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260 Cards in this Set
- Front
- Back
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negative antihitone antibodis
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excludes lupus
|
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ANA elevated in...
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SLE
RA Scleroderma Sjogren's MCTD Polymyositis Dermatomyositis Drug induced lupus |
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sensitive tests for lupus
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ANA 100%
antissDNA 70% Antihistone 70% |
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specific tests for lupus
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Anti dsDNA
Anti-smith |
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levels of complement in lupus
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decreased
|
|
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drugs that induce DILupus
|
hydralazine
procainabmide isoniazid chlorpromazine mythyldopa quinidine |
|
|
recurrent venous thrombosis
recurrent arterial thrombosis recurrent fetal loss thrombocytopenia livedo reticularis |
antiphospholipid antibody syndrome
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lupus anticoagulant and or anticardiolipin antibody
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antiphospholipid antibody syndrome
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type II lupus GN
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mesangial
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type III llupus GN
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focal proliferative
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type IV lupus GN
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diffuse proliferative
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type V lupus GN
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membranous
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lupus GNs that can cause renal failure
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IV - diffuse proliferative, only
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cANCA
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Wegerners
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pANCA
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PAN
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ESR
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infection
malignancy rheumatological disease tissue necrosis pregnancy |
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CRP
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inflammatory states and infection
MI vasculitis trauma malignancy pancreatitis |
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anti ds DNA
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SLE
also Sjogrens |
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antihistones
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drug induced lupus
(also lupus) |
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anti URP
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Mixed Connective Tissue disease (90%)
also SLE, scleroderma, Dermatomyositis, polymyositis, CREST |
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Anti Smith
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SLE
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Anti Ro
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Sjogrens
also SLE |
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Anti leucine aminopeptidase
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Sjogrens
also SLE |
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antiscleroderma-0
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scleroderma (20)
CREST (10%) |
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anticentromere
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CREST
also scleroderma (limitied form) |
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DR 2
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SLE
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DR 2 and 3
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SLE
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DR3
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SLE, sjogrens
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DR 4
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RA
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B27
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Ankyosing spondylitis
Rieters Psoriatic arthritis |
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antitopoisomerase ab
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diffuse (vs. limited, i.e., CREST) scleroderma
|
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limited scleroderma =
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CREST
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Sjogrens increases risk of
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NHL
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Anti La (SS-B)
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sjogens
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tx for sjogrens
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pilocarpine
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pleural fluid with very low glucose
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RA
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calcinosis of digits
raynauds esophageal dysmotility sclerodactyly of fingers telangiectasias over digits, under nails |
CREST
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synovial fluid >5000
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inflammatory arthritis (gout, pseudogout, Reiters, RA)
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synovial fluid >50,000
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septic arthritis (bacterial, TB)
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positively birefringent
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Pseudogout
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needle shaped crystals
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gout
also negatively birefringent |
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osteophytes
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Osteoarthritis
osteophytes osetoarthritis |
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anemia
neutropenia splenomegaly RA |
Felty's
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RA before 18
extra-articular manifestations predominate |
Stills
|
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RA tx
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symptoms - NSAIDS, long term low dose steroids
Disease modifying - methotrexate, hydroxychloroquine, sulfasalazine |
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serum uric acid level
|
not helpful in diagnosing gout because it can be normal even during an acute gouty attach
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medications causing gout
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thiazides
loops |
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acute gout tx
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NSAIDS
colchicine steroids |
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gout prophylaxis
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3-6 months of NSAIDS or colchicine
then probenecid or allopurinol |
|
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effect of allopurinol on acute gout
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makes it worse
|
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calcium pyrophosphate crustals
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pseudogout
|
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rod shaped crystals
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pseudogout
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punched out erosions with overlying rim of cortical bone on CSR
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gouty arthritis
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joint pain
nephrolithiasis |
gouty arthritis
|
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child
subcutaneous calcifications |
dermatomyositis
|
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symmetric proximal muscle weakness
elevetaed creatine phosphokinase EMG findings of myopathy biopsy evidence of syositis rash |
Polymyositis
|
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heliotrope rash
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dermatomyositis
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symmetrical proximal muscle weakness
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polymyositis
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gottrons papules (papular erythematous, scaly lesions over knucles (MCP, PIP, DIP)
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dermatomyositis
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rash on face, neck, anterior chest
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dermatomyositis
|
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rash on shoulders, upper back, elbows, knees
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shawl sign for dermatomyositis
|
|
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Anti signal recognition particle (SRP)
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cardiac polymyositis
bad prognosis |
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|
old man
insidious onset of slowly progressive, asymmetric, proximal and distal weakness decreased DTRs |
inclusion body myositis
|
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increasing muscle weakness
decreased DTRs |
inclusion body myositis
|
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abrupt onset hip and shoulder muscle pain
stiffness after inactivity, esp morning pain on movement constitutionall symptoms sometimes joint swelling ESR increased |
polymyalgia rheumatica
|
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tx for polymyalgia rheumatica
|
corticosteroids
|
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symmetrical pain to palpation trigger points
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fibromyalgia
|
|
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tx for fibromyalgia
|
stay active
SSRIs and TCAs can help no real Rx tx |
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ascending spine stiffness
|
ankylosing spondylitis
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enthesitis - inflammation at tendinous insertion sites
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ankylosing spondylitis
|
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tx for ankylosing spondylitis
|
indomethacin
|
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bugs for reactive arthritis (e.g., Reiters)
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salmonella
shigella campylobacter Chlamydia Yersinia |
|
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tx for Reiters
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NSAIDS
if no good, sulfasalazine, azathioprine |
|
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polyarticular or monoarticular?
reiters septic arthritis |
reiters is poly
septic is mono |
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40% of temporal arteritis atients have...
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polymyalgia
|
|
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age >50
new headache high ESR jaw claudication |
temporal arteritis
|
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pANCA
|
Churg Strauss
PAN |
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recurrent oral and genital ulcerations
arthritis eye involvement meningoencephalitis fever weight loss |
Behcets
|
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Behcet's tx
|
steroids
|
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ischemic cludication
cold, cyanotic painful distal extremities paresthesias of distal extremitis ulceration of digits |
buerger's disease
young male smoker |
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anemia shifts O2 dissociation curve
|
right
|
|
|
each unit of PrBCs should increase Hct by
|
3-4
|
|
|
why can't you mix PRBC with ringers
|
Ca++ causes coagulation
so use normal saline |
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what does FFP have? lack?
|
has all clotting factors
lacks RBC, WBC, Plt |
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effect of giving vitamin K during liver failure
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nada
|
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what does cryoprecipitate have?
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VIII and fibrinogen
|
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retic index >2%
|
excessive RBC destruction or loss
lesst than 2% is an inadequate response by marrow |
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transfusion given
fever, chills, N/V pain in flanks/back chest pain dyspnea |
Intravascular hemolysis
usually due to mismatch |
|
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C9 punching hols through RBC membranes
|
intravascular hemolysis post transfusion
|
|
|
reaction 3-4 weeks after transfusion
fever jaundice anemia |
extravascular hemolysis
caused by a minor RBC anticen, e.g., Kell |
|
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antigen often to blame in extravascular hemolysis 3-4 weeks post transfusion
|
Kell
|
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Fe and TIBC decreased
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anemia of chronic disease
|
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FE decreased
TIBC increased |
iron deficiency anemia
|
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Fe normal
TIBC normal or ecreased microcytosis |
lead poisoning
thalassemia |
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macrocytosis
normal B12 and folate |
liver disease
|
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idexes for hemolytic anemia
|
haptoglobin
LDH bilirubin |
|
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increased ferritin
increased iron normal TIBC |
sideroblastic anemia
|
|
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normal alpha chains
decreased beta chains |
beta thalassemia
|
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mechanism of trouble in beta thalassemia
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excess alpha chains bind to and damage RBC membrane (beta chain production deficient)
|
|
|
mechanism of trouble in alpha thalassemia
|
beta chains form tetramers
severity depends on number of gene loci dleted/mutated, and ranges from asymptomatic to prenatal death |
|
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severe anemia (microcytic, hypochromic)
massive hepatosplenomegaly elevated Hb F |
beta thalassemia major (Cooley's anemia, homozygous beta thal)
|
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tx for beta thal major
|
frequent PRBC
|
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mild microcytic hypochromic anemia
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beta thalassemia minor
or alpha thalassemia train |
|
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diagnosis of beta thalassemia
|
hemoglobin electrophoresis (HbF elevated in major)
|
|
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mild microcytic hypochromic anemia
Hb H on electrophoresis |
alpha thalassemia - HbH disease
requires mutation of 3 loci |
|
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increased serum iron and ferritin
normal TIBC ringed blasts in bone marrow |
sideroblastic anemia
|
|
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cause of sideroblastic anemia
|
inherited or
acquired: chloramphenicol, INH, alcohol, lead collagen vascular disease neoplastic disease like MDS |
|
|
tx of sideroblastic anemia
|
remove offending agent
consider pyridoxine |
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medications causing aplastic anemia
|
chloramphenicol
sulfonamides gold carbamazepine |
|
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viral infections causing aplastic anemia
|
parvo
hep C, B EBV CMV zoster HIV |
|
|
fatigue, dyspnea
petechiae, easy bruising increased infections |
aplastic anemia
|
|
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role of B12
|
cofactor in conversions of:
homocystein to methionin methylmalonyl CoA to succinyl CoA |
|
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anemia caused by lack of intrinsic factor
|
pernicious
|
|
|
stomatitis and glossitis
loss of positions/vibratory sense ataxia increased DTRs Babinski |
B12 deficiency
|
|
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tx for B12 deficiency
|
B12 IM once per month
|
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increasd homocystein and methylmalonic acid
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B12 or folate deficiency (differentiate by neuro symptoms)
|
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|
dark urine color due to hemoglobinuria indicates
|
intravascular hemolysis
|
|
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anemia
underlying disease jaundice dark urine hepatosplenomegaly cholelithiasis lymphadenopathy |
chronic hemolytic anemia
|
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|
schistocytes and hellmet cells
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hemolytic anemia due to:
TTP DIC prosthetic heart valves |
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schistocytes
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intravascular hemolysis
|
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spherocytes or helmet cells
|
extravascular hemolysis
|
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heinz bodies
|
G6PD deficiency
|
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low haptoblobin
|
hemolytic anemieas, especially intravascular
(if absent, it means hemoglobin was destroyed) |
|
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elevated LDH
|
hemolysis
|
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type of hyperbilirubinemia in RBC hemolysis
|
indirect
|
|
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direct coombs positive
|
autoimmune hemolytic anemia
(detects antibody or complement on RBC membrane) |
|
|
HbS vs Hb A
|
HbS has glu to val at 6th position of beta chain
(A is normal) |
|
|
painful swelling of dorsa of hands and feet in infancy and early childhood
|
often first sign of sickle cell
|
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cause of sickle crises
|
vaso-occulsion
|
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renal disease in sicklers
|
renal papillary necrosis with hematuria
|
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common site of ulcers in sicklers
|
lateral malleoli
|
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bacteria to which sicklers vulnerable
|
hemophilus
strep pneumo salmonella osteomyolitis |
|
|
diagnosis of sickle cell
|
smear
hemoglobin electrophoresis |
|
|
management of sickle crisis
|
hydration
morphine warmth )2 if needed |
|
|
how does hydroxyurea work in sicklers
|
increases Hb F levels, which interferes with sickling
|
|
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causes of spherocytosis
|
hereditary spherocytosis
G6PD ABO incompatiblity (not Rh) hyperthermia Autoimmune hemolytic anemia |
|
|
inheritance patter of spherocytoisis
|
AD
|
|
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painful swelling of dorsa of hands and feet in infancy and early childhood
|
often first sign of sickle cell
|
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cause of sickle crises
|
vaso-occulsion
|
|
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renal disease in sicklers
|
renal papillary necrosis with hematuria
|
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common site of ulcers in sicklers
|
lateral malleoli
|
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bacteria to which sicklers vulnerable
|
hemophilus
strep pneumo salmonella osteomyolitis |
|
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diagnosis of sickle cell
|
smear
hemoglobin electrophoresis |
|
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management of sickle crisis
|
hydration
morphine warmth )2 if needed |
|
|
how does hydroxyurea work in sicklers
|
increases Hb F levels, which interferes with sickling
|
|
|
causes of spherocytosis
|
hereditary spherocytosis
G6PD ABO incompatiblity (not Rh) hyperthermia Autoimmune hemolytic anemia |
|
|
inheritance patter of spherocytoisis
|
AD
|
|
|
hemolytic anemia
jaundice splenomegaly gallstones occasional crises direct coombs negative spherocytes on smear |
hereditary spherocytosis
|
|
|
cause of hereditary spherocytosis
|
defective spectrin
|
|
|
diagnosis of hereditary spherocytosis
|
osmotic fragility test
spherocyts on smear direct coombs negative |
|
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direct coombs positive
|
autoimmune hemolytic anemia
|
|
|
tx for herediatry spherocytosis
|
splenectomy
|
|
|
inheritance patter of G6PD
|
X
GxPD |
|
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tx for G6PD
|
hydration
transfusion trigger avoidance |
|
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tx for warm autoimmune hemolytic anemia
|
steroids
|
|
|
bite cells
heinz bodies |
G6PD
|
|
|
what is relationship between bite cells and heinz bodies
|
RBC-heinz=bite
|
|
|
warm vs cold autoimmune hemolytic anemia
|
IgG vs M
extravascular (spherocytes) vs. intra sequestration in spleen vs. liver causes of warm: Lymphoma, leukemia, SLE, methyldopa causes of cold: mycoplasma/EBV |
|
|
causes of warm autoimmune hemolytic anemia
|
lymphoma
leukemia SLe alpha methyldopa |
|
|
causes of cold autoimmune hemolytic anemia
|
mycoplasma
EBV |
|
|
which autoimmune hemolytic anemia has spherocytes
|
warm
|
|
|
tx for warm vs cold autoimmune hemolytic anemia
|
often none needed in both, but
Warm: steroids mainly, splenectomy if no response, immunosuppression, RBC transfusion if absolutely necessary, Cold: avoid cold, RBC transfusion if necessary, chemo, NOT steroids |
|
|
which autoimmune hemolytic anemia is treated with steroids
|
warm
|
|
|
cause of Paroxysmal nocturnal hemoglobinuria (PNH)
|
CD 55, 59 anchor protein deficiency (meaning that complement-mediated lysis can occur)
|
|
|
leads to venous thromboses and possibly Budd Chiari
|
PNH
|
|
|
diagnosis of PNH
|
Ham's test (cells incubated in acidified serum which triggers the alternative complement pathway
|
|
|
tx for PNH
|
steroids
failing that, transplant |
|
|
congenital thrombocytopenia
|
Fanconi's
|
|
|
congenital infection that can cause thrombocytopenia
|
rubella
|
|
|
IgG antiplatelet antibodies
|
ITP
|
|
|
petechiae and ecchymoses on skin
bleeding of mucous membranes no splenomegaly platelet count below 20,000 bone marrow with increased megakaryocytes |
ITP
|
|
|
tx for ITP
|
corticosteroids
IV immune globulin splenectomy platelet transfusions if desperate |
|
|
hemolytic anemia
thrombocytopenia mild acute renal failure fever transient neuorlogical signs |
TTP
|
|
|
tx for TTP
|
plasmapheresis as soon as diagnosis is established
steroids and splenectomy may benefit NO platelet transfusiosn |
|
|
microangiopathic hemolytic anemia
thrombocytopenia renal failure |
HUS
|
|
|
HUS plus fever and altered mental status
|
TTP
|
|
|
inheritance patter of Bernard Soulie
|
AR
|
|
|
Bernard-Soulier problem
|
platelet adhesion due to GPIb-IX problem
(size 9-10 shoes) |
|
|
abnormally large platelets on peripheral smear
|
Bernard Soulier
|
|
|
inheritance pattern of Glanzmann's thrombasthenia
|
ARR
|
|
|
problem in Glanzmans
|
platelet aggreatation due to GPIIb-IIIa deficiency
takes II to tango (associate) |
|
|
problem in vW disease
|
aggregation and adhesion
due to factor vWF deficiency vWF carries factor VIII in blood |
|
|
inheritance of vW disease
|
AD
|
|
|
prolonged bleeding time
normal platelet count |
Glanzmann
vW |
|
|
where is vWF synthesized? Factor VIII?
|
vWF in endothelial cells/megakaryocytes
VIII in liver |
|
|
decreased ristocetin-induced platelet aggregation
|
vW disease
|
|
|
tx for vW disease
|
desmopression (DDAVP) which induces endothelial cells to secretee vWF
factor VIII concentrates NO cryoprecipitate (risk of viral transmission) |
|
|
tx for pain in hemophilia
|
codeine with or without acetaminophen
avoid aspirin and NSAIDS!!! bleeding risk |
|
|
use of DDAVP in hemophilia?
|
A not B (B is factor IX vs VIII related)
|
|
|
what does thrombin time measure
|
fibrinogen
|
|
|
what does bleeding time measure
|
platelet function
|
|
|
normal bleeding time
|
2-7 minutes
|
|
|
increased PT and PTT means
|
liver disease
|
|
|
increased PT only menas
|
vitamin K deficiency
|
|
|
increased PT, PTT, bleeding time, TT
D dimer Fibrin split products |
DIC
|
|
|
what is decreased in DIC
|
fibrinogenplatelent count
|
|
|
tx for DIC
|
FFP
platelet transfusion Cryopercipitate Low does heparin O2, fluids |
|
|
what does vitamin K do
|
gamma carboxylation
|
|
|
of factors II, VII, IX and X, C and S, which has shortest half life?
|
VII
this means that prolonged PT is first lab finding in Vit K deficiency |
|
|
which is the only clotting factor not made in liver
|
vWF
|
|
|
inheritance pattern of antithrombin III deficiency
|
AD
|
|
|
inheritance pattern of Protein C deficienc
|
AD
|
|
|
tx for liver coagulopathy
|
FFP
Vit K if cholestasis Platelets cryo for fibrinogen |
|
|
is bleeding time increased in hemophilia? vW disease?
|
normal
increased |
|
|
are PT and PTT increased in ITP? tTP?
|
neither in both, just bleeding time
|
|
|
does liver disease affect PT? PTT?
|
both
|
|
|
what factors does Protein C inhibit
|
V, VIII
|
|
|
what does Factor V Leiden mutation affect
|
Protein C, so therefore V and VII
|
|
|
therapeutic PTT for anticoagulation by heparin
|
60-90 seconds
|
|
|
ideal INR in warfarin anticoagulation
|
2-3
|
|
|
decreased hemoglobin
increased ESR increased Ca increased protein in serum poor renal function |
Muliple myeloma
|
|
|
bence jones proteins
tubular casts |
MM
|
|
|
diagnositc driteria for MM
|
10% abnormal plasma cells in marrow
plus one of: M protein in serum or urine lytic bone lesions |
|
|
typical cause of death in MM
|
lung or urinary infection
|
|
|
rouleaux formation
|
MM
|
|
|
hyperviscosity
increased IgM no bone lesions |
waldenstroms macroglobinura
IgM is very large |
|
|
tx for MM
|
chemo with alkylating agents
radiation if no chemo response transplant (autologous blood stem cell>bone marrow) |
|
|
increased IgG
bence jones proteinuria |
MGUS
Monoclonal gammopathy of undetermined significance |
|
|
Reed strenburg cells
|
Hodgkins
|
|
|
Staging of Hodgkins
|
I - single node
II - II or more nodes, on same side of diaphragm III - both sides of diaphragm IV - disseminated A - no sx B - constitutional sx |
|
|
type of Hodgkins with worst prognosis
|
lymphocyte depleting
|
|
|
painless lymphadenopathy
fever night sweats weight loss pruirtis cough |
Hodgkins
|
|
|
tx for Hodgkins
|
I-IIIA - radiation
IIB and IV - chemo also |
|
|
what kind of Hodgkins is Burkitt's
|
NHL
|
|
|
HIV and Tcell lymphomas are associated with?
|
NHL
|
|
|
lymphadenopathy (can be only sx)
rare constitutional symptoms possible hepatosplenomgaly, abdominal pain or fullness |
NHL
|
|
|
indolent, low grade types of NHL
|
small lymphocytic
Follicular (t(14/18) |
|
|
t(14;18)
|
follicular lymphoma, an indolent, low-grade NHL
|
|
|
intermediate grade NHL type
|
diffuse large-cell
|
|
|
tx for diffuse largecell lymphoma (NHL)
|
CHOP
|
|
|
high grade types of NHL
|
lymphoblastic lymphoma
Burkitts |
|
|
t(8;14)
|
Burkitts NHL
|
|
|
lymphoma linked with EBV
|
African Burkitts
|
|
|
American vs. African Burkitts
|
America: abdominal organs
African: facial bones, jaw |
|
|
eczematoid skin lesions tha progress to generalized erythrodema
cribriform shape of lymphocytes |
Mycosis fungoides an NHL that is a T cell lymphpoma of the skin
|
|
|
in NHL what do LDH and Beta 2 microglobulin indicate
|
tumor burden
|
|
|
components of CHOP
|
cyclophosphamide
hydroxydaunomycin (doxorubicin) Oncovin (vincristine) Prednisone |
|
|
Downs is associated with which leukemia
|
AML
|
|
|
most common malignancy in children
|
ALL
|
|
|
what kind of leukemia has testicular involvement
|
ALL
|
|
|
what kind of leukemia has skin nodules
|
AML
|
|
|
Auer rods
|
AML (NOT ALL)
|
|
|
possible complication of chemo in Acute leukemias and high-grade NHL
|
tumor lysis syndrome
|
|
|
Cancer patient with:
increased potassium increased phos increased uricemia |
tumor lysis syndrome
|
|
|
monoclonal proliferation of lymphocytes
anemia thrombocytopenia neutropenia |
CLL
|
|
|
smudge cells
|
CLL
|
|
|
tx for CLL
|
observation
fludarabine, chlorambucil are so so |
|
|
t(9,22)
|
CML
|
|
|
increased alk phos
no splenomegaly precipitating event (infection) |
benign leukemoid reaction
|
|
|
increased RBCs independent of EPO
|
polycythemia
|
|
|
diagnositc criteria for Polycythemia
|
two major and two minor
Major: elevated RBC, psO2 >92, splenomegaly Minor: platelets >400, WBCs >12, alk phos >100, Vit B >900 |
|
|
hyperviscosity causing headache, dizziness, weakness, pruirits, visual impairment, dyspnea
DVT, CVA, MI, portal vein thrombosis bleeding, ecchymoes, epistaxis splenomegaly, hepatomegaly HTN |
polycythemia
|
|
|
tx for polycythemia
|
repeat phlebotomy
myelopsuppression with hydroxyurea or recombinant IFN-alpha |
|
|
pancytopenia despite a normal or hypercellular bone marrow
anemia, thrombocytopenia or neutropenia |
MDS
|
|
|
diagnosis of MDS
|
bone marrow biopsy
- dysplastic marrow cells - ringed sideroblasts |
|
|
ringed sideroblasts on bone marrow
|
MDS
|
|
|
Howell-Jolly bodies
|
MDS
Sickle cell Hemolytic anemia Hereditary spherocytosis splenic radiation |
|
|
peripheral smear with:
mildly elevated MCV low retics Howell-Jolly basophilic stippling nucleated reds hypolobulated PMN nuclei large agranular platelets |
MDS
|
|
|
MDS tx
|
supportive
cure only with bone marrow transplant |
|
|
teardrop cells on peripheral smear
|
MMF (Myeloid Metaplasia with Myelofibrosis)
|
