first line treatment of IDA

when should the Hct return to normal

ferrous sulfate 325 mg TID - on an empty stomach or with acidic juice

Hct should return to normal within 2 months - continue treatment 3 - 6 months after Hct has returned to normal

intolerance to oral iron

refactory disease (to the oral Fe)

GI disease or previous surgery

sodium ferric gluconate (ferrlecit)

IV dose 1.5 - 2 

given over 4 - 6 hours

serum ferritin normal or increased

underlying chronic disease

(MCV usually normal; Hct mildly or moderately reduced)

none usually needed

EPO for renal failure or cancer or IBD (but reserved for pts dependent on transfusions)

EPO

typically reserved for renal failure pts dependent on transfusions

alpha thalassemia trait = 2 alpha globin genes/chains

beta thalassemia minor = heterozygous

no treatment necessary

Hgb H dz = alpha thalassemia with only one alpha globin gene/chain

folate supplements

avoid iron supplements and oxidative drugs (sulfonamides)

transfusions may be required in times of stress/infection/exacerbation (similar to beta thalassemia intermedia)

typically, beta thalassemia major (homozygous)

regular transfusion schedule

folate supplements

if hypersplenism -> splenectomy

deferoxamine (chelates iron)

allogeneic bone marrow transplantation

bone marrow biopsy

showing ringed sideroblasts

(and marked eryhtroid hyperplasia)

occasionally requires transfusion

does NOT respond to EPO therapy

low serum Vit B12 < 100 ng/L (normal 160 - 200)

(PBS -> macro-ovalocytes and hypersegmented neutrophils w/mean lobe count > 4 or 1 six lobed neutrophil)

after Vit B12, 100 mcg IM for 1 week, then ever week for 1 month, and then every month for life...

hematologic picture normalizes in 2 months

RBC level of folic acid < 150 ng/mL

(PBS -> macro-ovalocytes and hypersegmented neutrophils w/mean lobe count > 4 or 1 six lobed neutrophil)

treatment for folic acid deficiency

with total correction of hematologic abnormalities within how many months?

folic acid, 1 mg PO daily

total correction of hematologic abnormalities within 2 months

Vit B12 deficiency

folic acid deficiency

reticulocytosis ALWAYS present

hyperchromic RBCs

increased osmotic fragility

(coombs negative (b/c of an intrinsic problem), PBS shows spherocytosis)

folic acid, 1 mg PO daily

splenectomy - eliminates site of hemolysis, but doesn't correct the membrane defect; may not be necessary in very  mild cases discovered late in adult life

folic acid, 1 mg PO daily

don't necessarily need a splenectomy if the case is mild enough

G6PD enzyme assay negative or < 12 IU/g

(PBS -> heinz bodies (oxidized Hgb percipitants) with crystal violet stain; RBC "bite" cells)

typically, no treatment necessary - self limiting; if severe - may require transfusion

avoid oxidative triggers

few will have chronic hemolytic anemia

no treatment necessary

genetic counseling is appropriate

hemoglobin electrophoresis -> homozygous S 95 - 97%

 Hgb F 2 - 5% (the higher the level, the mor benign the course)

NO Hgb A

identify precipitating factors

treat infections if present

aggressive analgesia administration

vigorous hydration

administer O2 only if hypoxic

transfusion for extreme cases (but doesn't shorten the crisis)

*CP, tachypnea, fever, cough, arterial O2 desat - reflecting insitu sickling w/in lung)*

medical emergency - treat in ICU

O2 if sat <90%

Maintain Hct > 30

splenectomy if prednisone ineffective or recurs

rituximab - if refractory to prednisone and splenectomy

immunosuppressive agents

high dose immune globulin - effective, but short lived

Coombs test positive for complement ONLY

(positive cold agglutinin test)

rituximab - relapses can  also be re-treated

avoid the cold

high dose immune globulin - effective, but short lived

immunosuppressive agents - for severe disease

supportive measures only for mild cases

antibiotics to treat infections

RBC and platelet transfusions as necessary

treatment of infection leads to correction of platelet count in most cases

can use IVIG/anti-D, interferon-alpha

corticosteroids are NOT recommended for pts with Hep C

treatment for relapsed/persistent ITP

(pt still has platelets < 30,000 or still bleeding)

corticosteroids

AND

IVIG or anti-D or rituximab

consider splenectomy

treatment for worsening/persistent/refractory ITP

(have already tried both first and second line drugs)

have already tried corticosteroids AND IVIG

consider additional agent OR chemo OR laparoscopic splenectomy

splenectomy is the definitive tx of ITP

reserved for severe thrombocytopenia pts who did not respond to initial prednisone or unacceptably high dose is required to maintain adequate platelet count, or pt prefers surgery

what are some laboratory findings relative to thrombotic thrombocytopenic purpura (TTP)

(these aren't necessarily diagnostic, just what was bolded from lecture)

PBS - showing microangiopathic changes with fragmented RBCs (shistocytes)

LDH markedly elevated

coag tests = normal

Decreased activity of ADAMTS-13 (von willebrand factor cleaving protease)

immunosuppression (cyclophosphamide, rituximab)

splenectomy for refractory

laboratory findings for hemolytic uremic syndrome (HUS)

(just bolded points from lecture, not necessarily diagnositc)

PMS with significant schistocytes

LDH elevated, out of proportion to degree of hemolysis

positive stool culture - determines causative agent (typically E. coli)

serum fibrinogen low

thrombocytopenia

progressive thrombocytopenia

PT and PTT prolonged

HELLP findings in pregnancy

transfuse blood products only if bleeding or high risk of bleeding

cryoprecipitate to replete fibrinogen

FFP to raise PT/aPTT values

PRBC to replete Hgb

if persistent bleeding - consider heparin (NO bolus)

low VWF activity level

lower factor VIII level

total plasma VWF antigen is decreased

thrombocytopenia

what is the first line treatment for von willebrand's disease - and what needs to be restricted during treatment

what is the max dosing of this treatment

DDAVP via IV or by high [  ] nasal spray

fluid restriction should be advised for all pts for the 24 hours following each dose

more than TWO doses in 48 hours is NOT recommended

DDAVP is not effective in VWF-containing factor VIII

cryoprecipitate is NOT recommended to treat von willebrand's disease

isolated reproducibly low factor VIII and factor IX activity level in the absence of other conditions

prolonged aPTT - which corrects upon mixing w/normal plasma

other coag tests are normal

plasma-derived or recombinant factor concentrates

severe pts are typically receiving long term prophylactic infusions of factor by the age of 4

generally don't use intense long term prophy

factor concentrates as needed for bleeding episodes or prior to high risk activities

*Factor V Leiden*

avoid medications that make pt hypercoagulable

consider anticoagulation prior to surgical procedures

anticoagulation for emoblic events

if no hisotry of embolus - no prophy

chemotherapy to induce remission:

anthracycline + cytrarbine

postremission therapy:

chemo

autologous transplantation

allogeneic thransplantation

to induce remission:

combination chemotherapy

to cure:

chemotherapy (but not enough for high risk pts when done alone)

autologous transplantation

allogeneic transplantation

chemotherapy

autologous transplantation

allogeneic transplantation

philadelphia chromosome, aka bcr/abl genee detected via cytogenic study t(9,22)

(WBCs > 100,000, hypercellular bone marrow w/ <5% blasts, mature myeloid cells on PBS)

dz is typically a proliferation of mosly B lymphocytes, therefore:

monoclonal B-cells.

isolated & persistent lymphocytosis = hallmark; they are typically small & mature

usually not emergent

tx of choice: Imatinib mesylate (inhibits tyrosine kinase activity of bcr/abl oncogene)

allogeneic stem cell transplant

best results if pt < 40 yrs and transplanted w/in 1 year from an HLA-matched sibling

fludarabine IV dialy for 5 days each month for 4 - 6 months

causes immunosuppression

best when combined with rituximab

cladribine

0.14 mg/kg dialy for 7 days

pathologic diagnosis by lymph node biopsy, seeing

Reed-Sternberg cells

(usually arises within a single lymph node area and spreads contiguously)

(A: asymptomatic pt)

radiation therapy +/- short course chemotherapy

(B: + constitutional symptoms of fever, night sweats, weight loss)

combination chemotherapy with ABVD or never regimen

adriamycin/doxorubicin

bleomycin

vincristine

decarbazine

tissue biopsy

(LDH is a useful prognostic marker, CXR may show mediastinal mass, bone marrow shows paratrabecular lymphoid aggregates)

rituximab +/- chemotherapy (CHOP-R)

allogeneic transplant

chemotherapy with CHOP-R

autologous stem cell transplant

hallmark = paraprotein on serum protein electrophoresis

(rouleau formation of RBCs is common on PBS)

thalidomide + dexamethasone

valcade

revlimid being investigated

bone biopsy culture is required for the diagnosis, unless blood cultures come back positive (ie. don't need bone biopsy, if the osteomyelitis was from a hematogenous spread and the bacteremia gave a positive blood culture)

(*cultures from overlying ulcers, wounds, or fistulas are unreliable)

antibiotics tailored to C & S - high doses IV for atleast 4 - 6 weeks

early surgical debridement and drainage

synovial fluid is turbid, serosanguineous or frankly purulent

leukocyte count in synovial fluid > 50,000 - 100,000 w/> 90% polys

gram stain positive in 75% of staph, and in 50% of G- orgs

positive cultures > 90%

elevated protein

elevated LDH

prompt IV antibiotic that covers staph, strep, and G-'s:

third generation cephalosporin, ie:

ceftriaxone, cefotaxime, or ceftizoxime

immediate ortho consult for timely drainage pf pus and necrotic debris from joint

avoid weight bearing until inflamm has subsided

early PROM exercises to maintain mobility

prompt IV therapy with vancomycin

immediate ortho consult for timely drainage of pus and necrotic debris from joint

avoid weight bearing until inflamm has subsided

early PROM exercises to maintain mobility

initial treatment: ceftriaxone (recephin) 1 g IV daily

OR

cefotaxime 1 g IV q 8 hrs

after 24 - 48 hours, and pt improvement: switch to an oral 7 - 10 day course of

oral ciprofloxacin, 500 mg BID

OR

oral cefixime, 400 mg BID

*REPORT to health department*

NSAIDs

if pt has Hep C arthritis - symptoms 2ndary to Hep C may respond to interferon-alpha

doxycycline

OR

amoxicillin

drainage and lavage of joint

IV antifungal agents

intra-articular antifungal agents

narrowing of the joint space

osteophyte formation

lipping or marginal bone

thickened, dense subchondral bone

bone cysts

weight reduction - esp reduces risk for knee OA

maintain normal Vit D levels - reduces occurence and progression

what is the non-medical treatment for osteoarthritis

(aka - pt has mild to moderate OA of weight bearing joints)

moderate physcial activity:

therapeutic walking program

hydrotherapy classes

tai chi

weight loss

acetaminphen

pts should not use NSAIDs unless they aren't responding to acetaminophen - avoid high doses

effective treatment: total hip and knee replacement

not effective: arhtroscopic surgery for the knee

identification of sodium urate crystals in joint fluid or material aspirated from a tophus

OR

when a "rate bite" (punched out erosion w/overhanging rim or cortical bone) is adjacent to a soft tissue tophus

urate crystals that are needle-like

and

negatively birefringe

when examined by polarized light microscopy

*treat arthritis first, hyperuricemia later*

NSAIDs - indomethacin 25 - 50 mg q 8hrs PO, until symptoms have resolved (5 - 10 days)

celecoxib, 200mg BID day1, then 100mg BID until resolution

OR

corticosteroids - IV or oral, tapered over 7 - 10 days

if monoarthritis, can use intra-articular

colchicine - 0.6 mg QD or BID

if uncontrolled by colchicine prophy:

uricosuric drug (probenacid or sulfinpyrazone) or

xanthine oxidase inhibitor (allopurinol or febuxostat)

uricosuric drug:

probenacid

OR

or sulfinpyrazone

xanthine oxidase inhibitor:

allopurinol

OR

febuxostat

joint aspiration and NSAIDs, or intra-articular steroids

resolution normally < 10 days

treatment of frequent recurrent attacks of CPPD/pseudogout

or when it is severe polyarticular attacks

(these are two different questions, was lazy and didn't want to make two cards)

frequenct recurrent: daily prophylactic colchicine

severe polyartiuclar: short course glucocorticoids or anakinra (IL-1B antagonist)

what are typical lab/image findings for osteoporosis

(not necessarily diagnostic)

serum Ca, phos, and PTH = NORMAL

alk phos = normal to elevated (elevated esp after a fx)

Vit D deficiency

T score > -1 : Normal

T score -1.0 - -2.5 : osteopenia (low bone density)

T score < -2.5 : osteoporosis

T score < -2.5 w/a fx : severe osteoporosis

ways to prevent osteoporosis

(this is not all inclusive)

adequate protein, total calories, Ca and Vit D

d/c corticosteroid use if poss

if hypercalciuric - take thiazides

stair climbing

weight bearing exercises

all women w/ T score below -2.5 (ie. osteoporosis)

all who have had fragility fx's

as prophy in advanced osteopenia (T score -2.0 - -2.5)

Vit D and Ca - give Ca only for pt w/low Ca diet

bisphosphonates

HRT (women = estrogen; men = testosterone, don't take give if pt has prostate CA)

SERMS: teriparatide

calcitonin

denosumab

denosumab

it decreases Ca levels, therefore do not use in a pt/HYPOcalcemia

history of back pain at night that is unrelieved by rest leads you to what diagnosis?

back pain that is worse with rest and improves with activity

worse with activity, and improves with rest

writhing back pain

pain at night: malignancy or compression fx

worse with rest: inflammatory:ankylosis spondylitis

worse with activity: degenerative arthritis or disk disease

writhing: kidney or gallstone, rupturing aneurysm

bilateral leg weakness (mult lumbar n root compression, epidural abscess)

saddle anesthesia, bowel/bladder incontinence, impotence (cauda equina syndrome)

pain most prominent w/less prominent numbness (herniated disk involving single root, epidural abscess)

positive if radicular pain is reproduced at 60% or less and indicated herniated disk at L4-5 or L5-S1

crossed straight leg: raise contralateral leg, if pain is reproduced on affected side = positive and supports above findings

when you have a high suspicion of:

abscess: bilat leg weakness; pain worse than amount numbness; incontinence

osteomyelitis: spinous process tender to palpation

cadua equina syndrome: incontinence, impotence

spinal stenosis

herniated disk: pain is worse than amount of numbness

mets

*< 4 weeks, 4 - 12 weeks*

remain active, NO ROLE FOR BED REST

avoid heavy lifting (>10 lbs), prolonged sitting, twisting/bending - until symptoms resolve

back exercises to minimise recurrent flares

NSAIDs 1 -2 weeks, and then PRN

muscle relaxants is spasm for first 1 - 2 weeks

for acute/subacute back pain, what would you give/do:

for pt with muscle spasms

to temporarily improve sciatica

for pt with serious symptoms

spasms: muscle relaxants for first 1 - 2 weeks

sciatica: steroid injection

serious symptoms: decompressive surgery

treatment of chronic back pain due to degenerative conditions:

spinal stenosis

facet joint hypertrophy

spinal stenosis: NSAIDs, exercise, pain management, surgery is not resolved

facet joint: surgical foraminotomy

NSAIDs and rest (rest may include splinting)

if refractory: consider PT

treat  underlying cause (RA, thyroid, Ca deposits, gout DM)

drain bursa - to send off for infection/gout

if due to overuse:

inject steroid

NSAIDs (try to avoid opioids)

compressive wrap

shoulder pain occurs with overhead motion

pt lacks full ACTIVE ROM but has full PASSIVE ROM w/pain

positive neer's sign

positive hawkin's sign

impingement syndrome

OR

rotator cuff tear

impingement syndrome

OR

rotator cuff tear

first line treatment for impingement syndrome of the shoulder

what is given for short term relief of impingement syndrome

if above two are not effective

first line:

activity modification (stop what they are doing)

physical therapy

NSAIDs

short term relief:

corticosteroid injection

above not effective:

arthroscopic acromioplasty

bursectomy (acromioplasty typically done before bursectomy)

debridement/repair of rotator cuff tears

MRI

(US is good for full tears, but not good at detecting partial rotator cuff tears)

PT strengthening

NSAIDs - pain control

if spasm - muscle relaxant

consider orthopedic referall

automatic orthopedic referral

usually requires surgery it young and healthy and active

if older - typically aren't good surgical candidates, therefore just limit their acitivty and the ROMs

NSAIDs

PT - ROM and stretching

steroid injection into subacromial space or glenohumoral joint

surgical: manipulation, arthroscopic release

lateral epicondylitis

(pain on resisted extension of middle finger)

what physical exam finding will support your suspicion of:

lateral epicondylitis

medial epicondylitis

lateral:

pain on resisted wrist extension

Medial:

pain on resisted forearm pronation

pain on resisted wrist flexion

treatment of epicondylitis

what would give short term relieve

when would you refer to ortho

modification of offending activity

NSAIDs

PT - stretching and strengthening

may require surgical release and repair

short term relief: steroid injection

refer: no improvement after 3 - 6 months, or to give steroid injection

carpal tunnel syndrome

*highest sensitivity/specificity of all specialty exams for carpal tunnel*

avoid repetitive movement of wrist

wrist splinting in neutral position up to 3 months

NSAIDs and oral steroids

injection steroids into carpal tunnel

surgical release of carpal tunnel - endoscopic, minimally invasive

*extensor pollicis brevis and abductor pollicis longus*

NSAIDs and ice

avoid offedning activities

thumb spical brace to rest EPB and APL tendons

steroid injection if initial therapy fails

NSAIDs

cyst aspiration and injection of steroid

surgical excision

1/3 resolve spontaneously

intial treatment of trigger finger

treatment of refractory trigger finger

initial:

NSAIDs and buddy tape finger in extension

steroid injection in tendon sheath

refractory:

surgical release of tendon sheath

aka: bone cysts, osteofibroma

local excision

serum alk phos is markedly elevated

urinary hydroxyproline elevated in active disease

radiographs: advancing flame shaped lytic lesions in long bones, and become subsequently sclerotic, eventually becoming thickened and deformed

bisphosphonates given cyclically, when alk phos normalized give a 3 month break until serum alk phos starts to rise again and then do another cycle

correct ant Vit D if present BEFORE prescribing bisphosphonates

osteosarcoma

chondrosarcoma

ewig's sarcoma

malignant fibrosis histiocytoma

enchondroma

osteochondroma

fibrous dysplasia (bone cyst/osteofibroma)

bone infarct's

paget's disease of the bone

destructive lesion w/moth eaten appearance, sunburst appearance and a cuff of new bone formation at the margin of the soft tissue mass

*likes metaphyses of long bones*

preop chemo

followed by limb-sparing surgery

followed by postop chemo

*RADIORESISTANT - XRT has NO ROLE in management*

influenced by H&P

new onset of pain, signs of inflammation, progressive increase in size of mass all suggest malignancy 

characteristic "onion peel" periosteal reaction w/generous soft tissue mass

mass is better seen with CT or MRI

*very aggressive*

systemic chemo, often preop

surgical resection usually w/limb sparing or radiation