in the mitral valves typically aﬀect the ﬁbrous annulus Leads to irregular, stony hard, sometimes ulcerated nodules at the base of the leaﬂets Usually doesn’t aﬀect valvular function Regurgitation to due contraction of the valve ring Stenosis by impairing valve opening Arrhythmias due to penetration of calcium into the atrioventricular conduction system These calciﬁc nodules provide a site for thrombus formation Increase risk of embolic stroke and infective endocarditis

Answer 32

one or both of the mitral valve leaﬂets are ﬂoppy and balloon back into the left atrium during systole Aﬀects 2 – 3 % of individuals in the US 7:1 female to male ratio Often benign, but may lead to sudden cardiac death Leaﬂets are enlarged, redundant, thick, and rubbery The tendinous cords may be elongated, thinned, or ruptured Mitral valve annulus is dilated

Answer 33

focal inﬂammatory lesions are found in various tissues Aschoﬀ bodies in the heart Foci of T lymphocytes, occasional plasma cells, and activated macrophages Anitschkow cells Pathognomonic

Answer 34

Diﬀuse inﬂammation and Aschoﬀ bodies may be found anywhere in the heart Inﬂammation of the endocardium and left-sided valves result in ﬁbrinoid necrosis within the cusps or tendinous cords Overlying these necrotic foci are small vegetations (verrucae) Subendocardial lesions develop (MacCallum plaques

Answer 35

Mitral Valve

Answer 36

a ﬁsh mouth deformity of the valve (fusion of the commissures here so the normal mitral leaflets start to thicken, they get fused here at the commissures) Tight mitral stenosis Progressive left atrium dilation Pulmonary congestion and vascular changes Right ventricular hypertrophy

Answer 37

In contrast to mitral valve prolapse, where had thinning and elongation of the tendinous cords, we see thickening and reduplication of these cords which can cause further dysfunction

Answer 38

Characterized by Migratory polyarthritis of the large joints Pancarditis  inflammation of the heart muscle Subcutaneous nodules Erythema marginatum of the skin Sydenham chorea Involuntary, rapid, purposeless movements

Answer 39

microbial infection of the heart valves or mural endocardium (just adjacent to the valves) that leads to the formation of (infected) vegetations Often associated with destruction of the underlying cardiac tissues (as well as the valves themselves) Most infections are bacterial (but there are a wide variety of causes) Classiﬁed into acute and subacute forms

Answer 40

typically caused by infection of a previously normal heart valve by a highly virulent organism (Staph aureus) Rapidly produces necrotizing and destructive lesions Diﬃcult to cure with antibiotics alone

Answer 41

endocarditis is typically cause by organisms with lower virulence (Strep viridans) that cause insidious infections of deformed valves

Answer 42

Obvious infection Contaminated needle shared by IVDU Dental or surgical procedures

Answer 43

Sterile vegetations characterized by deposition of small sterile thrombi on the leaﬂets of the cardiac valves Single or multiple Nondestructive Illicit no inﬂammatory response May embolize

Answer 44

Mitral or tricuspid valvulitis with small sterile vegetations Occasionally encountered in systemic lupus erythematosus Single or multiple Located on the undersurfaces of the atrioventricular valves Associated with intense valvulitis **INFLAMMATION!!!*** **these are the only lesions that are located on the undersurface of valves****

Answer 45

Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy

Answer 46

most common (90% of cases) Characterized by progressive dilation and systolic dysfunction, usually with concomitant hypertrophy – Ejection fraction < 40% Genetic factors, alcohol, peripartum, myocarditis, hemochromatosis, chronic anemia, doxorubicin toxicity, sarcoidosis Enlarged, heavy, and ﬂabby heart – Dilation of all four cardiac chambers Mural thrombi are common May result in secondary valvular dysfunction Typically aﬀects those between 20 and 50 Presents with slowly progressive signs and symptoms of CHF – Dyspnea, fatigue, poor exertional capacity

Answer 47

common, clinically heterogenous, genetic disorder characterized by myocardial hypertrophy, poorly compliant left ventricular myocardium leading to abnormal diastolic ﬁlling, and intermittent ventricular outflow obstruction Thick walled, heavy, and hypercontracting Ejection fraction is 50 – 80% Autosomal dominant disorder with variable penetrance Banana-like left ventricular cavity

Answer 48

reduced stroke volume due to impaired diastolic ﬁlling Reduced chamber size Reduced compliance of the ventricle Those with signiﬁcant outflow obstruction develop increased pulmonary venous pressures and dyspnea

Answer 49

this is the characteristic young athlete, who had no problems but just died suddenly during the filed of play ``` Intramural arteries thicken Focal myocardial ischemia is common Atrial ﬁbrillation Ventricular arrhythmias Mural thrombi and embolization Cardiac failure Sudden death ```

Answer 50

characterized by a primary decrease in ventricular compliance resulting in impaired ventricular ﬁlling during diastole – Ejection fracture 45 – 90% Systolic function is usually unaﬀected

Answer 51

``` Radiation Amyloidosis Sarcoidosis Metastatic tumors Inborn errors of metabolism ``` (secondary changes)

Answer 52

a diverse group of pathologic entities in which infectious microorganisms and/ or a primary inﬂammatory process cause myocardial injury May cause direct myocyte injury or elicit a destructive immune response The intense cytokine response produces myocardial dysfunction out of proportion to the degree of actual myocyte damage Myocarditis is a PRIMARY disease, and should be distinguished from secondary causes of inﬂammation (ie, ischemic heart disease)

Answer 53

Viral infections are the most common cause of myocarditis in the US Coxsackie A and B and other enteroviruses Occasionally, CMV, HIV, and inﬂuenza may be implicated Nonviral agents are common outside of the US Trypanosoma cruzi, trichinella spiralis, toxoplasmosis, borrelia burgdorferi, and Corynebacterium diphtheriae

Answer 54

inﬂammation can occur secondary to a variety of cardiac, thoracic, or systemic disorders, metastases, or cardiac surgical procedures Primary pericarditis is rare (viral) Serous pericarditis Characteristically produced by non-infectious diseases (RF, SLE, scleroderma, tumors, and uremia) Fibrinous pericarditis Most frequent type of pericarditis Serous ﬂuid mixed with ﬁbrinous exudate Acute MI, postinfarction syndrome (Dressler syndrome), uremia, radiation, RF, SLE, and trauma

Answer 55

``` Myxomas (most common tumor in adults) Fibromas Lipomas Papillary ﬁbroelastomas Rhabdomyomas (most common pediatric tumor) Angiosarcomas ``` ******Most common heart tumor is a metastasis******

Answer 56

pseudostratified columnar with cilia and goblet cells

Answer 57

Type 1- where gas exchange occurs and really hard to see. Long thin cell with a very small nucleus. Gas exchange occurring across the cytoplasm. As you breathe in the oxygen. they are at high risk of dying so they have a high turnover rate. Type 2-can terminally differentiate and become type 1 cells. They also have macrophage functions. They also make surfactant.

Answer 58

what surfactant does is it follows La Place’s Law and it lines these airways. And it decreases surface tension. So by having a hydrophobic surface towards the center of the alveoli, the fluid is thinned out and surface tension is less. And that’s important because the second breath the child takes is against less resistance because the child is not born with great muscles, they’re thin as can be. . So they realized that if you gave surfactant to a child born before 24 weeks, they could survive. Also give steroids to the mother to jack up surfactant production by the type 2 pneumocytes.

Answer 59

TLC: total lung capacity (5 to 7 l) VC: vital capacity (3 to 5 l) TV: tidal volume (1 to 2 l) FVC: forced vital capacity (3 to 4 l) FEV1: forced expiratory volume in 1 second (2 to 3 l) FEV1/FVC ratio (60 to 70%)

Answer 60

You can have obstruction because there's something blocking the way (fluid or mucus) You can have decreased diameter because the muscular hypertrophy or construction You can have a loss of tether

Answer 61

Emphysema: abnormal permanent enlargement of airspaces without obvious fibrosis Chronic bronchitis: persistent cough with sputum for at least 3 months in at least 2 consecutive years Asthma: hyperreactive airways leading to episodic reversible bronchoconstriction

Answer 62

so you can breathe the air in. But you can't get that carbon dioxide out which is bad for two reasons. One, the carbon dioxide levels are going to rise in your blood. But number two you don't have room for more oxygen.

Answer 63

tip the balancing act towards the elastases and the enzymes will destroy the lung and break down the lung. (Neutrophil elastase Proteinase 3 Cathepsins Matrix metalloproteinases) and away from the "anti-enzymes" (1-Antitrypsin Secretory leukoprotease inhibitor Elafin Tissue inhibitors of matrix metalloproteinases) Example is Tobacco blocks the antitrypsin and leads to the influx of a lot of neutrophils; and neutrophils stimulate the release of these proteins as well. So anything that leads to break down the lung and prevents the breakdown of lung, will lead to dissolving are melting of the lung tissue and that's what causes emphysema.

Answer 64

1-2% of patients with COPD Autosomal recessive disorder 1AT is a serine protease inhibitor---elastase Made in the liver Defective 1AT does not neutralize elastase---emphysema Defective 1AT accumulates in the liver---cirrhosis Mutated SERPINA1 gene PiZZ---panacinar emphysema at a young age PiSS, PiMZ and PiSZ--reduced levels of normal---emphysema if smokers

Answer 65

You plug the airways with mucus . So a smoker is most risk of developing this because the tobacco smoke harms the respiratory epithelium and predisposes to infection. So then you keep getting infections of the airways you get bronchitis, you get pneumonia, you get bronchitis Bronchiolar and bronchial injury leading the bronchospasm, and hypersecretion of mucus, infection. Obstruction and airways continued in repeated injury, smoking, continued in repeated infection chronic bronchitis.

Answer 66

You got more goblet cells than normal. You’ve got inflammatory cells. You’ve got thickened muscle and way too many seromucinous glands. So all of that is a response to whatever this immune trigger is. Bronchoconstriction leads to muscle thickening. Whatever the insult is damages the epithelium and leads to more seromucinous gland production of fluids and also hypertrophy of the seromucinous glands. Asthma Focus on T-cells

Answer 67

an antigen being presented by a macrophage and then shit really hits the fan because the T cells get revved up and they stimulate the B cells which secretes the antibodies that cross link on the mast cell. That lead to lead mediator release of leukotrienes, cytokines, and histamines and cause bronchospasm and edema and airway inflammation and so on. Still don't have a way to stop the T cells, so we treat asthma the same old fashioned way with bronchodilators and steroids. Bronchodilators to help the airways stay open and steroids to try and knock down this inflammatory cascade.

Answer 68

Incomplete expansion or collapse of lung (airless lung) REVERSIBLE DISORDER 3 types Resorption atelectasis: due to airway obstruction Compression atelectasis: due to pleural cavity expansion hemothorax, pneumothorax Contraction atelectasis: due to lung or pleural fibrosis

Answer 69

``` Spinal disorders Neurologic disorders Sarcoidosis Hypersensitivity pneumonitis Pneumoconiosis Idiopathic ```

Answer 70

Extrinsic allergic alveolitis Immunologic reaction to inhaled antigens Antigens not identified in up to 66% of cases Acute and chronic presentations ACUTE PRESENTATION Single large bolus exposure to antigen Dyspnea, cough, fever/chills 4-6 hours later ``` CXR: diffuse granular infiltrates Pathology: Pulmonary edema Pathogenesis: Type III hypersensitivity reaction (immune complex disease) Prognosis: Improvement in a day or so Reexposure: Recrudescence ``` CHRONIC PRESENTATION Prolonged exposure to small amounts of antigen Insidious dyspnea, dry cough, fatigue CXR: Mostly upper lobe interstitial reticulonodular infiltrates Lab: serum antibodies, skin tests? BAL: Increased CD8+ lymphocytes Pathology: Chronic bronchiolitis, interstitial pneumonia, and granulomas Pathogenesis: Type III and IV hypersensitivity reactions Prognosis: Improvement in 33%, stable in 33%, worsening in 33% if antigen not removed

Answer 71

Pathologic response related to: intensity of exposure duration of exposure quantity of exposure size of particle physiochemical properties of particle route of clearance efficiency of clearance host response interactions with other environmental pollutants

Answer 72

``` Asbesto Silica Silicates (talc, kaolin, mica) Mixed dust Coal Metals ```

Answer 73

Vesicle: a circumscribed collection of free fluid less than 0.5cm in diameter Bulla: a circumscribed collection of free fluid greater than 0.5cm in diameter Pustule: a circumscribed collection of purulent exudate that varies in size (pimples) Cyst: a cavity containing fluid or semisolid material surroudned by an epithelial layer

Answer 74

Freckle Lentigo Melanocytic Nevi Melanoma

Answer 75

small- Focal abnormality in pigment production Hyperpigmentation: increased amount of melanin pigment. Normal density of melanocytes

Answer 76

Liver spots- age spots They’re not related to sunlight exposure, they are stable in color that’s why they are very difficult to remove. The pathology is unknown

Answer 77

Addison’s disease | Peutz-Jeghers

Answer 78

Adrenal failure Activation of pituitary gland leading to increased ACTH and MSH . Stimulate melanocytes in the skin and mucosa

Answer 79

INHERITED!!! (they’re going to have a family history) They’ll also have other things in the skin and GI tract too.

Answer 80

Café au lait spots Histologically similar- larger and arise independently of sun exposure Neurofibromatosis is more a genetic disease and this is only one of the manifestations of that because the location of the skin lesions, different types of tumors, and even manifestations on the eyes too.

Answer 81

Hypopigmentation (loss of melanocytes or melanin production) Melanocytes are destroyed White patches of skin May be an autoimmune disease May be associated with another autoimmune disease

Answer 82

Benign neoplasms Numerous subtypes Acquired are the most common type

Answer 83

``` May be direct precursors of melanoma Many never progress Mutations or epigenetic changes NRAS and BRAF genes Inherited loss of function mutations in CDKN2A ```

Answer 84

Most deadly of all skin cancers Strongly linked to acquired mutations caused by exposure to UV radiation in sunlight Relatively common neoplasm Some studies suggest that periodic sunburns early in life are the most risk factors Predisposing factors/environmental factors Mutations in cell cycle regulators Blistering sunburn can double the chances of developing melanoma later in life Strong dose-response relationship

Answer 85

``` Asymmetrical Borders (irregular) Color Diameter (1/4in or 6mm) Evolving (changing) ```

Answer 86

Antibody Therapy Chemotherapy

Answer 87

have a 20% percent higher risk of melanoma have an 87% higher risk of melanoma if they start tanning before 35 are 2.5 times more likely to develop SCC and 1.5 more likely to develop Basal cell carcinoma

Answer 88

Seborrheic Keratoses Acanthosis Nigricans Fibroepithelial Polyp Epithelial or Follicular Inclusion Cyst

Answer 89

Benign Fibrous Histiocytoma Dermatoﬁbrosarcoma Protuberans

Answer 90

Mycosis Fungoides Mastocytosis

Answer 91

Dark velvety patches May be an important sign of underlying conditions GI Adenocarcinomas- middle aged and older individuals Type 2 diabetes

Answer 92

skin tag intraorally associated with an area of trauma usually

Answer 93

Actinic Keratosis Squamous Cell Carcinoma Basal Cell Carcinoma

Answer 94

Sun damaged skin-hyperkeratosis Exposure to ionizing radiation and arsenicals It seems like the skin is very dry, but they're going to have this type of ulcerations.. But these ulcerations are not healing

Answer 95

DNA damage induced by exposure to UV light P53 dysfunction Can progress from actinic keratosis, chemical exposure, thermal burn sites or in association with HPV infection in the sitting of immunosuppression Cutaneous cell carcinoma has potential for metastasis but is less aggressive than squamous cell carcinoma at mucosal sites

Answer 96

Locally aggressive tumor Rarely metastasize Pearly papules containing prominent blood vessels ( telangiectasias) Advanced lesions may ulcerate

Answer 97

Urticaria Acute Eczematous Dermatitis Erythema Multifomre acute lesions-days to weeks Inﬂammatory inﬁltrates-edema

Answer 98

hives – mast cell degranulation Antigen induced release of vasoactive mediators from the mast cells

Answer 99

T cell-mediated inﬂammatory reactions (type IV hypersensitivity) Acute allergic reaction due to antigen exposure

Answer 100

Uncommon – self-limited hypersensitivity reaction to certain infections and drugs Macules, papules, vesicles, bullae Any age Herpes simplex, penicillin, barbiturates, salicylates, cancer (lymphomas), lupus, polyarteritis nodosa. CHARACTERISTICS Target like lesions Central necrosis Macular erythema

Answer 101

A febrile form associated with extensive involvement of the skin Fever-ﬂu like symptoms Often in children but not exclusively Blisters Complications: Dehydration, sepsis pneumonia Multiple organ failure Etiology Medications- Antibiotics, carbamazepine and others SLE HIV/AIDS Immune reaction

Answer 102

Defuse necrosis- cutaneous and mucosal epithelial surfaces Flu like symptoms A history of drug exposure Large blisters More severe than SJS Dehydration, sepsis, pneumonia and multiple organ failure

Answer 103

It is called SJS when less than 10% of the skin is involved Diagnosis is based on a skin biopsy Hospitalization Skin regrows over 2 to 3 weeks. Recovery can take months

Answer 104

Psoriasis - This is autoimmune. Again you can have different severities, different locations, different manifestations. Some ppl have very little or have extension on almost every part of skin. Seborrheic Dermatitis - more common than psoriasis- regions with high density of sebaceous glands- scalp and forehead, nasolabial folds Flaky scales Unknown etiology Lichen planus Pruritic, purple, polygonal, planar, papules and plaques Disorder of skin and mucosa White dots or lines, Whickham striae

Answer 105

Pemphigus Bullous Pemphigoid Dermatitis Herpetiformis

Answer 106

Inﬂammatory- Pemphigus- Autoantibodies Primary lesions are superﬁcial vesicles and bullae that rupture easily Oral ulcers may persist for months

Answer 107

Chronic inﬂammatory subepidermal blistering disease

Answer 108

Inﬂammatory disorder Blisters- Itchy papulovesicular eruptions May be associated with Celiac disease Bilateral and symmetric

Answer 109

Epidermolysis Bullosa - blisters at sites of pressure, rubbing or trauma Inherited disorder- minor to fatal Porphyria

Answer 110

Metabolic disorders- excretion of porphyrins- purple red pigments Genetic factors and environmental factors Sensitivity to light Lesion in the skin- blistering Aﬀects the nervous system Attacks may be triggered by smoking stress and certain medications Complications High blood pressure Chronic kidney failure Live damage

Answer 111

Acne vulgaris | Rosacea

Answer 112

Hair follicles clogged Primarily- areas with high number of oil glands Genetics Role of diet and smoking is unclear Hormones

Answer 113

Common skin disease Etiology unknown- Triggers- heat, stress, sunlight, alcohol, caﬀeine, spicy foods People over 30 Flare-ups Redness on nose, cheeks, chin and forehead Dilated blood vessels Papules, pustules and swelling May be burning and sorenes

Answer 114

Group of diseases Inﬂammation of the subcutaneous adipose tissue Skin tender nodules Erythema Induratum ( nodular vasculitis) TB, hepatitis C Erythema nodosum- Associated with infections, IBD, oral contraceptives, pregnancy sarcoidosis

Answer 115

Verrucae (Warts) Molluscum Contagiosum Impetigo Superﬁcial Fungal Infections

Answer 116

Gingival bleeding often the first sign of a bleeding disorder Dental extractions often herald bleeding diatheses

Answer 117

Maintenance of clot-free, flowing blood within the vascular system, while also allowing the rapid formation of a solid clot to close ruptures/injury

Answer 118

The dissolution/breaking apart of fibrin clots; a normal component of hemostasis

Answer 119

the formation of a clot within the uninterrupted vascular system; a pathologic extension of hemostasis

Answer 120

A condition which makes the body tissues react with heightened susceptibility; clot too easy (e.g., bleeding diathesis)

Answer 121

Any disorder of blood coagulation (bleeding or thrombosis)

Answer 122

Blood Vessel Injury (usu. endothelial) Immediate Vasoconstriction (neurogenic) Platelet Adhesion to Collagen Platelet Activation Coagulation Cascade-Permanent Fibrin/Platelet Clot (Induction of Fibrinolysis)

Answer 123

The vascular endothelium, when uninjured, it has an anticoagulant function, or blood flow function and when injured, it has a pro-coagulant function.

Answer 124

Dense Bodies and Alpha Granules

Answer 125

Adhesion & Shape Change: platelets adhere to nonendothelial injury site (e.g., collagen). Big players: vWF, GPIb Platelets bind to vWF via GP1b on its surface basically forming the bridge that allow the platelet to adhere to the collagen. Secretion: Alpha granules and dense bodies released. Aggregation: platelets attach to each other via GPIIb/IIIa and fibrinogen which is one of the many coagulation proteins that normally circulates in your vascular system and is made in the liver. So fibrinogen is the bridge b/w platelets in the step of aggregation.

Answer 126

In the endothelial cells | *only one of the coagulation proteins not made in the liver

Answer 127

Ca++ critical in all stages of platelet activation; the more Ca++ in platelets, the more activation there is

Answer 128

The more vessel injury there is, the more platelet activation there is

Answer 129

Starts with Tissue Damage When there's tissue damage, there's something called tissue factor released from the endothelium, and it initiates the coagulation cascade. the coagulation cascade starts with INACTIVE Factor 7 which (with the tissue factor and the platelet activation) turns factor 7 to factor 7A. “A” means that its ACTIVATED activated factor seven then jumps right into this final pathway of the coagulation cascade. This final pathway is called the common pathway. The final common pathway always ends with factor 10 getting activated. Then factor 5 gets activated through factor 2 (Prothrombin --> Thrombin (IIa)) And Thrombin (IIa) transforms Fibrinogen is into fibrin.

Answer 130

Begins with more subtle microscopic injuries are called intrinsic injuries. As a result of that, factor 12 is activated to factor 12A, factor 11 then factor nine, then finally factor 8.

Answer 131

Factors II, VII, IX, and X

Answer 132

if the gall bladder is not functioning properly, you can't absorb a fat-soluble vitamin like vitamin K, you might have a bleeding disorder because 4 factors are going to be effected by deficiency of vitamin K

Answer 133

run a coagulation profile

Answer 134

Incidental lab result in a pre-op or medical work-up Patient presents with bleeding complaints Patient gives a personal or family history of bleeding

Answer 135

``` Platelet count (CBC) Bleeding Time (BT) PT PTT Specific Factor Assays ```

Answer 136

150,000-400,000

Answer 137

Qualitative Platelet Defects and Vessel Wall/CT Disorders like Marfan's syndrome

Answer 138

Thrombocytopenia

Answer 139

Deficiencies of Extrinsic Pathway (i.e., VII)

Answer 140

Deficiencies of Intrinsic Pathway

Answer 141

Deficiencies of Common Pathway

Answer 142

1) Bleeding Diathesis (aka abnormal bleeding) | 2) Thrombotic Diathesis

Answer 143

Relatively common Mostly acquired and often treatable Mostly small hemorrhages (petechiae, purpura) Coag. Profile: Normal or +/- prolonged BT structural abnormality of the blood vessels and connective tissue disroders like Marfans, or something like that.

Answer 144

Infectious (esp. meningococcemia, rickettsiae, bacterial endotoxins) Drug-Induced Vasculitides Collagen-Vascular Diseases (e.g., SLE) Age (poor connective tissue, malnutrition) Vitamin C Deficiency (scurvy)-rare Cushing’s Syndrome (wasting)

Answer 145

Ehlers-Danlos Syndrome (connective tissue disorder) Henoch-Scholein Purpura (vasculitis) Hereditary Hemorrhagic Telangiectasia

Answer 146

– Thrombocytopenias (reduced platelet count) – Thrombocytopathies (defective platelet function)

Answer 147

Vast majority acquired/iatrogenic Signs/symptoms: (small vessel) bleeding from skin, mucous membranes of gingiva, GI, and GU tracts (hematuria)--petechiae, purpura Bleeding from surgery or trauma when counts 20-50,000 Spontaneous bleeding only if <20,000 Increased risk of intracranial bleeds if <10,000 Coag. Profile: low plt count, prolonged BT

Answer 148

Infiltrative bone marrow diseases (leukemias, metastatic cancers) Defective platelet production (aplastic anemia, suppressive drugs/ chemotherapeutic agents, alcoholism, B12/ folate deficiency, HIV/AIDS) Increased platelet destruction in blood (infectious agents, certain drugs, splenomegaly, anti-platelet Ab’s in ITP & AIDS, TTP, prosthetic heart valves, HTN, DIC, atherosclerosis) “Dilutional thrombocytopenia” (from massive transfusions) (Rare hereditary diseases)

Answer 149

Defective platelets Vast majority acquired/iatrogenic Similar presentations to patients with thrombocytopenias Coag. Profile: Normal platelet count, prolonged BT

Answer 150

**Aspirin (ASA): 1000mg prolongs BT significantly and irreversibly for 10 days! NSAID’s (ibuprofen, indomethacin, etc.): effects last only 1-2 days, except ibuprofen, which lasts 10 days as ASA does Uremia (end stage renal disease) Liver Disease Alcoholism

Answer 151

Bernard-Soulier Syndrome: Aut. rec., GPIb deficiency--adhesion dysfunction Glanzmann’s Thrombasthenia: Aut. rec., GPIIb/ IIIa deficiency--aggregation dysfunction Storage Pool Disorders: Usually aut. rec., alpha and dense granule deficiencies--secretion dysfunction

Answer 152

Vast majority acquired Presentation: Deep bleeding (e.g., hemarthroses, GI/GU bleeding), ecchymoses (bruises)/hematomas following trauma, prolonged bleeding following a cut or surgery. Petechiae/purpura UNCOMMON Coag. Profile: (usu.) Normal plt count; prolonged PT &/or PTT

Answer 153

Vitamin K Deficiency &/or Intestinal Malabsorption Liver Disease Renal Insufficiency/Failure DIC Acquired vWD Acquired Anti-Factor Antibodies (Inhibitors)

Answer 154

Deficient factors II, VII, IX, and X Prolonged PT (and PTT) Common in malnourishment, alcoholism, gallbladder disease/biliary obstruction, intestinal disease (with malabsorption), neonatal period (esp. preemies and breast-fed neonates) Treatment: Oral/IM/IV Vitamin K PT/PTT correct (if liver healthy) within days with oral tx, 24hrs with IM tx, and 3hrs with IV tx

Answer 155

All coag. factors (except vWF) synthesized in liver; therefore, all potentially affected in severe liver disease (e.g., cirrhosis) 85% of liver disease patients have at least one hemostatic abnormality 15% have clinical bleeding Vit K-dependent factors affected first Coag. Profile: prolonged PT early in disease course; prolonged PTT only with severe disease; (+/- decreased platelet count &/or prolonged BT) Treatment: – Fresh Frozen Plasma (FFP)-has all factors except XII – Treat underlying liver disease, transplant

Answer 156

Decreased factor production in damaged liver cells (bleeding) Production of abnormal (nonfunctional) factors (bleeding) Decreased platelet # or function (bleeding) Impaired clearance of activated factors (thrombosis)

Answer 157

Complex, secondary complication to a variety of conditions Patients usually hospitalized &/or severely debilitated (sepsis, malignancies, shock, severe burns, transfusion reactions, liver disease, **obstetrical complications) Begins as excessive, multifocal thromboses; after a period, platelets and factors consumed, causing life- threatening hemorrhage &/or thromboses (strokes, MI’s, ARF) Coag. Profile: Prolonged PT and PTT, decreased platelets and fibrinogen, increased fibrin split products Treatment: Very difficult; double edged sword; treating underlying condition is best approach, but often impossible

Answer 158

Von Willebrand’s Disease (VWD) Hemophilia A Hemophilia B (Christmas Disease) (All other factor deficiencies-exceedingly rare)

Answer 159

Very common (frequency 1%), but highly variable & often subclinical until a procedure is performed (often dental!) Most cases autosomal dominant; variable family histories; men and women equally affected 3 types: I-III (mild-severe), III being rare and aut. recessive Defective platelet adhesion and decreased Factor VIII level/activity Variable Presentations: Asymptomatic to spontaneous bleeding from mucous membranes, menorrhagia, etc. Usually similar to platelet coagulopathy presentations

Answer 160

(variable) +/- prolonged BT; normal plt count; +/- prolonged PTT * *Abnormal Ristocetin Test, decreased Factor VIII levels Diagnosis: often difficult due to variability of disease; must have a high index of suspicion Treatment: Cryoprecipitate (VWF + VIII) before surgical/dental procedures

Answer 161

the most common hereditary coagulation disorder causing serious (deep) bleeding Factor VIII deficiency &/or dysfunction X-linked recessive: Mothers are carriers, passing disease on to their sons (only rare female carriers with symptomatic disease) Presentation: Deep bleeding, hemarthroses with crippling deformities; petechiae and ecchymoses characteristically absent 30% with no family history (brothers, maternal uncles, etc.); new mutations? Coag Profile: Normal platelet count; Prolonged PTT; decreased Factor VIII assay or activity Treatment: Frequent cryoprecipitate or **Factor VIII concentrate (recombinant lowest risk of disease transmission) Complications: Crippling joint deformities, AIDS, hepatitis, hemochromatosis, life-threatening hemorrhages

Answer 162

6-50% Factor VIII activity = mild disease 2-5% Factor VIII activity = moderate ds < 2% Factor VIII activity = severe ds

Answer 163

X-linked recessive (mother to sons) Factor IX deficiency or dysfunction Clinically indistinguishable from Hemophilia A Presentation: Depends on Factor IX level/ activity; from asymptomatic to deep bleeding/hemarthroses Coag. Profile: Normal plt count; prolonged PTT & reduced Factor IX assay/activity Treatment: Factor IX concentrate

Answer 164

Hematologic Abnormalities: Excessive platelet production (e.g., essential thrombocytosis), RBC production (p. vera, COPD), or WBC production (leukemias) Malignancies (hypercoagulability, DIC) Multiple Myeloma (increased protein in circulation) Oral Contraceptives, Pregnancy Smoking, Atherosclerosis Prosthetic Heart Valves Post-operative Periods (immobilization and increased platelets) (Rare) Hereditary Deficiencies of Natural Coagulation Inhibitors

Answer 165

Deﬁnition: Reduction in red cell measurement on CBC: Hb less normal range or decreased Hct (ratio of packed RBCs to total blood volume) Low RBC count Reduces oxygen carrying capacity Leads to hypoxia/ischemia

Answer 166

You can have anemia if you have a profuse bleeding. But anemia doesn’t cause bleeding

Answer 167

the volume percentage of red blood cells in the blood. It can indicate if there is a problem, but cannot determine the underlying condition

Answer 168

Males they have more, females have less.

Answer 169

Blood Loss Decreased Production Hemolytic (increased destruction)

Answer 170

``` Weakness Fatigue Dyspnea on mild exertion Light-headedness, headache Pallor and/or jaundice Rapid pulse Skin atrophy; brittle, concave ﬁnger nails (koilonychia) ```

Answer 171

Microcytic and hypochromic cells

Answer 172

Absorption closely modulated by hepcidin Hepcidin inhibits iron absorption in enterocyte and iron release by macrophages In iron deﬁcient states, hepcidin levels are low and iron absorption is increased Absorbed proximal duodenum Absorption regulated by hepcidin made in liver and released in response to intrahepatic iron levels When body has enough iron, hepcidin inhibits absorption into blood by keeping it in mucosal cells (as mucosal ferritin) When hepcidin low, more iron absorbed

Answer 173

Iron deﬁciency microcytic anemia

Answer 174

``` Oral manifestations: “Burning” tongue Patchy or diﬀuse erythema Atrophy of ﬁliform papillae Taste alteration Fissuring Angular Cheilitis ```

Answer 175

Iron deﬁciency anemia caused by malnutrition, malabsoption Northern European women 30-50 yrs Esophageal web- Anemia, glossitis and dysphagia risk of squamous cell carcinoma (esophagus, oral, pharyngeal)

Answer 176

Lack of nutrients: B12 and folate Bone marrow issues: aplastic anemia, red cell aplasia, acute leukemia, metastatic tumors Erythropoietin deﬁciency: renal failure Chronic disease /inﬂammation /malignancy: decreased GI absorption of iron, reduced release from macrophages, relative low erythropoietin

Answer 177

Inherited: Sickle cell disease Thalassemia (specifically the major one because the other ones are not going to have the same manifestations) Hereditary Spherocytosis (which is a very rare inherited disease). Acquired: there are different things that can cause increased destruction: Malaria Parvo 19 infection HUS (hemolytic uremic syndrome - something related to kidney function) TTP thrombotic thrombocytopenic purpura DIC disseminated intravascular coagulation (this can happen with different conditions. This will not only cause problems with RBCs but the major issue is with the platelets. The crazy coagulation that can happen and it can effect the number of RBCs.) Drug Induced (you will see in Pharmacology different medications that can have this effect too.) And Autoimmune hemolytic anemia. Like that is related to how the immune system works.

Answer 178

Shortened erythrocyte life span < 100 days (normal lifespan is 120) Accumulate products of hemoglobin catabolism (iron) Marked increase in erythropoiesis Problems within erythrocyte (intracorpuscular /intrinsic) Problems outside erythrocyte (extra corpuscular /extrinsic)

Answer 179

This occurs in hemolytic anemia. You see irregularities Target cell – has something in the center People can call it in diff ways – some look like helmets = they call is schistocytes, fragmented red cells They call it in completely different names In reality, cells in different shape.

Answer 180

Clinical presentation depends on severity speed of onset underlying pathogenic mechanism Compensatory mechanisms increase in plasma volume increase in cardiac output increase in respiratory rate Hyperbilirubinemia, jaundice, and pigmented gallstones Inappropriately high levels of iron absorption from gut (iron overload) Childhood-Growth retardation, skeletal abnormalities

Answer 181

Fatty change Reversible accumulation of fatty vacuoles in cytoplasm in response to hypoxia (and to some other insults) Main organs aﬀected Liver, myocardium, kidneys

Answer 182

problems with vitamin B12 deficiency Macrocytic megaloblastic anemia Bigger cells – takes more volume in blood Lack intrinsic factor Autoimmune destruction of parietal cells in fundus Atrophic gastritis Defective DNA synthesis Common signs of anemia and neuro changes Symmetric paresthesia, Jaundice from hemolysis of RBCs, removal of abnormal RBC Demyelination of dorsal + lateral tracts in spinal cord Gives rise to sensory ataxia. Neuropsychiatric symptoms such as psychosis and dementia

Answer 183

Megalobastic = B12 and folate deficiency Non-megalobastic = alcohol reticulocytosis, liver disease, hypothyroidism

Answer 184

Necessary for DNA synthesis In acidic stomach, B12 binds to intrinsic factor(IF) stomach Vit B12 + IF / small bowel ,intestinal absorption in terminal ileum- transcobalamin Deﬁciency: decreased absorption or decreased intake for vegans

Answer 185

Malabsorption may be limited: Lack intrinsic factor so cannot absorb B12 from gut (pernicious anemia) Gastric bypass Malabsorption process in IBD aﬀecting terminal ileum (Crohn) or resulting from pancreatic insuﬃciency (pancreatic proteases usually help release bound B12) or from bacterial overgrowth (utilizes B12)

Answer 186

Pernicious Anemia

Answer 187

``` Burning sensation of tongue, lips, taste disturbances Patchy or diﬀuse erythema, or pallor Surface atrophy, lobulation Jaundice Skin may be yellow-gray ```

Answer 188

Important in DNA synthesis Megaloblastic anemia Correct macrocytosis with folate supplementation No neurological changes**

Answer 189

Inherited Microcytic anemia Abnormal production and increased RBC destruction

Answer 190

Alpha-thalassemias Hydrops fetalis (fatal before birth) - -/- - Hemoglobin H disease (moderately severe anemia): - -/-alpha Alpha thalassemia trait (similar to beta- thalassemia trait) - -/aa or -a/-a Silent carrier -a/aa

Answer 191

More in people with certain characteristics, living in certain parts of the world – Africa, Mediterranean, middle east and Asia – can be carriers for that or have more common disease Single mutation – carrier state This is the most common but asymptomatic Its only decreased on the alpha chain Remember, you have two alpha and two beta.

Answer 192

Its also a characteristic Mediterranean/middle eastern but not common in Africa Abnormalities are going to have different manifestation: Abnormal Hb: decreased function, altered erythrocyte plasticity, Increased erythrocyte destruction (hemolysis), Extramedullary hematopoiesis In the body, will have different manifestation Cooley’s anemia = is the one that has major severities related with that; near absent beta chain synthesis in homozygotes or compound heterozygotes

Answer 193

Frontal bossing, “hair-on-end” Crew cut appearance of calvaria Enlarged jaws ‘Honeycomb’ bone pattern The marrow is more active, it’s going to expand & its going to reduce the cortex.

Answer 194

Microcytic and hypochromic . Some of them have like a ‘tear drop’ shape but one of, they say, the characteristic is to have these “target cells"

Answer 195

Precipitated by hypoxia, infection, hypothermia, dehydration other 3-10 days Manifestations Capillary blockage: -- ischemia, infarction. and severe pain Hemolysis: anemia, jaundice +/- fever

Answer 196

Major effects on bones, lungs, liver, brain, spleen (due to vascular occlusion) Bone pain common in children Stroke Sequestration crisis – rapid accumulation of RBC in spleen Acute chest syndrome Fever, cough, chest pain, infiltrates and inflammation Kids feel like they're having a heart attack

Answer 197

``` Reduced trabeculation “Hair-on-end” appearance of calvaria Delayed dental eruption Dental hypoplasia Ischemic Neuropathy ```

Answer 198

Result from drugs lymphoproliferative disorders collagen vascular diseases malignancy idiopathic

Answer 199

RBCs fragment Occurs with HUS and TTP Eclampsia and pre-eclampsia with elevated liver enzymes and low platelets in HELLP HELLP: hemolysis, elevated LFTs and low platelets Seen with DIC, snake envenomation Exposure to ticlodipine and cyclosporine Mechanical heart valves

Answer 200

Carried on X chromosome Chronic or intermittent Function normally except under oxidative stress with certain meds or acute infection Present with anemia and jaundice Meds: quinine, sulfonamides, dapsone, primaquine, fava beans Bite cells on smear Conﬁrm diagnosis by checking G6PD levels 2-3 months auer hemolytic event Normal reticulocytes have this immediately following hemolysis

Answer 201

Looks like someone took a bite out of the RBC ("bite cells")

Answer 202

Decreased Production: (Pluripotent) Stem Cell Defect DECREASE ALL BLOOD CELLS - precursor cell failure Reticulocytes / hypo cellular marrow Features: anemia, bleeding disorders, susceptibility to infections <500 granulocytes/L (Normal = 3-8,000) <20,000 platelets/L (Normal = 150-450,000) <10,000 reticulocytes/L (Normal = 50,000) Microscopically, they’re not going to have cells,but you are going to see large spaces with fatty tissue.

Answer 203

Idiopathic Radiation Drugs: chloramphenicol, sulfonamides, alcohol, chemotherapy Infections: CMV/EBV, Parvovirus, hepatitis, HIV Bone marrow transplant in young, immunosuppress in older

Answer 204

pallor gingival bleeding, mucosal petechiae, purpura, ecchymoses gingival enlargement mucosal ulcers

Answer 205

APLASTIC ANEMIA

Answer 206

Normochromic, normocytic anemia Defective mobilization of iron to erythrocytes Low erythropoietin – common in renal disease Low serum iron, transferrin saturation Bone marrow not depleted Treat: iron and erythropoietin

Answer 207

Erythrocytosis (INCREASE IN RBCS) Primary: Clonal, autonomous proliferation of myeloid stem cells (polycythemia vera); often thrombocystosis, leukocytosis Secondary: increased erythropoietin levels Compensatory response to host/environment changes: lung disease, high-altitude living, cyanotic heart disease Response to erythropoietin-secreting tumors (RCC renal cell carcinoma, hepatoma) Use of erythropoietin

Answer 208

Elderly with anemia +/or thrombocytopenia +/ or leukopenia w hyposegmented PMN Macrocytic anemia with normal B12 and folate Bone marrow: increased blasts, dysplastic precursors Primary or secondary due to chemo Common cause of death is due to low WBCs: infection

Answer 209

varies from 4400 to 11,000 cells/microL (4.4 to 11.0 x 109/L) (60% are mature neutrophils)

Answer 210

absolute neutrophil count (ANC) <1500 implication of neutropenia varies between, mild say if you have WBC between 1000 and 1500 or very severe actually people that have neutrophil count less than 200, they can just die because of an infection.

Answer 211

Infections: HIV, CMV, EBV etc Medications: Harm WBC or cause BM suppression Alcohol, B12 or folate deﬁciencies Aplastic Anemia (Hypocellular marrow) Lymphadenitis: Inﬂammation in LNs and can be associated with lympadenopathy

Answer 212

psychiatric medication. They all cause bone marrow suppression

Answer 213

``` Acute Myeloid Leukemia (AML) Myeloproliferative Neoplasms (MPN) -Chronic Myeloid Leukemia (CML) -Polcythemia Vera (PCV) -Essential Thrmobocytosis (ET) -Primary Myeloﬁbrois (PMF) ```

Answer 214

the most common acute leukemia in adults and accounts for approximately 80% of cases in this group Because the more we live, the more we acquired in our life, some genetic abnormalities. And that's what leads for the body to misbehave and you can get AML. So the median age of diagnosis is 65. However, unfortunately recently we're seeing younger and younger patients. The incident increases with age, as I mentioned. Even with the best treatment availability, there's still a higher risk of relapse, and mortality. And the five-year survival is not great at all only 24%, and because most of the patients relapse there's a mis-behave in the genetic in our body that pathways almost turned on. So basically, yourself are always trying to produce more and more WBC, because you cannot cope, the bone marrow cannot cope to produce all cells including

Answer 215

``` Chemical exposure Benzene, Pesticides Other environmental exposures Hair dyes, smoking, non-ionic radiation Genetic disorders Down syndrome, Bloom Syndrome, Fanconi’s anemia, Ataxia-Telangectasia, Wiskott-Aldrich Prior chemotherapy or XRT Alkylating agents, Topoisomerase II inhibitors ```

Answer 216

``` Alkylating agents -Preceding MDS phase -Evolution to AML 5-7 yrs -Abnormalities: -5 or -7 Topoisomerase II inhibitors -No MDS phase -Monocytic morphology -11q23 ```

Answer 217

We do bone marrow biopsy and find they have 20% leukemic cells. Defined as myoblast. that they are precursors are early cells, like stem cells, they all expressing CD34 positive and HLA-DR. So when they express these two markers, they are very early cells, like stem cells and leukemic cells.

Answer 218

``` subset of AML cure rate is 98% 10-15% of AML (FAB M3) Higher incidence in Hispanics (20-25%) and with increase BMI Median Age at Dx is 40yrs Coagulopathy and hemorrhage CD 34 and HLA-DR negative t(15,17) associated with PML/RAR-α fusion ```

Answer 219

AML, you are stuck in myeloblast, in APL you are stuck the step afterword, which is promyelocyte, So what happened is that you just matured cells by giving them a medication, a pill. And then the cells start maturing. And you do very well, 98% cure rate

Answer 220

Associated with the fusion of 2 genes: BCR (on chromosome 22) and ABL1 (on chromosome 9) resulting in the BCR-ABL1 fusion gene (PHILADELPHIA CHROMOSOME) 15 to 20% of leukemias in adults The only risk factor that we know of CMS is radiation, people that get exposed to radiation, working in radiation, or even people that get been exposed to high radiation scans or something like that frequently not just once.

Answer 221

1) a chronic phase, 85% of patients at diagnosis 2) an accelerated phase, in which neutrophil diﬀerentiation becomes progressively impaired and leukocyte counts are more diﬃcult to control with treatment 3) blast crisis, a condition resembling acute leukemia

Answer 222

collectively characterized by clonal proliferation of myeloid cells with variable morphologic maturity and hematopoietic eﬃciency. They are characterized of splenomegaly and JAK2 mutations 2-Polycthehemia Vera (PCV) PCV is distinguished clinically from the other MPNs by the presence of an elevated red blood cell mass However, an increased red blood cell mass alone is insuﬃcient to establish the diagnosis, since this is also observed in conditions associated with chronic hypoxia and with erythropoietin-secreting tumors 3-Essential Thrmobosis (ET) ET has also been called also primary thrombocytosis. It is characterized by excessive, clonal platelet production with a tendency for thrombosis and hemorrhage. 4-Primary Myeloﬁbrosis (PMF) PMF is the least frequent among the chronic myeloproliferative diseases Burned out marrow

Answer 223

JAK mutation will lead to: 1, proliferation, so you have more production of cells to certain pathways ; 2, angiogenesis you have more vessels to supply the organs so they can grow and produce more, 3 you have more immunosuppression. the body can be prone to infection, can actually allow cells to grow more and more.

Answer 224

``` Acute Lymphoblastic Leukemia (ALL) Chronic Lymphocytic Leukemia (CLL) Non Hodgkin’s Lymphomas (NHL) Hodgkin’s Lymphomas (HL) NK/T cell lymphomas ```

Answer 225

more frequently presents in its leukemic form than its lymphomatous form B-ALL accounts for approximately 2% of the lymphoid neoplasms diagnosed in the US Previous chemotherapy and exposure to radiation increase the risk of developing ALL Signs and symptoms include fever, feeling tired, and easy bruising or bleeding Txt: complex 3 year chemotherapy treatment

Answer 226

The most common one, on the rise. We'll talk about it in a bit is CLL. This is hundreds per thousand patient. The next is, AML, and then come CML and ALL. If you notice that CLL is significantly under rise. AML actually on the rise and the CML as well, but look at ALL. If anything, you are higher risk for have a younger age and then it goes down and the plateaus doesn't really increase the risk

Answer 227

The younger you are, the better you do so. If you are someone that have an ALL at 15 and 19, that cure rate is 70% now was newer drugs, probably it is around 80%. But if you are 66 year old and you get to leukemic the survival is 30%, because the drugs the chemotherapy that you really need for cure, the body cannot tolerate them. They are meant for young, and healthy pediatric people. So the older the patient gets, it's very hard to cure.

Answer 228

The most important thing we look at is cytogenetics. Philadelphia chromosome: it’s seen in CML and if it’s seen in ALL, it results in a worse outcome. If someone has Philadelphia chromosome, then you’re going to treat them with chemotherapy and again you’re going to give them the pill that you gave patients with CML. The outcome is worse in this case Age: the younger the better. We always try to give them a pediatric inspired regimen because they do much better. If they have Philadelphia chromosome mutation or they are old, you’re always going to try to replace their stem cells and that’s what we call high risk. If they are a candidate for stem cell replacement, this is the only cure in the elderly or as they relapse we may not be able to cure them.

Answer 229

A subtype of leukemia arising from immunologically less mature lymphocytes that spread to the blood, bone marrow and lymphatic tissues The longer people live, the greater the risk of CLL Staging is different. It’s usually based on if you have WBC, if you have the spleen involved, if you have the liver involved, if you have the bone marrow involved or not. The most important key in CLL and that’s why it’s on the rise is the microenvironment. Risk Factors: Family history Age and Gender Race/Ethnicity: more common in people of Russian and European descent, and hardly ever develops in people from China, Japan, or Southeast Asian countries. It also occurs commonly in black people. Agent Orange Prognosis: Majority indolent disease associated with a prolonged (10 to 20 years) clinical course (about 40-50%)

Answer 230

Non Hodgkin’s Lymphoma (NHL) Hogkin’s Lymphoma (HL) NK/ T cells Disorders They start taking over the lymph node. The lymph node (or any of these organs) can no longer fit them as they start growing. This is what happens in lymphoma. It presents with large lymph nodes, large spleen, large thymus gland and you have symptoms because of this. Most of the time the symptom is PAIN. Or you can end up (because there’s so many lymph nodes), you can end up with B symptoms which is fevers, night sweats, and weight loss.

Answer 231

DL-BCL (diffuse large B cell lymphoma) -- it accounts for 70% of lymphomas. Usually the problem happens in the center of the lymph nodes If the problem happens outside the lymph nodes, it may happen in plasma cells. Plasma cells are part of lymphocytes and then you end up having a different disease called multiple Myelenoma. Non-Hodgkin lymphoma usually the problem happens inside the germinal center inside the lymph node. The Hodgkin lymphoma is usually outside the germinal center and that’s because the cells mature a little bit more and that’s why you end up with Hodgkin lymphoma.

Answer 232

Small cells or large cells This is most of the time when they are small they are INDOLENT lymphoma – slow growing. When they become large cells, the lymph nodes are larger. The lymphocytes are large as a result. They become more aggressive. This is an example of Non-Hodgkin lymphomas. Some people when they take a look at this slide (C)  highly aggressive type of lymphoma called Burkit Lymphoma that‘s associated with EBV virus and HIV. Multiple types of lymphomas that range from indolent to aggressive form.

Answer 233

Note there’s only really four types of Hodgkin lymphoma and most of the time you can cure it. Only accounts for 10% of lymphomas. About 80% cure rate Cell presentation: OWL. Looks like an owl, it’s called Reed Sternberg cells. Very characteristic presentation. Two cells that are close to each other, usually they have a large cytoplasm and nucleus. That’s what you see in Hodgkin lymphoma.

Answer 234

This is when you end up having a disease called multiple myeloma (MM) It’s the second most hematologic malignancy. The most common hematologic malignancy = Non-Hodgkin’s lymphoma. The most common Hodgkin lymphoma  70% of cases diffused largely B cell lymphoma. It accounts for about 20% of deaths from hematological malignancies and 2% of deaths from cancers. In 2012, there was about 90.000 patients living with myeloma and every year we’re estimating about 50,000 US people will end up with MM. Approximately, the risk of having multiple myeloma is 0.7% in men and women. If you have about 150 people, 1 of them will have MM. Medium age – it’s a disease of the elderly, age 65. The 5 year survival as of 2018 is only 50%. Unfortunately, we lose 50% of the patients so we're still not doing that good. Dong better than AML, but How do people present with MM? What they present with is the CRAB. This stands for Hypercalcemia, Rena, Anemia, and Bone lesions. They have HIGH calcium (note).

Answer 235

Induction HDT/ASCT Consolidation Maintenance

Answer 236

key of multiple myeloma is lytic bone lesions

Answer 237

Using your own WBC from your immunity to kill your defected WBCs

Answer 238

Need to treat underlying etiology and use prophylactic antibodies, anti fungal and anti viral

Answer 239

Monoclonals + cytotoxic agents-- inotuzumab Biallelic monoclonal (CD3 + CD19)-- blinatumomab Modiﬁed expanded Tcells-- CART cells

Answer 240

Nasal clearance: sneeze, blow or swallow Tracheobronchial clearance: 3 to 10 mm mucociliary action, IgA Alveolar clearance: 1 to 5 mm macrophages, IgM, IgG, C3b, T cells

Answer 241

Virus --> Infection of type I pnuemocytes --> alveolar injury --> interstitial pneumonia Pyogenic bacterium --> acute inflammatory response to bacterium --> intra-alveolar pneumonia

Answer 242

Community acquired Nosocomial Opportunistic Most bacteria causing pneumonia are oro- and nasopharyngeal flora

Answer 243

Staphlococci (gram +): what you find on your skin Strep (gram +): is so commonly associated with pneumonia that theres a pneumococcal streptococci (pneumo meaning air/lungs) Haemophilus (gram -) : children Psudomonas (gram -): cystic fibrosis Klebsiella (gram -): alcoholics Legionella: transplant patients

Answer 244

``` Streptococcus pneumoniae (pneumococcus) Gram + facultative anaerobic cocci in pairs ``` Found in Nasopharyngeal flora in up to 20% Capsular polysaccharides--antigenically specific--84 types Types 1,2,3,4 = pathogenic Extracellular pathogen Capsule: antiphagocytic property Pneumolysin (is a chemical that allows it to break open pneumocytes) Neuroaminidase Most common cause of community-acquired pneumonia Frequently follows a viral URI Bronchial secretions provide environment Vaccines for young/old offer up to 90% protection

Answer 245

Symptoms: Fever, malaise, productive cough and +/- chest pain Signs: Tachypnea, decreased breath sounds, dullness to percussion Radiology: Infiltrate(s) Treatment: Afebrile within 48 to 72 hours after antibiotics < 10% of pneumonia admissions die!!

Answer 246

1. Congestion: bacteria with edema and vascular engorgement 2. Red hepatization: red cells, neutrophils and fibrin 3. White hepatization: fibrin, fibroblasts with few neutrophils 4. Resolution: return to normal

Answer 247

Abscess (lung tissue can die. It can be necrotic) Empyema (pus in the pleura) Organization (consolidation of lung/ it is permanently in that white hepatization with scarring and fibrosis) Bacteremic dissemination…meningitis, endocarditis Bronchiectasis (end up with a chronic lung infection that scars the lung and dilates the airways )

Answer 248

Abscess formation: local necrosis of the lung Following pneumonia S. aureus, K. pneumoniae, pneumococcus type 3 fungi Aspiration of infective material Mixed organisms including oral anaerobic flora Bacteroides, Fusobacterium, Peptococcus Septic emboli deep leg veins or right heart valves Neoplasia--10 to 15% of cases post-obstructive pneumo

Answer 249

Chronic necrotizing infection with dilation of airways Bronchial obstruction Tumor, foreign body, mucus impaction (COPD) Congenital or hereditary conditions Cystic fibrosis, Kartagener’s syndrome Necrotizing pneumonia M. Tuberculosis and Staphlococcus infections

Answer 250

``` Lower respiratory tract only Adenovirus influenza A and B Respiratory syncytial virus Corona Parainfluennza ``` ``` Systemic with pulmonary involvement Measles Herpes 1 and 2 Varicella-zoster Cytomegalovirus Ebola ```

Answer 251

Candidiasis--normal oral flora Candida albicans usually aspiration or hematogenous spread Histoplasmosis--Ohio and Mississippi rivers Histoplasma capsulatum Self-limited, chronic and disseminated forms Coccidiomycosis--Southwest and Far West Coccidioides immitis All inhalers become infected 10% have fever, cough and chest pain + skin lesions San Joaquin Valley fever complex ``` Pneumocystis jiroveci (carinii) A true fungus; ubiquitous Does not cause disease in immunocompetent folks Inhaled fungi attach to type I pneumocytes HIV+ with <200 T cells at risk! ```

Answer 252

Mycobacteria grow slowly and drug sensitivities can take 6 weeks Mycobacteria are aerobic non-spore forming nonmotile bacilli with a waxy coat M. tuberculosis and M. bovis cause tuberculosis M. avium and M. intracellulare afflict immunocompromised folks M. leprae causes leprosy M. tuberculosis infects 33% of the world’s population M. tuberculosis kills 3 million patients yearly Single most important infectious cause of death

Answer 253

Pathogenicity: ability to escape initial macrophage and T cell attacks Cord factor: grow in cords Lipoarabinomannan (LAM): g- endotoxin-like substance Inhibits macrophage activation by INF-gamma Induces macrophage TNF-alpha secretion fever, weight loss, tissue damage Induces macrophage IL-10 secretion suppresses T-cell proliferation CR3 uptake receptor faciliates macrophage uptake without respiratory burst Heat-shock protein All leading to granulomatous inflammation and destruction by self! (granuloma formation)

Answer 254

Healed lesions: scars without organisms Latent lesions: dormant organisms Progressive primary Tb: massive lung spread Miliary Tb: massive hematogenous spread

Answer 255

lesion seen in the lung in primary tuberculosis. The lesions consist of a calcified focus of infection and an associated lymph node.

Answer 256

parenchymal subpleural lesion around upper/lower lobe fissure enlarged caseous lymph nodes draining lung lesion

Answer 257

dormant organisms disseminate host weakens…cancer, infection reactivation of infection Reactivation in 5 to 10% of cases Lung apices where there is high O2 tension Possible outcomes scar with or without therapy localized cavitation...bronchial...laryngeal…intestinal spread bronchopneumonia “galloping consumption” lymphangitic spread to other areas of lung or other organs vascular spread

Answer 258

Lung cancer behind heart disease (#1) and 3rd is lower respiratory disease (also smoking related).

Answer 259

Lung cancer

Answer 260

tobacco – 90% of lung cancers Environment Infections – NO!!!!!!!!!!!!!! Diet – (if you eat well it probably indicates a confounding variable that you’re of a higher SES so you’re less likely to smoke) Genes - play a HUGE role but it’s often hard to tease out as we’ll see because smokers usually grow up in households where the parents smoked

Answer 261

Tobacco smoke causes 90% of lung cancer 1.9% of men, 13.0% of women non-smokers!! Duration and intensity of smoking correlate with incidence and mortality Low tar-low nicotine cigarettes--cotinine levels!! Involuntary or passive smoking is a proven cause of lung cancer

Answer 262

``` Oropharynx Larynx Esophagus Trachea Bronchus Lung Leukemia Stomach pancreas Kidney Ureter Cervix Bladder ```

Answer 263

20 minutes later your BP will drop, your CO levels will return to normal, things will taste better, your lung function will improve, your risk of heart disease will drop in a very short time, your risk of stroke will drop in a short time. Your risk of dying from lung cancer over about 20 years it will take to come back to the risk of almost a non smoker – it never comes down but close

Answer 264

Synergistic-multiplicative effect with tobacco smoke

Answer 265

Asbestos Indestructible and fire resistant fibers Amosite, tremolite, chrysotile, crocidolite Incite inflammation, fibrosis, malignancies ``` Nickel compounds Copper compounds Arsenic compounds Chromate compounds Mustard gas Benzene ```

Answer 266

Radioactivity in cigarette smoke Alpha emitters polonium-210 and lead-210 Particles accumulate at bifurcations of segmental bronchi 1.5 packs per day = 300 CXR per year to the skin Radon...gaseous product of radium-226 decay Half life 3.8 days; decays into 2 alpha emitting daughters Particles deposit on dust inhaled into the bronchial tree Beware of well insulated airtight homes in the Reading Prong Synergism between decay products and tobacco smoke 2nd cause of lung cancer in US according to EPA 20,000 deaths per year Uranium Navajo uranium miners

Answer 267

EGFR mutations most commonly found in women (non-smokers with lung cancer)

Answer 268

Peak age 60-70 years, range 11-whenever ``` Slight male predominance Symptoms: asymptomatic cough or change in existing cough weight loss chest pain dyspnea wheezing/stridor/hoarseness sputum production/hemoptysis SVC syndrome Pancoast syndrome Paraneoplastic syndromes metastatic disease ```

Answer 269

Symptom complexes not explained by tumor or hormones produced by organ involved by tumor. Occurs in 1-10% of lung cancer patients ``` Hypercalcemia Hypocalcemia Gynecomastia Carcinoid syndrome Cushing syndrome Eaton-Lambert myasthenic syndrome Syndrome of Inappropriate ADH secretion (SIADH) ```

Answer 270

Cytology sputum bronchial wash/brush fine needle aspiration Transbronchial biopsy Mediastinoscopy or scalene lymph node biopsy Surgical resection wedge biopsy lobectomy pneumonectomy

Answer 271

Small cell carcinoma (SCLC) 25% Non-small cell carcinoma (NSCLC) [including rare salivary gland-types] 75%

Answer 272

``` HORROR OF HORRORS Smoking-related Widespread at the time of diagnosis Paraneoplastic syndromes Initially responsive to chemotherapy Anatomic extent of limited disease can be included within an irradiation field (limited to area 30%) There are almost NO survivors ``` Present with systemic complaints...H/A, fatigue... Up to 85% have extrathoracic disease at diagnosis Present with paraneoplastic syndromes SIADH or Cushing syndrome 5 year survival = 4% Gross features often virtually undetectable bronchial wall lesions Microscopic features small cells with little cytoplasm dark nuclei without nucleoli mitotically active

Answer 273

Non-small cell carcinoma been the most common type for years Most common type found in men Correlates with smoking Usually central/hilar location = 15% 5 year survival Microscopic features squamous metaplasia keratinization intercellular bridges leading to dysplasia and then carcinoma

Answer 274

Non-small cell carcinoma arising from a glandular cell & NOT from a squamous cell.** And, it is most common type in women & non-smokers. Usually peripheral location 5-25% 5 year survival associated with scars Gross features can be very very small, but still very very bad! ``` Microscopic features acinar papillary micropapillary solid lepidic ```

Answer 275

T1: <= 3 cm not within the main bronchus T2: > 3 cm but <= 5 cm Involves the main bronchus but not carina Invades visceral pleura Any tumor causing atelectasis or pneumonia to hilum T3: > 5 cm but <= 7 cm Any tumor extending into chest wall, diaphragm, pericardium Any tumor in the main bronchus within 2.0 cm of the carina Separate cancer in the same lobe! T4: > 7 cm Any tumor invading mediastinum, heart, esophagus... Any tumor involving the carina Separate cancer in same side lung but different lobe

Answer 276

N0: No nodes N1: Peribronchial or ipsilateral hilar nodes N2: Ipsilateral mediastinal nodes or subcarinal nodes N3: Contralateral hilar or mediastinal nodes; any neck nodes

Answer 277

M0: No known metastases M1: Separate carcinoma in other lung Pleural nodules Malignant pleural effusion Distant metastasis ``` Regional lymph nodes (>50%)*** Adrenal gland (>50%) Liver (30-50%) Brain (20%) Bone (20% ```

Answer 278

carcinomas, sarcomas metastasis | NOT LUNG CANCER

Exam 1 Flashcards

(302 cards)