Metastatic calcification

Calcium infiltration into normal tissue

(ex: Hypercitaminosis D.)

Dystrophic calcification 

Calcium infiltration into Abnormal/damaged tissue

(ex: atherosclerosis or atheroma. Damage to tunica intima)

 calcium

most abundant protein in the body

 Actin

 Caseous degeneration

 Tuberculosis

 Cloudy swelling degeneration

 Albuminous

(Membrane injured which effects ionic transfer)

 Infarction

(MI)

 Liver aka "nutmeg liver"

Heart  ="tabby cat heart"

Fatty dot -> fatty streak ->atheroma (fatty plaque)-> fibroatheroma(Fibrous tissue,calcium)

CNS - (ex: brain melts, tertiary syphilis, leprosy)

OR

SKIN - (ex: if from infection)

 Wallerian degeneration

 Peripheral nerves

(Dengenerates to the next NODE OF RANVIER. "dying back phenomenon")

Zenker's (Waxy) degeneration

Hyaline

Most common in skeletal muscle. Muscle replaced with hyaline. (ex: myositis ossificans)

 Small remnants of the organ present

(lack of development - not functioning properly)

 Organ smaller than normal

(fully functioning)

 agenesis 

aplasia

hypoplasia

atrophy

hypertrophy

hyperplasia

metaplasia

dysplasia

anaplasia

Atrophy

Decrease in size (disuse atrophy)

(seen most commonly in muscle)

Hypertrophy

Increase in size 

(ex: muscle exercise)

Hyperplasia

Increase in number of cells

(ex: pregnant uterus, goiter)

goiter

increase in thyroid cells (hyperplasia) in response to lack of iodine

Metaplasia

Changes from one cell type to another.

(ex: pseudostratified ciliated columnar epithelium(PCCE) changing into stratified squamous in lungs of a smoker -defense mechanism)

Dysplasia

Change in size, shape or funciton of a cell.

(Precancerous, but last stage that can reverse to normal)

Analplasia

Complete disorganization of a cell (cancer)

(ex: squamous cell carcinoma)

Collagen types

Type collagen 1

Type collagen 2

Type collagen 3

Type collagen 4

Type Collagen 1

Bone, muscle, tendons, and ligaments

(annular fibers)

Type Collagen 2

Disc (specifically nucleus pulposis)

Type Collagen 3

"reticular collagen"

found in spleen and lymph nodes also seen in EARLY WOUND HEALING

Type 4 Collagen

Basement membrane

Pneumoconoisis

Inhaltion & deposition of dust particles in the lung

Pneumoconoisis has 8 possible resultants

1. Silicosis
2. Abestosis
3. Siderosis
4. Byssinosis
5. Anthracosis
6. Histomycosis/Histoplasmosis
7. Coccidiomycosis
8. Blastomycosis

Silicosis

Silica from sand, rock, or glass.

"sandblaster's risk"

"Rocky Quarry disease"

Asbestosis

Asbestos leads to Mesothelioma (malignant tumor)

(Mesothelial cells are in pleura of lungs)

Siderosis

Iron Dust 

(smelting/smoldering smoke stack from procesing plants)

Byssinosis

Cotton Dust

"Brown Lung"

Anthracosis

Black Lung Disease from miners.

("Caplan's syndrome" = anthracosis + RA)

Histomycosis / Histoplasmosis

Endemic fungus around Mississippi or Ohio valleys.

"Ohio Valley Fever"

aka "Mississippi Valley Fever" 

(calcification of lungs similiar to Tb)

Coccidiomycosis

Endemic fungus in deserts of Southwest 

(aka "San Joaquin Valley fever"

called "Valley Fever' in other states)

Blastomycosis

Endemic fungus on Eastern seaboard. 

From Florida to Nova Scotia.

Lung Diseases

1. Asthma
2. cystic Fibrosis
3. Hemosiderosis
4. Pulmonary Fibrosis
5. Tuberculosis
6. Wegener's Granulomatosis
7. Emphysema(COPD)

Asthma

*Sputum analysis* yields "Curschmann's Spirals" or "Charcot Leyden crystals"

Cystic Fibrosis

Affects chloride channels. "Sweat test."

Mother notices 1st...tastes salt -> "Salty baby syndrome"

Affects GI and Respiratory mucous linings and exocrine secretions. 

Susceptible to Pseudomonas aeruginosa. Lung transplants frequently required. 

Hemosiderosis

Iron in lungs due to bleeding

Pulmonary Fibrosis

Honeycomb Lung

Tuberculosis

*Caseous Necrosis in Lungs*.  

Gohn complex/lesions. Granulomas. Epithelioid Histiocytes (inclusion bodies).

If spread to spine = "Pott's Disease". 

If vertebra collapses from TB ="Gibbus deformity"

Wegener's granulomatosis

Vasculitis of the lung and kidney

Emphysema

Alpha-1-antitrypsin deficiency 

Break down of Lung tissue

(protease/antiprotease theory is associated with this)

Hemochromatosis

Iron accumulation in organ resulting in skin bronzing

(ex: bronze diabetes)

Renal diseases

1. Azotemia
2. Hydronephrosis
3. Kidney stones
4. Nephritic syndrome
5. Nephrotic Syndrome
6. Polycystic Kidney
7. Shock
8. Wilm's

Azotemia

Decrease tubular excretion of nitrogen

(↑ nitrogen in the blood = ↑BUN)

kidneys can't excrete

Hydronephrosis

Most commonly caused by prostate problems or ureter stones. 

(kidney failure)

Kidney Stones

"Nephrolithiasis". 

Most common  is calcium oxalates. Kidney stone pain pattern = starts in back and radiates to the flanks.

Once stone is in ureters, pain localizes in groin.

Nephritic Syndrome

RBCs & small amounts of protein in the urine.

(ex: acute glomerulonephrits)

Nephrotic syndrome

"pre-ecclampsia"="HEP" Hypertension, Edema, Proteinurea(massive)

(Ecclampsia = HEP + convulsions + coma = potentially fatal)

Polycystic Kidney

Moth eaten appearance

Shock

Acute tubular necrosis

Wilm's tumor

"Nephroblastoma". Mixed tumor of kidney in children (malignant)

Thyroid & Adrenal diseases 

1. Addison's
2. Conn's 
3. Cushing's 
4. Goiter 
5. Graves 
6. Hashimotos
7. Myxedema

↓ Cortisol. Hypoadrenia.

low blood pressure. Hyperpigmentation due to excess 

ACTH → ↑MSH(melanocyte stimulating hormone). fatigue

Conn's disease

Excess Aldosterone (Zona Glomerulosa).

hypertension, water retention

Excess Cortisol (Zona Fasciculata).Hyperadrenia.

Moon faces, buffalo hump, purple striae, central obesity

Hypothyroidism, Cretinism in the child.

cold intolerance, weight gain, slower heart rate, loss of lateral 1/3 of eyebrows, slow mental faculties (ex:memory, common sense)

1. Cervical cancer
2. Cryptorchidism 
3. Endometriosis
4. Epispadias
5. Hypospadias 
6. Increases HcG
7. Leiomyoma
8. Polycystic ovary disease
9. Seminoma

Cervical Cancer

Can cause testicular cancer

Failure descent of the testis 

Urethra exits  on the dorsal surface of the penis

(epi = dorsal)

Urethra opens on the ventral surface of penis

(Hypo = ventral)

Tumor of smooth muscle. Fibroids in the uterus.

Seen in pelvic inlet as uterine fibroid.

Congenital (5 signs)

 or

Acquired "3 stages" - organism= Treponema pallidum

Congenital Syphilis

(5 signs)

1. Hutchinson's teeth - Notched permanent upper incisors
2. Rhagades - fissures or cracks at edge of mouth
3. Saddle nose deformity - bridge of nose flattened
4. Sabre Blade Tibia
5. Interstitial keratitis - produces visual changes (blindess)

Deafness can also be associated with congenital syhilis, but not always 

Acquired Syphilis

"3 stages"

Primary: hard singular painless nodule (chancre) on perineum

Secondary: maculopapular rash and condylomata lata (flat worms on the vulva)

Latency: may or may not have symptoms

Tertiary: Tables Dorsalis (slap foot gait), aortic aneurysm, Gummas inclusion body in CNS(aoric valve + arch). Argyll Roberston pupil aka "Prostitute's Pupil". Not react to light, but constricts on accommodation. 

Note on pupils

Adie's pupil

Miosis

Adie's pupil : tonic pupil dialation (Mydriasis) due to injury to CN III Edinger Westphal nucleus (preganglionic parasympathetic neurons)

Miosis: Tonic pupil constriciton due to injury to sympathetic cervical ganglia

"My" --> dialation  "Mi"--> constricted

Chancroid

gardnerella vaginalis 

gonorrhea

lymphogranuloma venereum 

trichimonas

chlamydia

Chancroid

Similar to Trichimonas, except bacterial, 

Green, frothy, fishy, foul discharge

Gram(-) Diplococcusm, most common cause of Pelvic inflammatory disease(PID), Salpingitis.

Burning urination. Pus (yellow/green) in urine. May produce arthrisis most common -> DJD in knee.

"coffee bean shaped" organism on microscopy

"Gonorrhea knee"

Lymphogranuloma Venereum

(3 things)

1. Chalamydia = organism
2. produces rectal strictures
3. Diagnose with Frei test

Purulent vaginal discharge

Green and Frothy, Fishy, foul 

PROTOZOAN

Causes white pus.

#2 most common cause of PID

Implicated by reitter's syndrome

1. Achalasia
2. Barrett's esophagus
3. Budd-Chiari
4. Celiac Disease 
5. Cholecystitis
6. Cholelithiasis
7. Crohn's
8. Diverticula
9. Dysphagia
10. Enteritis 
11. Hemorrhoids
12. Hirschbrung's
13. Intussusception
14. Irritable Bowel
15. Mallory-Weiss Syndrome
16. Meckel's diverticulum
17. peutzjegher's 
18. Plummer Vinson syndrome
19. Pyloric Stenosis
20. Sliding hiatal hernia
21. Steatosis
22. Ulcerative Colitis
23. Volvulus
24. zenker's Diverticulum 

Spasm of lower esophageal sphincter. Causes megaesophagus  of upper esophagus.

Lack of motility. (due to absence of myenteric plexus (degeneration))

Occlusion of Hepatic Veins

Triad of : Abdominal pain, ascities, and hepatomegaly

Acute: rapid severe upper abdominal pain, jaundice, hepatomegaly, ↑ liver enzymes and eventually encephalopathy... esophageal varicies 

Celiac Disase

aka "non-tropical sprue"

Intolerance to gluten. Loss of villi, therefore loss of absorption.

Stay on gluten-free diet (Gluten enteropathy)

Inflammation of gallbladder.

#1 cause of Gallstones

Both small and large intestine

seen in young people, affects distal small intestine (ileum), cecum and ascending colon. Right sided problem.

Skip lesions or "cobblestone" appearance. No Bloddy diarrhea.

Autoimmune. Aka's: Regional ileitis, dital ileitis, terminal ileitis regional enteritis.

Leads to dehydration b/c most water reabsorbed in small intestines.

Outpouching in the intestine.

Usually lower left quadrant (sigmoid and descending)

CONGENITAL MEGACOLON

Absence of myenteric plexus =parasympathetic motor plexus

(aside: Meissner's plexus detects chemicals. It is a chemosensitive plexus)

Telescoping  of intestine on itself, can be caused by

rotovirus vaccine.

Irritbale bowel syndrome

IBS

Hematemesis (vomiting of blood) due to alcoholism.

lacerations of esophagus and proximal stomach

Outpouching of Distal Ileum

(congenital)

Polyposis characterized by polyps in the entire GI tract

"Multiple polyps"

Ulcerative colitis

aka TOXIC MEGACOLON

Pathological LARGE intestinal changes, ulcers, bloody diarrhea affects LEFT SIDE of abdomen (descending colon, sigmoid colon)

LEAD PIPE rigidity, starts at the colon and moves distally

Outpouching of the Pharynx

(congenital)

1. Diabetes Insipidus
2. Diabetes Mellitus
3. Hemochromatosis
4. Kwashiorkor
5. Marasmus
6. Wilson's

Decreased (lack) of ADH(vasopressin) due to posterior pituitary problem

Causes  dehydration

Decreased production of insulin by the beta cells of pancreas (Islets of Langerhans)

1st seen in the eyes

most commonly die from heart disease or renal failure.

produces Polydypsia (↑thirst), Polyuria(↑urination), and Polyphagia(↑hunger)

Iron in organs and skin

Bronze Diabetes

Protein malnutrition

(African children w/ big bellies → abdominal ascites)

caloire malnutrition, includes protein

(bone skinny... nothing but skin and bones appearrance)

Inborn error of copper metabolism. 

Kayser Fleisher Rings =copper deposits in eye

Hepatolenticular degeneration

1. ALS
2. Alzheimer's
3. Arnold Chiari
4. Brain Tumors
5. Friedreich's ataxia
6. Guillain-Barre
7. Horner's Syndrome
8. Leprosy
9. Multiple Sclerosis
10. Myasthenia Gravis
11. Neurofibromatosis
12. Onion bulb tumor
13. Parkinson's
14. PLS
15. Poliomyelitis
16. Syringomyelia
17. Wernicke-Korsakoff

ALS

Amyotrophic Lateral Sclerosis

Lou Gherig's disease

Anterior horn and corticospinal tracts affected with no sensory loss.

Motor ONLY. LMN in upper extremities, UMN in lower.

it is progressive - never get better from the first day of diagnosis

Can Perceive but can't respond

Men over 50 , die from respiratory paralysis

#1 cause of Dementia

Progressive severe  atrophy of the cerebral cortex,

Neurofibrillary tangles, decrease in Acetyl-choline.

Type 1: cerebellar tonsils herniate (specifically the vermis)

Type 2: cerebellar tonsil herniation with meningiomyelocele in lumbar spine (more advanced)

(Note: can get syrinx b/c of hydrocephalus w/ arnold chiari)

BRAIN TUMORS

(6)

1. Astrocytoma
2. Glioma's
3. Gliobastoma Multiform
4. Medulloblastoma
5. Oligodendroglioma
6. Schwannoma

Type I and II = most common

best prognosis out of all brain tumors

In CNS

golf ball size, well capsulated

Most common brain tumors in CNS

It's the "umbrella" tumor.. all others underneath it

WORST prognosis out of all the brain tumors

affects cerebrum

Most commonly seen in cerebellar vermis in children.

Most common primary cerebellar tumor

slowest growing 

affects cerebrum

CN VIII -(vestibulocochlear) = ACOUSTIC NERUOMA

ONION BULB TUMOR

most common cause of unilateral sensorineural hearing loss

Lesions of sclerosis of the Spinocerebellar tract(neurofibrillay tangles.)

chromosome 9

Aka Post-infectious polyradicuopathy, most commonly seen after a recent flu or vaccination.

Peripheral Demyelinating Disease.

Areflexia and ascedning paralysis.

fatal if hits diaphragm

Interruption of cervical sympathetics.

(ex: trauma whiplash, pancoast tumor)

Symptoms = ptosis, miosis, anhydrosis, enophthalmosis

aka Hansen's disease

lequefactive necrosis. 

Skin Damage  and destruction of senroy nerves

Multiple Sclerosis

"it's a SIN to have MS"

Central Demyelinization and plaquing of the spinal cord or brain. 

Charcot's triad (SIN): Scanning speech, Intention tremor and Nystagmus. 

• characterized by exacerbations and remissions.
• effects distal muscles and causes visual disturbances
• affects Corticospinal Tracts (hyperreflexia)

Formation of antibodies that block the Ach receptors at the myoneural junciton. 

Thymic enlargement (thymoma).Causes progressive weakness and fatigue,first starts in the ocular mm's, gets worse at end of day. Jaw is tight and sore. Autoimmune, females middle age 20-30

aka "Von Recklinghausen's disease"

cafè au Lait spots

"coast of california" appearance

schwann cell tumor

(schwannoma)

• aka Paralysis agitans, "Mohammad Al Lewy"
• problem with dopamine in basal ganglia. 
• Inclusion bodies = lewy bodies
• masked faces, stooped posture, resting tremor, and festinating/shuffling gait and propulsive gait.
• Cogwheel rigidity
• Substantia nigra of mesencephalon effected

PLS

Posterolateral sclerosis

• aka combined systems disease
• dorsal columns and corticospinal tract affected
• results from long term pernicious anemia (↓intrinsic factor) or ↓vitamin B12.

If affects anterior horn, produces LMNL.

Effects brainstem, then cranial nerves affected, possibly breathing apparatus as well

cyst like formation from the central canal of the spinal cord that leads to loss of pain and temperature and signs of an Upper motor neuron lesion(UMNL). "Cape like" distribution of pins and needles Sensation.

Caused by syrinx, a central dialation of the spinal cord.

loss of pain and temp bilaterally

Alcoholic psychosis with dementia.

Depletion of thiamine (B1) due to severe alcoholism

Horizontal Nystagmus

vs.

vertical nystagmus

horizontal nystagmus = MS

vertical nystagmus = lesion of the pons

Aneurismal Bone Cyst

"ABC"

Benign bone tumor

Metaphyseal/diaphyseal. ECCENTRIC.

"blister of bone"

less than 20years old

encapsulated. Short zone of transition.

Asymptomatic (usually)

"Geographic lesion"= confined

Starry sky appearance. 

jaw mass

Central African child.

Epstein Barr Virus

Benign bone tumor

less than 20 yrs old....under 20

epiphyseal/metaphyseal

Cancer that is most commonly seen in Sacrum

Crosses joints

primary -> sacrum

secondary -> occiput 

most commonly found in US

Greastest form = Adenocarcinoma

Absence of one or both testicles in scrotum(failure of descent of the testes).

Can cause testicular cancer

Multi-laminated (onion skin appearance) periosteal reaction

Cortical saucerizatoin (concavity)

Children 10-25.

Moth-eaten appearance in medulla →mimics Osteomyelitis

most common breast tumor

associated with estrogens.

upper outer quadrant of the breast

Myxomatous tissue

spindled and polyhedral cells

benign tumor, ground glass appearance.

cafè au lait, "coast of Maine" appearance(jagged)

w/ early puberty="Albright's disease"

Monostotic form = "rind sign" "rind of stenosis"

(looks like the rind of a fruit ex: orange)

tumor of osteoclasts. Osteoclastoma.

Most common in KNEE (distal femur/prox. tibia)

age 20-40 ... over 20

Quasi-malignant (still benign), epiphyseal/metaphyseal

"soap bubble appearance"

Benign tumor of blood vessels, most commonly found in spine

(Vertical striations aka corduroy cloth appearance)

Most common Primary Liver Cancer

aka Hepatoma

Histiocystosis X

Young kids

"Hans-Schüller-Chirstian","Histolyticgranuloma", "Eosinophilic Granuloma". 

 Lipid accumulates→ "Vertebra Plana"

(12 year old w/ "waffer thin vertebrae")

Pel-ebstein fever, Reed sternberg cells, hepatosplenomegaly, enlarged lymph nodes.

↑ IgE

Cancer of stomach that metastasizes to ovaries.

signet ring cell tumor

Tumor of smooth muscle

Fibroids  in the uterus

white patch  on oral mucosa from tobacco.

Cannot be wiped off (precancerous)

Lung Cancers

(3)

1. Adenocarcinoma
2. Oat cell and small cell
3. Squamous cell

(Misc:  can get lung cancer from arsenic poisoning. arsenic in treated wood)

metastatis from lung most commonly goes to the brain

most common lung cancer with  non-smokers

BEST PROGNOSIS

Lung cancer

most common in Smokers

non-encapsulated. long zone of transition

Pain at night =deep and boring. weightloss

Most common form of cancer in bones 

(axial and appendicular skeleton)

Multiple Myeloma

"plasma cell leukemia/sarcoma"

most common primary Bone Malignancy in adults

Amyloid buildup. Inclusion bodies=Russell bodies

Xray: "punched out lesions", raindrop skull (spots) Cold on a bone scan

Lab: IgG M-spike, Bence Jones proteinurea, Reverse A/G ratio, rouleaux formation (stacked coins), M-spike on immunophoresis, normocytic, normochronmic anemia, elecated ESR

most commonly seen in neural arch (posterior elements fo the spine)

Age 10-30

most  common  benign tumor of extremities

2 types:

1)Sessile (broad bump in the bone)

2)Pedunculated (cartilaginous cap appearacnce, coathanger's exostosis, mushroom projeciton or cauliflower appearance)

*Mulitple osteo chondromas = hereditary multiple exostosis (HME)

Pain worse @ night, relieved by Aspirin.

age 15-25, Radiolucent nidus w/ surrounding reactive sclerosis

#1 DDX = Brodie's Abscess

most common benign tumor of the skull

most common in Frontal sinus (frontal bone)

Brodie's Abscess if chronic.

sequestrum, involucrum, cloaca.

most common cause = Staph Aureus

Most common PRIMARY bone cancer in children

age 10-30

Spickulated (sunburst, radiated)

worst prognosis

very painful

NEUROblastoma, benign tumor of adrenal medulla (chromaffin cells)

causes episodic hypertension

cancer of Connective tissue

usually spread by vascular system

"SBC" or "UBC". 

benign bone tumor.

Metaphyseal/diaphyseal. Concentric. "fallen fragment sign". Age = <20. Found in long bones

Most common in Lesser Curvature (pyloric antrum)

"leather bottle" shaped stomach presentation (adenocarcinoma)

enlarged supraclavicular lymph nodes due to metastasis, usually GI tract, usually LEFT side supraclavicular lymph nodes. 

Not age dependant, abdominal metastasis

NEPHROblastoma

Mixed tumor of kidney in children

Immune & blood terminology

(11)

1. Anoxia
2. Hypoxia
3. Septicemia
4. Bacteremia
5. Chemotaxis
6. Pavementing/margination
7. Diapediesis AKA "leukocyte extravasation"
8. clot
9. thrombus
10. embolus
11. exudate
12. Transudate

white blood cells lining the damaged vessels.

(adension of leukocytes to endothelium wall)

aka: leukocyte extravasation

neutrophils and monocytes squeeze through the walls

 of the blood vessels towards the site of damaged tissue or infection

foreign body in the blood travels. (thrombus onvacation)

Fatty Emboli from a fractured in long bones. 

Emboli in lung most common from veins in lower extremity

Fluid and high protein (damaged capillary wall)

"everything exits".. lots of protein 

Fluid and Low protein (normal capillary wall)

"a little protein"

1. Injury/wound/insult
2. Vasoconstriction
3. Vasodilatation 
4. Swelling/edema
5. Healing

Bring more blood to injured site. 

Histamine and bradykinin dilate vessels

Cells move into damaged area. 

Chemotaxis →diapedesiss/leukocyte extravasation

Mast cells make heparin & serotonin. 

Serotonin: for wound healing, stimulates the myofibroblasts which cause constriction and form type 3 collagen for 'early wound healing'. 

firbrin forms the scar.

Inflammatory Substances

(7)

1. Bradykinin/histamine: vasodialation (↑inflammation)
2. Leukotrienes: Inflam. pathway (↑inflammation)
3. Prostoglandins: Inflam. pathway (↑inflammation)
4. Substance P: Inflam. pathway (↑inflammation)
5. Enkephalins/endorphins: Pain control(↓inflammation) rub it when it hurts
6. NSAIDs: Non-steroidal anti-inflamm. Cycloxygenase (COX)inhibitor. ex: advil, motrin, ↓inflammation
7. Steroids: Phospholipase A2 inhibitor, ex: prednisone (↓inflammation)

Immune cells

(7)

1. Basophils
2. B-lyphocytes
3. Granulocytes
4. Agranulocytes
5. Macrophage
6. Natural killer cells
7. T-lymphocytes (mature in thymus)

Become mast cells,

make histamine, bradykinin, serotonin, heparin.

monocyte at tissue layer

Kupffer = liver

Dust cells =lung

Microglia = brain

Langerhan = skin

"MEN": Monocytes, eosinophils, neutrophils.

"MEN" are natural born killers 

mature in thymus

1. T-Helper cells (CD4)
2. T-Killer cells (CD8)
3. T-Suppressor cells (T-regulatory cells)

CD4 cells

activate B cells

"Quarterbacks of the immune system"

CD8 cells

cytotoxic cells

Destroys cancer. Kills foreign agents (cancer cells)

ex: transplant rejections

T-regulatory cells

↓ immune cells. slows antibody production

White Blood Cells

(5)

Never Let My Engine Blow

60 - 30 - 8 - 3 - 0

1. Neutrophils
2. Lymphocytes
3. monocytes
4. eosinophils
5. Basophiles

5-8% Chronic conditions. 

Changes in to phagocytes at tissue level

Most abundant for chronic inflammation

2-4% Increase in parasites and allergies

Produces histaminase

0-0.5% Like mast cells, produce heparin & histamine

↑ conditions that cause histamine release

Long term dibilatating conditions (ex: Hodgkin's)→see basophiles in blood

1. Antigen → Macrophage (antigen presenting cell, is this friend or foe?)(Present antigen to CD4)"Quarterback"
2. →CD4 cell → produces lymphokines
3. →stimulates B-lymphocytes→ converts to plasma cells → produce antibodies→neutralizes the antigen that came in.

Natural Immunity

"innate immunity"

Involves: 

1. Natural keller cells (MEN)
2. Mast Cells
3. Basophils
4. Macrophage (monocyte)

Blood cell disorders

(6)

1. Bruton's Agammahlobulinnemia
2. DiGeorge's
3. Burkett's Lymphoma
4. Hodgkin's
5. Multiple Myeloma
6. Sever combined Immune Deficiency

Bruton's B cells

↓ B cells and Decreased IgG

Most common primary immunodeficiency at birth

X-linked

Leukemia of bone marrow

Affects plasma cells. age 50+

Example of non-hodgkins lymphoma

Newborn: without proper B or T-cells. Problem with Bone marrow

Adult: most common due to aplastic anemia. Possibly form bezene poisoning (gas)

1. Injury →though to release thromboplastin (aka tissue factor,aka factorIII)(need vitamin K to be present)
2. Thromboplastin triggers production of Prothrombin in liver
3. Prothrombin (using Vitamin K & Calcium) converts to thrombin
4. Fibrinogen (using thrombin) converts to Fibrin(clot)

Fibrinogen is also made in the liver. Positive Feedback aka "feed-forward". More thrombin produced, the more fibrin produced. 

5. Clot breakdown: firbolysin/plasmin dissolve clots

Factor number: 8 deficiency

Von Willibrand disease

Decrease in platelet adhesion

Factor number: 9 deficiency

• Christmas factor disease
• x-chromosome
• female = carrier, males = christmas disease

Factor number: 10

• Stuart factor
• clotting factors 

Factor number: 11 defeiciency - mild

Factor number: 12 

• Hageman Factor
• clotting factor

1. Aplastic anemia
2. Decrease production of RBCs
3. Loss of blood
4. Pancytopenia
5. polycythemia
6. thrombocytopenia

Lack of development (immature)

decrease RBCs and WBCs  in the blood(B+T lymphocytes)

Caused by benzene poisoning(acquired), (associated with degeneration of bone marrow) -atrophy

Decreased production of RBCs

Nutritional

1. Iron Deficiency= most common cause of chronic blood loss(menses) Microcytic, Hypochromic
2. Prenicious anemia= lack of intrinsic factor (fastritis can be a cause)
3. Folic Acid(B9)= in pregnancy (megaloblastic anemia. Leads to spina bifida occulta)
4. B-12 =seen in vegetarians (macrocytic, hypochromic anemia)

Decreased production of RBCs

Bone marrow suppression

1. Toxins (Benzene →aplastic anemia)
2. Chemotherapy - increases RBC production

Anemia's Loss of blood causes

(5)

1. Chronic blood loss→ ↓iron = microcytic, hypochromic anemia
2. Hemorrhage = normochromic/normocytic anemia
3. Hemolytic breakdown due to sickle cell or malaria
4. Thalassemia Major,aka "cooley's anemia," "Homozygous Beta thalassemia" aka "Mediterranean Anemia." "Hair on end" appearance on skull xray
5. Erthroblastosis fetalis: Rh- mother, Rh+ father. Rh+ baby. Type II Cytotoxic

↑RBCs

1° polycythemia = Polycythemia Rubra Vera = Pathological

2°polycythemia = relative polycythemia =normal response to high altitudes

Heart and Blood vessel disease

(15)

1. Aneurysm
2. atherosclerosis
3. Buerger's
4. Cardiac tamponade
5. Coarctation of aorta (congenital)
6. Monkeberg's sclerosis
7. myxoma
8. Prinzmetal's angina
9. Raynaud's
10. rhabdomyoma
11. sickle cell anemia
12. takayasu arteritis
13. temporal arteritis
14. tetralofy of fallot (congenital)
15. valvular lesion

deposition of fatty plaque(atheroma)

form of arteriosclerosis

get irregularly distributed lipid deposits in the intima of large and medium sized arteries, causing narrowing of arterial lumens

aka Thromboangitis obliterans

Lower extremity, males with history of smoking

Intermittent Claudication = cramping with exertion

Fluid in pericardial space

Prevents proper centricular filling, Emergency room referral

Coarctation of aorta

(congenital)

narrowing of aorta distal to the left subclavian

pulse pressure is greater in upper extremity vs lower.

Produces hypertension in upper extremities and hypotension in lower extremities.

Tunica media of medium-sized arteries calcified

in smokers and diabetics

Vasospasm of arteries. Maybe primary or secondary to other collagen diseases such as lupus.

Found in hands and feet. Produce triphasic color change, from pallor (white) to Cyanosis(blue) to Rubor(red).

Brought on by cold or emotion. Can lead to dry gangrene.

Typically in Upper extremities of females with a history of smoking.

half moon shaped RBC, "H-shaped vertebra"

homozygous sickle cell allele

aka "pulseless disease"

granulomatous inflammation of the aortic arch

aka "giant cell arteritis." Affects temporal arteries. 

Associated with long-standing hypertension.

If affects ophthalmic artery, can lead to blindness.

↑ESR. Best eway to diagnose is by biopsy

Tetraolgy of Fallot

(congenital)

pneumonic = "DRIP"

• Dextrorotation of aorta
• Right ventricular hypertrophy
• Interventricular septal defect
• Pulmonic stenosis

Due to tooth extraction (Strep infection causing Aschoff bodies from Rheumatic fever).

Mitral valbe affeted

Syphilis affects aortic valve and strep affecs mitral valve(rheumatic fever).

• acute lymphoblastic (ALL)
• Acute Myeloblastic (AML)
• Chronic Lymphocytic (CLL)
• Chronic Myelocytic (CML)

ALL

most commonly seen in children

AML

any age

Most common = ACUTE leukemia in adults (85% of cases are in adults)

Worst prognosis

CLL

most common chronic in adults

chronic leukemia

CML

young adulthood (30-40), increased granulocytes,

"philadelphia chromosome" #22

Genetic diseases

• Achondroplasia
• Alkaptonuria
• Charcot Marie Tooth
• Down's syndrome
• Edward's syndrome
• Huntington's chorea
• Marfan's 
• osteogenesis imperfecta
• Phenylketonuria
• Sickle cell anemia 
• Von Gierke's 
• Cri du Chat

Most common form of genetic dwarfism: growth plates affected(epiphyseal plate closes too early)

failure of normal epiphyseal cartilage formation.

Radiology: "bullet vertebra", "Trident hand"

Alkaptonuria

Accumulation of homogentisic acid, blue-black deposits in cartilage, ears, nose, and cheeks. 

Causes ochronosis (calcification of discs in spine)

Urine turns black (d/t oxidation) on standing collection

(can get↑ of tyrosine Homogentisic acid is intermediate in phenylalanine & tyrosine metabolsim)

hereditary progressive peroneal muscle atrophy

Affects tibialis Anterior also

Trisomy 21 male or female. retarded. Flat, hypoplastic face.

Transverse palmar crease.(Simian crease)

Transpostion with chromosome 14 (translocation)

Trisomy 18

retardation

Herediatry onset age 30-50

basal ganglion affected: atrophy of caudate nucleus

on Chromosome #4. Decreased in GABA

Produces dementia and death

Arachnodactyly(spider-like fingers)

subluxation or dislocation of the lens of the eye,

dissecting aortic aneurysms

tall stature.

Defect in type I collagen

blue sclera and brittle bones

can't convert phenylalanine to tyrosine due to

phenylalanine hydroxylase

Retardation can be controlled by diet if caught early.

(phenylalanine free diet for life)

half moon shaped RBC, "H-shaped vertebra"

Homozygous sickle cell allele

Lacks enzyme (glucose-6-phosphatase) in liver

Excess glycogen stored in liver, can't break down.

Glycogen storage disease

• Gaucher's
• Krabbe's
• Niemann Pick's
• Tay Sach's

Lipodystrophy. 

Excess glucocerebrosides in brain, liver and spleen

Lipodystrophy

Excess galactocerebrosides in white matter

Lipdystrophy

↑sphingomyelin, a sphingomyelinase deficiency

Tay Sach's disease

Lipodystrophy

↑Gangliosides in brain. Glycosphingolipid. 

Hexosaminidase A deficiency.

Cherry red spots on the macula, infant death.

most common in Ashkenasi Jews.

• Duchene's Muscular dystrophy
• Fascioscapulohumeral dystrophy
• limbgirdle dystrophy
• Myotonic dystrophy

Muscular dystrophy. boys age 3-7

Pseudohypertrophy of calves. Muscle replaced by fat. Recessive X-linked. ↑CPK (creatine phosphokinase).

Gower's maneuver. Waddling gait. Proximal muscles affected. Females are carriers.

Autosomal dominant form of muscular dystrophy in adults. Affects face, scapula, upper arms.

X-linked, form of duchene's

Autosomal class of muscular dystrophy. 

effects hips and shoulders

• Fragile X: m/c form mild mental retardation cause by fragile site at "XQ27"
• Kleinfelter's: 47XXY karyotype, tall male, low IQ,  testicular atrophy, gynecomastia, sterile
• Turner's: 45XO Karytotype, female, short, webbed neck,  amenorrhea, lacks female secondary sex characteristics. Can't turn neck b/c web neck.
  

non-inflammatory, aka "Diffuse idiopathic skeletal hyperostosis", "Forrestier's disease" flowing hyperostosis","candlestick appearance of 4 or more segments

age,S/sx= male 50+, Trouble swallowing due to calcification of ALL

Associated with DM(2-hr postprandial, urinalysis), increased appetite, thirst, urination

(30% correlation between DISH and diabetes mellitus)

Never causes a decrease in disc heights

Calcium or bone within muscle due to trauma

Most common in quads or biceps.

If in adductors, known as "Prussian's disease"

Example of metaplasia