Clin Med - Id, Heme/onc, Pituitary/adrenal

Front 1

growth hormone overview

Back 1

release is pulsatile,

circadian (Dawn phenomenon),

higher in adolescents

major target = liver (makes IGF-1)

decreases glucose uptake in muscle, fat;

stimulates gluconeogenesis & glycogenolysis

Front 2

growth hormone is released from the...

Back 2

anterior pituitary

Front 3

factors that increase GH?

that decrease GH?

Back 3

increase GH: hypoglycemia, exercise, starvation, other hormones (estrogen, testosterone, thyroid)

decrease GH: IGF-1, somatostatin

Front 4

growth hormone target?

Back 4

liver (releases IGF-1)

Front 5

effect of GH & IGF-1?

Back 5

decrease glucose uptake in muscle, fat;

stimulate gluconeogenesis & glycogenlysis

raise blood glucose

(cartilage, bone, tissue growth)

Front 6

Dawn phenomenon

Back 6

morning increase in blood sugar

not assoc. w/ nocturnal hypoglycemia

(unlike Somogyi)

Front 7

pituitary dwarfism

Back 7

is a congenital GH deficiency;

proportionate;

normal birth wt but fail to grow;

usually noted by 2-3 y/o

Front 8

Laron syndrome

Back 8

congenital GH receptor deficiency

(GH resistant)

low IGF-1;

hypoglycemia & seizures;

resistant to diabetes & CA

Front 9

Tx Laron syndrome

Back 9

synthetic IGF-1 (mecasermin)

(don't respond to Tx w/ GH)

Front 10

S/Sx acquired GH deficiency

Back 10

central obesity,

high systolic BP,

high LDL,

impaired memory/concentration,

depression

Front 11

work-up for GH deficiency

Back 11

electrolytes (low bicarb)

IGF-1 & IGFBP-3 (low), TSH,

karyotype (XO Turner syndrome),

*MRI (to exclude tumor craniopharyngioma)*

GH difficult to measure d/t pulsatile release

Front 12

Tx GH deficiency

Back 12

synthetic GH =

somatrem (Protropin)

SQ injection 3-7x/week

Front 13

Why do we no longer use naturally obtained GH to treat GH deficiency?

Back 13

Creutzfeldt-Jakob disease

(fatal brain disorder d/t a prion)

Front 14

ADRs of using synthetic GH

Back 14

acromegaly,

myopathy (bigger but weaker),

diabetes,

cardiomyopathy & CHF,

leukemia

Front 15

role of ACTH?

Back 15

stimulates adrenal gland to produce cortisol

(and DHEA)

Front 16

S/Sx ACTH deficiency

Back 16

less dramatic than adrenal gland destruction

(e.g. Addison's)

weakness, fatigue,

wt loss, HOTN

Front 17

Dx ACTH deficiency

Back 17

low FBG, sodium (esp. if TSH deficit too), DHEA, Epi (K+ unaffected)

ACTH stimulation testing

MRI (thick stalk in sarcoid, hypophysitis)

consider labs for hemochromatosis, underlying metabolic disorder

Front 18

ACTH stimulation testing

Back 18

to Dx ACTH deficiency

(unreliable, contraindicated in some)

give ACTH IM; check cortisol at 30-60 min;

below 20 mcg/dL (550 nmol/mL) = abnormal

Front 19

Kallman syndrome

Back 19

congenital gonadotropin deficiency;

hypogonadotropic hypogonadism;

(sporadic, X-linked, or auto recessive)

olfactory bulb often affected = anosmia;

consider in DDx for primary amenorrhea

Front 20

S/Sx acquired gonadotropin deficiency

Back 20

decreased androgen production

decreased libido,

hair loss,

muscle atrophy,

osteopenia

Front 21

function of ADH?

Back 21

causes distal renal tubules

to increase water reabsorption

(decreases serum osmolality,

increases urine concentration)

Front 22

S/Sx deficiency of ADH

Back 22

polydipsia & polyuria (2-20 L/d)

usually can maintain fluid/lyte balances,

but if not = dehydration, hypernatremia

Front 23

primary central diabetes insipidus

Back 23

1/3 of diabetes insipidus cases;

autoimmune against vasopressin-secreting cells;

MRI: no masses, stalk thickening

familial, Wolfram (DIDMOAD) syndrome

Front 24

Wolfram syndrome

Back 24

aka DIDMOAD;

rare genetic disorder causing:

- diabetes insipidus (primary central)

- diabetes mellitus

- optic atrophy

- deafness

Front 25

secondary central diabetes insipidus

Back 25

damage to pituitary stalk

(e.g. trauma, infxn, metastatic disease)

Front 26

vasopressinase induced diabetes insipidus

Back 26

in 3rd trimester in pregnancy;

enzyme destroys native vasopressin

(respond well to Tx w/ synthetic vasopressin)

resolves after pregnancy

Front 27

nephrogenic diabetes insipidus

Back 27

insensitivity to vasopressin

Front 28

hormones released from the posterior pituitary gland?

Back 28

ADH (aka vasopressin),

some oxytocin

Front 29

Dx diabetes insipidus

Back 29

24 hour urine

(if less than 2L, r/o DI)

glucose, urea nitrogen, K+, Na+, uric acid

vasopressin challenge test

Front 30

vasopressin challenge test

Back 30

for eval. of diabetes insipidus;

give desmopressin acetate (IN, IV, or SC);

measure urine volume 12h before & 12 hr after

monitor for hyponatremia

Front 31

Tx mild diabetes insipidus

Back 31

fluid intake & low sodium diet;

avoid aggravating agents (e.g. corticosteroids)

Front 32

Tx mod-severe diabetes insipidus

Back 32

dDAVP (desmopressin)

*doesn't work if nephrogenic

(low doses to prevent hyponatremia)

adjuncts:

- carbamazepine (inc. ADH response)

- Hctz (dec. polyuria)

- chlorpropramide (anti-diuretic)

- indomethacin

Front 33

most common cause of acromegaly/gigantism?

Back 33

pituitary adenoma (90%)

(sporadic, familial (MENI), paraneoplastic syndromes, etc.)

Front 34

S/Sx acromegaly

Back 34

big hand/feet size; facial changes

insulin resistance (30%),

laryngeal hypertrophy (deep voice),

OSA, HTN (50%), wt gain,

cardiomegaly, CHF,

acanthosis nigricans, acne,

hypogonadism (cosecretion of prolactin)

Front 35

work-up for acromegaly

Back 35

serum IGF-1, IGFB-3 (elevated)

give 75-100mg glucose syrup,

check GH in 1hr (in normal pt, GH suppressed);

prolactin (20% GH-secreting adenomas also secrete PRL; consider others like TSH, FSH, LH);

TRH stimulation test (unnecessary)

Front 36

Tx acromegaly (surgery)

Back 36

transphenoidal microsurgery

(curative 60-70%;

complications = infxn, hypopituitarism)

+/- sterotactic radiosurg (gamma knife)

Front 37

Tx acromegaly (meds)

Back 37

somatostatin analogs (octreotide);

GH recept. antag. (Pegvisomant);

DA recept. agonists

(adjunct; decrease prolactin levels;

Cabergoline (+/- bromocriptine, pergolide))

Front 38

patho: prolactinoma

Back 38

(one cause of hyperprolactinemia)

women more than men;

microadenoma;

sporadic or familial (MEN I)

Front 39

causes of hyperprolactinemia

Back 39

prolactinoma,

pregnancy, exercise, stress,

antipsychotics, estrogens, SSRIs,

acromegaly, chest/breast trauma,

cirrhosis, hypothyroidism

Front 40

S/Sx hyperprolactinemia

Back 40

women: galactorrhea, amenorrhea (oligomenorrhea)

men: hypogonadism

Front 41

work up for hyperprolactinemia

Back 41

prolactin levels

(look for other causes:

TSH, FSH, LH, kidney/liver disease)

MRI

Front 42

Tx prolactinoma

Back 42

DA agonists (Cabergoline, bromocriptine)

- normalize PRL

- shrink tumor up to 50%

- recurs if stopped

estrogen replacement/oral contraceptives;

transphenoidal pituitary surgery

+/- XRT/gamma knife (refractory cases)

Front 43

from where is aldosterone released?

Back 43

zona glomerulosa of the adrenal cortex;

is a mineralocorticoid

(production stimulated by angiotensin II)

Front 44

from where is cortisol released?

Back 44

zona fasciculata of the adrenal cortex;

is a glucocorticoid

(production stimulated by ACTH)

Front 45

from where is DHEAS released?

Back 45

zona reticularis of the adrenal cortex;

is an androgen

(production stimulated by ACTH?)

Front 46

from where are catecholamines released?

Back 46

adrenal medulla

Front 47

causes of adrenocortical insufficiency (Addisons)?

Back 47

autoimmune (PGA, DM I, thyroid, ovarian fail);

trauma, infxn (TB),

adrenoleukodystrophy,

congenital adrenal hypo/hyperplasia,

exogenous steroid use

Front 48

adrenoleukodystrophy

Back 48

a cause of adrenocortical insufficiency

(Addison's disease)

X-linked, boys,

psych, neuro

Front 49

S/Sx Addison's disease

Back 49

insidious (years), anorexia,

weakness, fatigue, N/V,

diarrhea, ortho HOTN,

myalgias, arthralgias,

salt craving (d/t hyponatremia),

delirium, fever,

oral hyperpigmentation (1st deg only)

Front 50

serology results for Addison's disease

Back 50

normochromic normocytic anemia,

eosinophilia,

low Na+ (88%), serum glucose;

high K+, Ca++

Front 51

Dx Addison's disease

Back 51

8am cortisol levels below 3 mcg/dL

(elevated ACTH)

confirm w/ cosyntropin stim. test

(misc: 21-hydroxylase ab, long fatty acid chains in males (adrenoleukodystrophy), low DHEA, serum Epi)

Front 52

cosyntropin stimulation test

Back 52

confirms Dx of Addison disease

give synthetic ACTH (cosyntropin);

primary disease will not respond

w/ approp. increase in cortisol levels

Front 53

Tx Addison's disease

Back 53

hydrocortisone (or prednisone) BID;

fludrocortisone (mineralocort. to preserve Na+),

+/- DHEA

consider increasing hydrocortisone dose

during "stress"

Front 54

causes of Addisonian crisis?

Back 54

stress (infxn, trauma, surgery),

withdrawal of steroid in pt w/ insufficiency,

bilat. adrenalectomy,

pituitary destruction,

Etomidate (anesthetic)

Front 55

S/Sx Addisonian crisis

Back 55

non-specific; often mistaken for other things

HA, N/V, abd pain,

diarrhea, confusion/coma,

fever

Front 56

Tx Addisonian crisis

Back 56

hydrocortisone (IV then PO),

+/- mineralocorticoid (fludrocortisone)

Front 57

Cushing disease vs. syndrome

Back 57

both are forms of hypercortisolism

disease: ACTH hypersecretion by pituitary

syndrome: adrenal/primary disorder or exogenous steroids

Front 58

causes of hypercortisolism?

Back 58

Cushing disease,

Cushing syndrome,

idiopathic,

ectopic ACTH tumors,

alcoholism, anorexia,

GHB, depression

Front 59

S/Sx Cushings syndrome

Back 59

amenorrhea (high androgen),

truncal obesity (90%),

HTN, glucose intolerance,

hirsutism (70%),

osteoporosis,

wide purple striae (abd, thighs),

moon facies,

buffalo hump

Front 60

screening for Cushing's disease

Back 60

24hr urine for cortisol,

midnight salivary cortisol,

dexamethasone suppression test (low dose)

Front 61

dexamethasone suppression test

Back 61

screening test for Cushing's disease;

give 1mg dexamethasone at 11 pm;

check cortisol levels at 8 am

(normal = suppressed cortisol)

Front 62

Cushing's disease - how to differentiate between adrenal vs. pituitary source?

Back 62

high dose dexamethasone suppression test

pituitary = cortisol suppressed

adrenal = cortisol not suppressed

(ectopic source = will not suppress

& ACTH will remain elevated)

Front 63

dexamethasone

Back 63

exogenous steroid;

suppresses ACTH secretion from pituitary

Front 64

Tx Cushings

Back 64

surgical resection

Front 65

prognosis Cushings

Back 65

adrenal adenoma: 90% 10 yr survival

pituitary adenoma: 90% 10 yr survival, but may be inoperable (+/- XRT/gamma knife)

ectopic ACTH secreting tumor: depends

Front 66

Conn disease/syndrome

Back 66

aldosterone-producing adrenal adenoma

most common cause

of primary hyperaldosteronism

Front 67

causes of hyperaldosteronism

Back 67

primary: adrenal adenoma (Conn),

adrenal hyperplasia (excess ACTH stim.),

unilat. adrenal hyperplasia,

adrenal carcinoma

secondary: renin-producing tumor,

edematous states (nephrotic syndrome)

Front 68

function of aldosterone?

Back 68

mineralocorticoid

acts on distal tubules

to retain salt & water; waste K+;

antagonist to atrial natriuretic peptide

= increased BP

Front 69

S/Sx hyperaldosteronism

Back 69

HTN (refractory),

HA, muscle weakness (hypokalemia)

often asymptomatic

Front 70

labs: hyperaldosteronism

Back 70

hypokalemia (37%),

+/- mild hypernatremia

metabolic alkalosis (elevated bicarb)

Front 71

Hypokalemia + HTN

= 50% predictive value for...

Back 71

hyperaldosteronism

Front 72

when to screen for hyperaldosteronism?

Back 72

Stage II or III HTN,

refractory HTN (3 drug regimen),

HTN w/ adrenal incidentaloma,

FHx of early onset HTN or CVA under 40 y/o,

HTN w/ 1st deg relative w/ Hx PA

Front 73

main screen for hyperaldosteronism?

Back 73

aldosterone-to-renin ratio (ARR)

(plasma aldosterone)/(plasma renin activity)

20-40 = normal

AM testing, sit upright x2 hrs,

correct hypokalemia 1st, watch out for meds

Front 74

misc. tests for hyperaldosteronism

Back 74

saline infusion test,

oral salt loading test,

captopril test,

fludrocortisone suppression test

differentiates unilat. vs. bilat.;

CT or SPET/CT;

adrenal vein sampling

Front 75

Tx hyperaldosteronism

Back 75

unilat (adenoma): surgical

bilat: spironolactone (1st line);

alts: eplerenone, amiloride

(monitor K+ & creatinine q6 mo)

Front 76

etiology: pheochromocytoma

Back 76

usually benign tumor adrenal medulla (90%);

excess catecholamine production

peaks 3-5th decades

Front 77

S/Sx pheochromocytoma

Back 77

HTN (secondary),

HA, diaphoresis,

tachycardia/palpitations,

chest pain, anxiety,

tremor, flushing

may have acute crises d/t stress, meds

Front 78

Dx pheochromocytoma

Back 78

24 hour urine metanephrines,

catecholamines, VMA

misc: plasma free metanephrines & catecholamines; clonidine suppression test

Front 79

clonidine suppression test

Back 79

test for pheochromocytoma

check catecholamines before

& after giving clonidine

pos test = no decrease in levels

Front 80

Tx pheochromocytoma

Back 80

alpha & beta blockade

+/- surgery

Front 81

inherited causes of pancreatic tumors?

Back 81

MEN1,

Von Hippel-Lindau disease,

neurofibromatosis 1 (NF-1),

tuberous sclerosis complex

Front 82

types of pancreatic cells

Back 82

A cells (20%) - secrete glucagon

B cells (70%) - secrete insulin

D cells (5%) - secrete somatostatin or gastrin

F cells - secrete pancreatic polypeptide

Front 83

insulinoma

Back 83

type of pancreatic tumor;

82% benign

secrete excess insulin, causing hypoglycemia;

often assoc. w/ MEN1 (multiples)

Front 84

gastrinomas

Back 84

type of pancreatic tumor;

acid secretion (Zollinger-Ellison),

duodenum, pancreas, lymph nodes;

benign or malignant (mets to liver),

often assoc. w/ MEN1

Front 85

glucagonomas

Back 85

type of pancreatic tumor;

malignant w/ liver mets

Front 86

somatostatinomas

Back 86

type of pancreatic tumor;

very rare, causes DM

Front 87

vasoactive intestinal polypeptide (VIPoma)

Back 87

type of pancreatic tumor;

causes diarrhea

Front 88

S/Sx MEN1

Back 88

multiple endocrine neoplasia 1;

hyperparathyroidism,

pancreatic tumors

(insulinomas, gastrinomas, non-secretory),

pituitary adenomas

others: adrenal adenomas, nonendocrine tumors

Front 89

S/Sx MEN2a

Back 89

aka Sipple Syndrome

medullary thyroid cancer,

hyperparathyroidism,

pheochromocytoma

Front 90

S/Sx MEN2b

Back 90

medullary thyroid cancer,

pheochromocytoma,

mucosal neuromas,

GI problems

Front 91

causes of SIADH

Back 91

malignancy (bronchogenic carcinoma),

lung disease (PNA - legionella),

neuro (tumors, meningitis, MS, Guillain-Barre),

post-op, meds

(chemo, Tegretol, amiodarone,

theophylline, SSRIs)

Front 92

S/Sx SIADH

Back 92

serum: hypoosmolar hyponatremia,

hypouricemia

urine:

- high osmolarity (over 100),

- high sodium (over 20)

Front 93

Tx SIADH

Back 93

Tx underlying cause,

fluid restriction,

furosemide,

hyponatremia

if refractory, induce nephrogenic DI

w/ demeclocycline, lithium

Front 94

Tx for strep pharyngitis?

Back 94

Pen VK

Front 95

S/Sx scarlet fever

Back 95

strep throat w/ sandpaper rash;

circumoral pallor, facial flushing,

strawberry tongue

Front 96

S/Sx & Tx erysipelas

Back 96

abrupt onset; typically on face;

well-defined macular erythematous lesion;

need admission!

Tx superficial cellulitis:

PCN, cephalosporin, macrolide

Front 97

S/Sx & Tx impetigo

Back 97

crusty honey yellow lesions

(Staph can cause bullous impetigo)

Tx Bactroban ointment

Front 98

Tx necrotizing fasciitis

Back 98

call surgeon ASAP!

Tx w/ broad spec antibiotics

Front 99

Tx toxic shock syndrome

Back 99

abx & supportive

specific IVIG to neutralize toxin

Front 100

non-suppurative complications of Group A Strep

Back 100

acute glomerulonephritis,

rheumatic fever (Jones criteria),

guttate psoriasis,

scarlet fever

Front 101

major Gram neg. bacteria?

Back 101

SPACE organisms:

Serratia

Pseudomonas

Acinetobacter

Citrobacter

Enterobacter (E. colI)

Front 102

S/Sx & Tx botulism

Back 102

(Clostridium botulinum)

in soil, honey, foods

diplopia, flaccid paralysis,

dry mouth, dysphagia,

N/V, AMS, resp. depression

Tx: anti-toxin

Front 103

source, S/Sx, & Tx anthrax

Back 103

(Bacillus anthracis)

sheep, goats, swine, horses

derm - painless eschar (black center);

pulm - flu-like;

GI - bloody diarrhea

Tx: fluoroquinolone

Front 104

rice water diarrhea

pathogen? treatment?

Back 104

Vibrio cholerae

(contaminated food/water)

fluids, Bactrim, quinolone

Front 105

S/Sx & Tx tetanus

Back 105

(Clostridium tetani)

in soil

opisthotonos, trismus,

hyperreflexia, nuchal rigidity,

spasms

Tx: tetanus immunoglobulin IM

Front 106

pea soup diarrhea

pathogen? Treatment?

Back 106

Salmonella enterica

(enteric fever - Typhoid)

Tx: fluoroquinolone

Front 107

pathogens, S/Sx, Tx shigellosis

Back 107

(S. sonnei, S. flexneri, S. dysenteriae)

abrupt onset;

bloody diarrhea, cramps,

tenesmus, chills, fever

Tx: Fluoroquinolone, Bactrim

Front 108

grey thick membrane over tonsils & pharynx.

pathogen? treatment?

Back 108

C. diphtheriae

Tx: Bactrim, erythromycin, antitoxin

(also treat contact)

Front 109

S/Sx, Tx gonorrhea?

Back 109

(N. gonorrhea)

discharge, dysuria, pain;

can cause unilat. conjunctivitis

Tx: ceftriaxone, Cefixime, azithromycin

Front 110

Neisseria meningitidis

Back 110

Gram neg. intracellular diplococci

(A, B, C, W-135, X, Y)

causes meningitis

Tx: Pen G, ceftriaxone, dexamethasone

Front 111

prevention of meningitis?

Back 111

MCV4 vaccine - Menactra

(against N. meningitidis)

2 doses, separated by 1 mo or more

prophylaxis for contacts: Rifampin, Cipro

Front 112

Lone Star Tick

disease? on blood smear?

treatment?

Back 112

Ehrlichiosis

flu prodrome, encephalitis

blood smear: Morula

Tx: doxycycline, rifampin (pregnancy, peds)

Front 113

infection assoc. w/ cat bites

Back 113

Pasturella

(Tx w/ Augmentin)

Front 114

infection assoc. w/ human bites

Back 114

Eikinella

(Tx w/ Augmentin)

Front 115

infection assoc. w/ "cat scratch disease"

Back 115

Bartonella henselae

Front 116

Tx thrush

Back 116

PO fluconazole/Nystatin

Front 117

Tx cutaneous candida

Back 117

topical antifungal

(Lotrimin / Miconazole)

Front 118

white cottage cheese vaginal discharge

pathogen? treatment?

Back 118

vulvovaginal candidiasis

Tx: fluconazole (PO x1),

intravaginal miconazole

Front 119

Tx fungemia

Back 119

IV amphotericin + flucytosine

Front 120

fungal infxn assoc. w/

Mississippi/Ohio river valley?

treatment?

Back 120

Histoplasmosis

Tx amphotericin, then itraconazole

Front 121

encapsulated budding yeast in soil

w/ pigeon dung?

can cause what S/Sx?

How to Dx?

Tx?

Back 121

Cryptococcus

can cause pulm disease, meningitis, etc.

(immunocompromised)

Dx India ink stain, latex agglutination assay

Tx: amphotericin, then fluconazole

Front 122

most common infxn in advanced HIV?

How to Dx? Tx?

Back 122

Pneumocystis jiroveci

Dx silver stain

Tx Bactrim

Front 123

Gardener's & rose handlers disease

pathogen? Tx?

Back 123

Sporothrix schenckii

(in soil)

Rx: azoles (for cutaneous disease)

Front 124

S/Sx leprosy

Back 124

chronic skin lesions, paresthesias,

lumps, nodules

claw fingers,

foot drop,

lack of eyebrows/lashes,

lion facies

(Tx Dapsone x2 yrs)

Front 125

pathogens: malaria

Back 125

Plasmodium genus

(P. vivax, P. malariae,

P. ovale, P. falciparum)

dx w/ Giemsa stain

Front 126

Tx malaria

Back 126

chloroquine

or mefloquine;

if severe: quinine IV + doxy

(not if G6PD defic.)

Front 127

prophylaxis of malaria

Back 127

1st choice: chloroquine

if resistant: doxy, malerone, mefloquine

Front 128

amebiasis

Back 128

E. histolytica

(in soil, water)

dysenteric colitis,

tenesmus, colic, vomiting

Dx: stool O&P

Tx flagyl + luminal amebicide (diloxaninde)

Front 129

hookworms

Back 129

Ancylostomiasis

dermatitis, cough, wheeze,

bloody sputum, low fever

stool = eggs, occult blood

Tx: Mebendazole, Albendazole,

ferrous sulfate

Front 130

Tx pinworms

Back 130

Albendazole

(Mebendazole,

Pyrantel Pamoate)

Front 131

Tx tapeworms

Back 131

Praziquantel

Front 132

most common intestinal protozoa in the US?

Back 132

Giardia lambia

(Dx stool O&P;

Tx flagyl, albendazole)

Front 133

Tx toxoplasmosis

Back 133

(from cat feces!)

Tx Bactrim DS PO daily

Front 134

disorders assoc. w/ Epstein Barr virus

Back 134

mono, Burkett's lymphoma,

CVD, chronic fatigue syndrome

Front 135

S/Sx mononucleosis

Back 135

(Epstein-Barr virus)

exudative pharyngitis,

palatal petechiae,

post. LAD,

splenomegaly (no contact sports 6 wks)

leukopenia, then lymphocytic leukocytosis

Front 136

rabies: pathogen, Dx, Tx

Back 136

retrovirus in Rhabdovirus family

Dx: LP (CSF RT PCR)

Tx: rabies immunoglobulin

Front 137

rabies: post-exposure immunization

Back 137

rabies immunoglobulin (HRIM) in wound

& vaccine (HDCV - RabAvert)

(if only a possible bite,

vaccine but NOT HRIM)

Front 138

Tx cytomegalovirus

Back 138

Gancyclovir,

Valganciclovir

Front 139

progressive multifocal leukencephalopathy

Back 139

d/t JC virus;

neurodegen. disease in AIDS pts;

Dx w/ PCR:

Tx reduce immunosuppression,

Cidofovir (if non-AIDS related)

Front 140

BK virus

Back 140

opportunist in transplant pts;

hemorrhagic cystitis (marrow transplant);

graft failure (renal transplant)

Dx: PCR of urine or blood

Tx: IV Cidofovir, GCSF,

decrease immunosuppression

Front 141

West Nile virus

Back 141

flavivirus (via mosquitos - arbovirus);

dead birds

S/Sx: non-specific viral, meningitis, encephalitis

Dx: IgG & IgM abs of serum/CSF (PCR)

Tx: supportive; maybe ribavirin?

Front 142

Lyme disease

Back 142

Borrelia burgdoferi

(deer tick - Ixodides scapularis)

erythema migrans;

then HA, malaise, joint problems,

pericarditis, encepahlopathy, etc.

Dx: ELISA w/ western blot

Tx: Doxy

Front 143

Rocky Mountain Spotted Fever

Back 143

Rickettsia rickettsii

(wood tick aka American dog tick)

S/Sx fever, rigors, HA, N/V,

conjunctivitis, cough,

rash on palms/soles, then spreads

Tx doxy (5-7 days)

Front 144

define "fever of unknown origin"

Back 144

fever (over 38.3 deg) x at least 3 wks

& no Dx after 3 outpt visits or 3 days inpatient

(most commonly d/t infection)

Front 145

plague

Back 145

Yersinia pestis

(gram neg; prairie dogs, squirrels, etc)

Wright stain;

Tx streptomycin,

gentamicin, chloramphenicol

Front 146

hantavirus

Back 146

RNA virus from rodents;

if severe: pulm edema, HOTN, ARF, MSOF, shock

Dx: PCR

Tx: isolation, supportive, ribavirin

Front 147

S/Sx typhoid fever

Back 147

progressive fever,

travel abroad (Africa),

relative bradycardia,

non-bloody diarrhea,

pink rash

Front 148

leading cause of bladder CA worldwide?

Back 148

schistosomiasis

(fresh water helminth;

travel abroad)

Dx: eosinophilia, stool eggs

Tx: praziquantel

Front 149

Trypanosomiasis

Back 149

African Sleeping Sickness;

protozoa (Trypanosoma brucei)

from tsetse fly;

Tx: anti-trypanosomal therapy

Front 150

traveler's diarrhea

Back 150

classically nonhemorrhagic E. coli

Tx: cipro, lomotil for diarrhea

Front 151

dengue fever

Back 151

RNA virus of Flavivirus genus;

DENV 1, 2, 3, 4; from travel;

mosquito vector

myalgias, HA, malaise, arthralgia,

"breakbone fever"

Tx supportive, fluids, APAP, ICU

(no ASA or NSAIDs d/t bleed risk)

Front 152

in adult, hematopoiesis occurs in...

Back 152

sternum, ribs,

vertebral bodies, pelvic bones,

prox. portions of long bones

Front 153

sites of extramedullary hematopoiesis?

Back 153

liver, spleen, lymph nodes

Front 154

RBC lifespan?

Back 154

90-120 days

(then removed by liver & spleen)

Front 155

myeloblasts can develop into...

Back 155

neutrophils,

eosinophils,

basophils,

monocytes

Front 156

avg lifespan of a WBC?

Back 156

13-20 days

(but varies by type & body condition)

Front 157

neutrophils

Back 157

first to arrive to site of injury

band cells - early response to infxn

Front 158

basophils

Back 158

release granules that contain

histamine & heparin

Front 159

monocytes

Back 159

become macrophages in tissues;

responsible for phagocytosis

possible relationship w/ EBV

Front 160

lymphocytes

Back 160

primarily respond to viral infxn

T cells: activate phagocytes, destroy bad cells

B cells: involved in making antibodies

NK cells: remove/contain viruses & tumors

Front 161

platelet lifespan

Back 161

9-12 days

(then removed by spleen)

Front 162

normal neutrophil count?

Back 162

2500-6000

(neutropenia below 1500-1800;

severe if below 500)

Front 163

new onset of isolated neutropenia is often due to...

Back 163

a drug reaction

(e.g. sulfonamides, PCN, cimetidine, chemo, cephalosporins, ART, phenytoin, etc)

Front 164

Felty syndrome

Back 164

immune disorder involving:

RA, splenomegaly, neutropenia

Front 165

non-marrow causes of neutropenia?

Back 165

hypersplenism,

sepsis,

HIV,

Felty syndrome

Front 166

Pts w/ agranulocytosis may present w/ sudden symptoms including:

Back 166

malaise, fever/chills,

pharyngitis, tachycardia,

tachypnea, HOTN,

Sx of septic shock

Front 167

most common infxns assoc. w/ neutropenia?

Back 167

septicemia,

cellulitis,

pneumonia

Front 168

Tx neutropenia

Back 168

supportive (prevent infxns)

myeloid growth factors

(Neupogen, Leukine, Neulasta)

(if autoimmune, use corticosteroids)

Front 169

Pt w/ neutropenia develops a fever. What now?

Back 169

infectious until proven otherwise!

can be life-threatening

start broad spec abx

(fluoroquinolones, cephalosporins);

if maybe fungal, use azoles

Front 170

Tx pure WBC aplasia & aplastic anemia?

Back 170

immunosuppression

(ATG, cyclosporine)

Front 171

myeloproliferative disorders include...

Back 171

polycythemia vera,

primary myelofibrosis,

essential thrombocytosis,

CML

(all assoc. w/ JAK2, older age, splenomegaly

may progress to AML)

Front 172

myeloproliferative disorder

w/ very elevated WBC count?

Back 172

chronic myeloid leukemia (CML)

Front 173

myeloproliferative disorder

w/ abnormal RBC morphology?

Back 173

primary myelofibrosis

Front 174

S/Sx polycythemia vera

Back 174

asymptomatic;

major concern = thrombosis

HA, blurred vision, dizziness,

tinnitus, fatigue, red skin, orthopnea,

erythromelalgia (extremity tingling/numb),

pruritus after warm shower (basophilia),

splenomegaly (abd fullness, bloating)

Front 175

polycythemia vera - pt demographic

Back 175

60% are men;

median age = 60 yrs

rarely seen below age 40;

not seen in younger women

Front 176

Dx polycythemia vera

Back 176

Hct over 54% (males) or 51% (females);

normal RBC morphology; low EPO;

high vit B12, hyperuricemia

(Hct & Hgb normal if ongoing bleeding)

if JAK2 not present, question the diagnosis

Front 177

when to suspect secondary polycythemia?

Back 177

no splenomegaly,

elevated Hct w/o elevated WBC or platelets

(causes: smoking, COPD, CHF, high altitudes)

also: Hct may be high d/t dehydration, diuretics

Front 178

Tx polycythemia vera

Back 178

phlebotomy until Hct below 45%

(if can't, myelosuppression via hydroxyurea)

don't Tx the iron deficiency

Front 179

essential thrombocytosis: pt demographic

Back 179

50-60 y/o;

slightly more common in women

Front 180

S/Sx essential thrombocytosis

Back 180

asymptomatic;

thrombosis at unusual sites

(mesenteric, hepatic, portal veins)

may have mucosal bleeding.

erythromelagia (Tx w/ ASA),

HA, dizziness, chest pain,

weakness, vision changes

Front 181

Dx essential thrombocytosis

Back 181

elevated platelet count

(may be above 2 million)

WBC elevated, but below 30,000;

normal Hct & RBC morphology;

make sure Philadelphia chrom. is ABSENT

Front 182

causes of secondary thrombocytosis?

Back 182

inflammation (RA, UC),

surgery (inflm state),

hypo- or asplenism

(poor platelet breakdown)

Front 183

Tx essential thrombocytosis

Back 183

reduce platelets to under 500;

daily low dose ASA

Tx of choice = hydroxyurea

Front 184

patho: primary myelofibrosis

Back 184

bone marrow fibrosis,

likely d/t increased PDGF

(platelet derived growth factor)

leads to extramedullary hematopoiesis

in liver, spleen, lymph nodes

Front 185

primary myelofibrosis: pt demographic

Back 185

usually older than 50 y/o;

JAK 2 mutation

Front 186

S/Sx primary myelofibrosis

Back 186

insidious onset

fatigue (anemia),

abd fullness (splenomegaly),

thrombocytopenia (bleeding),

bone pain (esp. upper legs),

portal HTN (varices, ascites, etc)

Front 187

Dx primary myelofibrosis

Back 187

teardrop poikilocytosis,

WBC/RBC blastic blood,

giant abnl plateletes

bone marrow dry tap;

anemic at presentation

Front 188

Tx primary myelofibrosis

Back 188

mild: supportive w/ transfusion

only cure: allogeneic stem cell transplant

immunomod. therapy (lenalidomide)

for anemia & thrombocytopenia

radiation therapy for pain assoc w/

extramedullary hematopoiesis

Front 189

patho: myelodysplastic syndromes (MDS)

Back 189

group of disorders w/ ineffective production

of myeloid class of blood cells

usually idiopathic; maybe assoc.

w/ prev. chemo/radiation

may progress to AML

Front 190

myelodysplastic syndromes: pt. demographic

Back 190

typically after age 60,

rarely before age 50

in younger pts, assoc. w/ Down syndrome;

slightly more in males

Front 191

S/Sx myelodysplastic syndrome

Back 191

asymptomatic;

anemia (fatigue, SOB, chest pain),

neutropenia (infxns),

thrombocytopenia (bleeding, bruising),

wasting (fever, wt loss),

splenomegaly,

paraneoplastic (psoriasis, vasculitis,

hemolytic anemia, hypothyroid, DI)

Front 192

chronic myelomonocytic leukemia (CMML)

Back 192

subset of myelodysplastic syndrome (MDS)

overlaps w/ myeloproliferative disorders;

may cause splenomegaly, LUQ pain,

early satiety

Front 193

Dx myelodysplastic syndromes

Back 193

macroovalocytes on smear;

abnl erythropoiesis (erythroid hyperplasia);

Prussian blue stain (ringed sideroblasts)

dwarf megakaryocytes w/ unilobed nucleus;

may involve chrom 5 (5q syndrome);

monosomy 7 = aggressive disease

Front 194

myelodysplastic syndromes: DDX

Back 194

Dx of exclusion!

must r/o:

megaloblastic anemia, aplastic anemia,

myelofibrosis, HIV, drug effect

^ get bone marrow eval;

r/o AML (less than 20% blasts)

Front 195

Tx myelodysplastic syndrome

Back 195

depends on type;

anemia (EPO, transfusions PRN),

lenalidomide, iron chelation (deferasirox),

myeloid growth factor

allogeneic stem cell transplant (curative)

Front 196

most common causes of death in pts

w/ myelodysplastic syndrome?

Back 196

infection, bleeding

(pts w/ excess blasts or CMML

= higher risk of progression to AML)

Front 197

patho: chronic myeloid leukemia (CML)

Back 197

unregulated proliferation of granulocytes

(neutrophils, eosinophils, basophils)

Philadelphia chrom

(long arm transloc. of chrom 9 & 22)

aka bcr/abl fusion gene

Front 198

3 phases of chronic myeloid leukemia (CML)

Back 198

chronic: months, yrs; little/no symptoms

accelerated: leukemia cells grow more quickly

blast crisis: bone marrow failure

Front 199

chronic myeloid leukemia: pt demographic

Back 199

median age = 55 years old

Front 200

S/Sx chronic myeloid leukemia (CML)

Back 200

fatigue, night sweats, low fever;

splenomegaly, bone pain;

bleeding, infxn (blast crisis)

+/- leukostasis syndrome

(blurred vision, resp. distress, priapism)

WBCs over 500,000

Front 201

Dx chronic myeloid leukemia (CML)

Back 201

elevated WBC

(avg. 150,000 at Dx)

not anemic, normal morphology;

hypercellular marrow w/ left-shift

(but not necessary for Dx)

PCR for Philadelphia chromosome

Front 202

Tx chronic myeloid leukemia (CML)

Back 202

tyrosine kinase inhibitor

(imatinib (Gleevac) nilotinib, dasatinib)

monitor w/ qualitative PCR

if leukostasis, need leukapheresis & myelosuppressive treatment

Front 203

patho: chronic lymphocytic leukemia (CLL)

Back 203

B lymphocytes slowly proliferate

(don't respond to antigens

or make abs like they should)

higher in Russian/Eastern Euro Jews

median age = 70 y/o

Front 204

S/Sx chronic lymphocytic leukemia (CLL)

Back 204

indolent course;

fatigue, lymphadenopathy,

fever, infxn, wt loss,

splenomegaly, hepatomegaly

Front 205

Staging chronic lymphocytic leukemia (CLL)

Back 205

Rai system

stage 0 - lymphocytosis

stage 1 - & lymphadenopathy

stage 2 - & hepato/spleno-megaly

stage 3 - & anemia

stage 4 - & thrombocytopenia

Front 206

Dx chronic lymphocytic leukemia (CLL)

Back 206

isolated lymphocytosis

peripheral smear: smudge cells

FISH: prognosis

17p or 11q = poor

13q = good

Front 207

Tx chronic lymphocytic leukemia (CLL)

Back 207

fludarabine w/ rituximab

+/- cyclophosphamide

(improves response, but inc. infxn risk)

radiation to dec. organ enlargement;

transplant if refractory

Front 208

patho: acute myeloid leukemia (AML)

Back 208

CA of myeloid cell line;

accumulation of abnl WBCs

most common leukemia in adults;

typically presents after age 65;

more common in males

Front 209

risk factors for acute myeloid leukemia (AML)

Back 209

smoking, benzene exposure,

previous chemo/radiation, MDS,

myeloproliferative disorders,

family history

Front 210

acute promyelocytic leukemia (APL)

Back 210

subtype of acute myeloid leukemia (AML);

more likely to have bleeding/clotting issues

(epistaxis, DVT, PE, DIC)

Tx w/ anthracycline + AT-retinoic acid (ATRA)

Front 211

Dx acute myeloid leukemia (AML)

Back 211

peripheral smear: blast cells

(more than 20% blasts)

Auer rods

stains pos. for myeloperoxidase, Sudan black

Front 212

Tx acute myeloid leukemia (AML)

Back 212

anthracycline (daunorubicin) + cytarabine

post-remission: chemo, transplant (curative)

Front 213

patho: acute lymphocytic leukemia (ALL)

Back 213

proliferation of immature lymphocytes;

most common leukemia in children,

(peaks between 3-7 y/o)

rarest type in adults

Front 214

risk factors: acute lymphocytic leukemia (ALL)

Back 214

radiation/chemo, immunosuppression,

benzenes, some viral infxn, Down's,

Klinefelter, whites, sibling w/ ALL

Front 215

Dx acute lymphocytic leukemia (ALL)

Back 215

stain for TdT

(terminal deoxynucleotidyl transferase)

leukocytosis, anemia, thrombocytopenia;

marrow = excess blasts (over 20%)

Front 216

Tx acute lymphocytic leukemia (ALL)

Back 216

chemo (vincristine, prednisone,

anthracycline, cyclophosphamide)

post-remission chemo, transplant

Front 217

patho: multiple myeloma

Back 217

proliferation of abnl plasma cells;

increased osteoclast activity

heavy chain abs (IgG, IgA, IgM, IgD)

median age = 65;

more in men, AA

Front 218

S/Sx multiple myeloma

Back 218

asymptomatic

anemia, infxn, bone lesions,

bone pain (back, hips, ribs),

pathologic fx (esp. femoral neck, vertebrae),

hypercalcemia

Front 219

Dx multiple myeloma

Back 219

normal RBC morphology,

but rouleaux formation common

serum protein electrophoresis (SPEP)

^ M spike

urine: Bence Jones protein

Front 220

Tx multiple myeloma

Back 220

thalidomide, lenalidomide

w/ dexamethasone (or prednisone)

+/- Bortezomib,

bisphosphonates

Front 221

Dx non-Hodgkins lymphoma

Back 221

requires lymph node biopsy

serum LDH: prognostic marker

stage w/ PET/CT, marrow biopsy, LP

Front 222

Ann Arbor staging

Back 222

for non-Hodgkins lymphoma

Stage I: 1 node

Stage II: 2 nodes on same side of diaphragm

Stage III: nodes on both sides of diaphragm

Stage IV: outside of lymph system

(e.g. marrow, liver, brain, spinal cord, pleura)

Front 223

Tx non-Hodgkins lymphoma

Back 223

rituximab +/- chemo (R-CHOP)

if intermediate/aggressive:

R-CHOP +/- radiation

early transplant

Front 224

Hodgkin lymphoma: pt demographic

Back 224

dual peak: in 20s, over age 50

slightly more common in males

pts w/ past EBV infxn more susceptible

Front 225

painful lymph node after alcohol consumption?

Back 225

Hodgkin lymphoma

Front 226

Dx Hodgkin's lymphoma

Back 226

lymph node biopsy: Reed Sternberg cells

Ann Arbor staging

via PET/CT & bone marrow biopsy

Front 227

Tx Hodgkin's lymphoma

Back 227

chemo: ABVD

(doxorubicin, bleomycin,

vinblastine, dacarbazine)

+/- radiation, stem cell transplant

Front 228

Traditional chemo kills cells that divide rapidly. This includes CA cells, but also...

Back 228

bone marrow, GI tract, hair follicles

Front 229

stages of chemotherapy

Back 229

induction: kills most CA cells in blood, marrow

consolidation: kills leftover CA cells

maintenance: low dose x months-years

Front 230

side effects of chemo?

Back 230

hair loss, mouth sores,

loss of appetite, N/V, diarrhea;

possible longterm:

fertility problems, develop. of leukemia

Front 231

ADR: doxorubicin?

Back 231

cardiac damage

Front 232

ADR: bleomycin

Back 232

pulmonary toxicity

Front 233

ADR: lenalidomide, vincristine, bortezomib

Back 233

neuropathy

Front 234

ADR: cyclophosphamide

Back 234

hemorrhagic cystitis

Front 235

tumor lysis syndrome

Back 235

most common in 1st chemo cycle

S/Sx: high K+, phosphate, uric acid;

low Ca++; lactic acidosis, renal failure

prevention: excess IVF, allopurinol, rasburicase

Front 236

graft vs. host prophylaxis

Back 236

cyclosporine,

tacrolimus,

methotrexate

Front 237

anthracyclines: used in...

Back 237

non-Hodgkins, Hodgkins,

AML, ALL

(e.g. daunorubicin, idarubicin, doxorubicin)

Front 238

lenalidomide & thalidomide: used in...

Back 238

multiple myeloma, myelofibrosis, MDS