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Cyanosis Diagnostic Tests
? Arterial Blood Gases
? PaO2 measures oxygen dissolved in the liquid component
of blood (the plasma)
? Oxygen saturation is calculated, not measured
? Carbon monoxide poisoning and methemoglobinemia
can have a normal PaO2 yet poorly oxygenated blood
? Cyanosis unresponsive to oxygen -- consider
? Hemoglobinopathy
? Right to left cardiac shunts (congenital / functional)
Carboxyhemoglobinemia doesn't cause
cyanosis (no reduced hemoglobin)


Methemoglobinemia (1)
? Methemoglobinemia
?Chemicals: aniline dyes (e.g. shoes), nitrate food
additives (e.g. sausage)
?Drugs: lidocaine, sulfonamides, dapsone,
benzocaine, Pyridium, nitrates, nitrites,
sulfonamides; not at usual exposure levels
?Fe++ (ferrous) iron in Hb becomes oxidized to Fe
+++ (ferric) state ? hemoglobin is then incapable
of carrying O2
?Approximately 1.5gm of Hb need to be in the Fe+++
(ferric) form for cyanosis to develop (chocolate
brown blood)

Methemoglobinemia (2)
? Methemoglobinemia (cont'd)
- Anemic patients require a higher % of Hb being
Fe+++ (ferric) to be cyanotic (e.g., 1.5gm/6gm =
25% vs. 1.5gm/15gm = 10% (although may be
disproportionately symptomatic)
- Antidote: methylene blue (reduces Fe+++ back to
- PaO2 is normal (because ABGs measure oxygen
that is dissolved in the plasma ? not than
attached to Hb)
- Pulse ox misleading (usual y 80-85% with
metHb) ? but reading is likely to be falsely high
with significant methemoglobinemia (>15%)
?Chocolate colored blood = methemoglobinemia

Methemoglobinemia (3)
Amyl nitrite
Signs & Symptoms
Aniline derivatives
Butyl nitrite

Bismuth subnitrite
Mild cyanosis
30-40 Headache, fatigue, tachycardia,
weakness, dizziness

>35% Dyspnea, lethargy

50-60 Acidosis, arrhythmias, coma, seizures,
badycardia, hypoxia

>70% Fatal
Silver nitrate
Room deoderizer


Packed Red Cells (PRBC)
? Shelf life of up to 42 days
? Stays unclotted due to calcium chelation by citrate
? Hematocrit 65-80%
? One unit PRBCs increases Hb by 1.5 g/dL
? Human blood volume = 70ml/kg = about 5 liters in
70kg adult
? Has essential y no platelets / limited clotting factors
Type O = universal donor
Type AB = universal recipient

Packed Cells vs. Whole Blood (1)
? Packed Cel s
?Less volume / less fluid overload
?More RBCs per volume transfused
?Decreased citrate infusion = better coagulation
?Decreased infusion of protein antigens
(less autoimmunization)
?Decreased infusion of potassium
(from lysed RBCs)
?Only diluent for RBCs is normal saline
?Calcium in Ringer's lactate causes microclots
to form

Packed Cells vs. Whole Blood (2)
Human Whole Blood CPD
Packed Red Cells MAP (Mannitol/
Red cel s are separated from 200mL or
Human Whole Blood CPD: Whole blood
400mL of whole blood by centrifugation
in col ected in 200 mL or 400 mL bag
and a preservative, MAP is added. This
with CPD, an anticoagulant and
is the most popular product among red
cel products. This is for patients with
This is for patients who need both red
anemia or dysfunctional red cel s.
cel s and plasma. Irradiated products
are also available.

Massive Blood Transfusion
? Results in a dilutional coagulopathy since
packed cells are deficient in clotting factors and
? Monitoring of coagulation by lab tests is likely to be
impractical in massive transfusions
? Use of fixed ratios of packed cel s, FFP and platelets
is now the trend (but the ideal ratio is not known)
? Hypothermia can be a complication -- blood warmer
? Microaggregates from RBC, WBC, platelet debris
showered into pulmonary capil ary bed causing
ARDS -- use 40 micron filter to decrease this risk
? Citrate toxicity is seen in large volume whole blood
transfusions ? can result in bleeding and
manifestations of hypocalcemia (decreased cardiac
pumping power)(QT prolongation)

Immediate Transfusion Reactions (1)
? Acute hemolytic transfusion reactions
?Results from infusion of incompatible RBCs
(usual y ABO incompatible)
?RBCs are destroyed by antibodies
?Fever, chil s, low back pain, breathlessness,
burning at infusion site
?May progress to hypotension, bleeding,
respiratory failure, ATN

Immediate Transfusion Reactions (2)
? Acute hemolytic transfusion reactions (cont'd)
? Stop transfusion
? Hydration to promote brisk diuresis
? Symptomatic
? Free hemoglobinemia and hemoglobinuria
? Haptoglobin (binds to free hemoglobin) is
? Coombs testing of pre- and post-transfusion
blood (a test for globulin antibodies on the
surface of RBCs)


Immediate Transfusion Reactions (3)
? Febrile nonhemolytic transfusion reaction
?Most common transfusion reaction
?Fever and chil s
?Due to interaction between recipient and donor
non-RBC components
?May be hard to distinguish from early acute
hemolytic reaction
?Must stop transfusion and r/o hemolysis

Immediate Transfusion Reactions (4)
? Allergic transfusion reactions
?Range from minor to anaphylaxis
?Due to plasma protein incompatibilities
?Erythema, urticaria, pruritus, bronchospasm,
vasomotor instability
?Reaction severity is not dose-related
?Discontinuation of transfusion is not always

Delayed Transfusion Reactions (1)
? Infections
?Overal risk of acquiring a viral, bacterial, or
parasitic infection is 1:500
?Risk of hepatitis B is 1:200,000
?Risk of hepatitis C or HIV is 1:2,000,000
?Risk of West Nile virus and Creutzfeldt-Jakob
disease is unknown
?Chlamydia transmission is common
? Delayed hemolytic reaction
?Antigen-antibody reaction 7-10 days after
? Other transfusion-related risks
?Volume overload

Delayed Transfusion Reactions (2)
? Other transfusion-related risks
?Noncardiogenic pulmonary edema
? Pulmonary edema secondary to
incompatibility of passively transferred
leukocyte antibodies
?Electrolyte imbalance
? Hyperkalemia from lysed RBCs
? Low calcium from excess citrate causing
chelation (causes prolonged QT)
? Hypokalemia from citrate being metabolized
to bicarbonate and resultant plasma alkalosis

? Five day storage life
? 1 unit of platelets derived from one unit of whole
blood wil raise the recipients platelet count by
? A platelet pheresis pack contains about six units
of platelets and is derived from
a single donor
? Give ABO compatible platelets
whenever possible (a smal
amount of RBCs contaminates
platelet packs)

Platelet Disorders (1)
? Nonpalpable purpura ? think low or dysfunctional
? Palpable purpura ? think angiopathy /
? Causes of dysfunctional platelets (increases the
bleeding time / platelet function test):
?Aspirin (for the life of the platelet)
?NSAIDS (only as long as in the blood stream)
?Ticlopidine (Ticlid) / clopidogrel (Plavix)
?Other drugs less commonly (penicil in /
cephalosporins / calcium channel blockers /
propranolol / nitroglycerin / antihistamines / others

Platelet Disorders (2)
? Causes of low platelet counts (increases the
bleeding time / platelet function test)
?Decreased platelet production
? Aplastic anemia / viral infections / drugs
(ethanol, thiazides, estrogens, chemotherapy
drugs, heparin)
?Increased platelet destruction
? ITP / TTP / HUS / DIC / viruses / drugs
?Splenic sequestration (trapping of RBCs)
? Hypersplenism (enlarged, overactive spleen
[rapidly and prematurely destroying RBCs])
? Hypothermia
?Platelet loss
? Bleeding / hemodialysis

Immune Thrombocytopenic Purpura (1)
? Can be primary where the immune trigger is
unknown (cal ed by some idiopathic
thrombocytopenic purpura ? but is stil immune in
? Can be secondary ? immune stimulus is known
(the vast minority of cases)
? Pediatric version
?Peak age, 5 y/o, both sexes equal y
?Sudden onset of petechiae or purpura several
weeks after an infectious il ness
?Most cases resolve within six months
?General y do wel without treatment

Immune Thrombocytopenic Purpura (2)
? Adult version
?Insidious onset / chronic duration / mostly women
?Genetic propensity in certain families
? Diagnosis largely via exclusion from other causes of
? General y no other findings except petechiae and
purpura ? CBC normal except for low platelets
? Platelet count >50,000 usual y found incidental y
? 50,000-30,000 = excess bruising with minor
? 30,000-10,000 = spontaneous petechiae and
? < 10,000 = spontaneous visceral hemorrhage 21

Immune Thrombocytopenic Purpura (3)
? Thresholds for treatment
?A platelet count of 20-30,000
?Active bleeding with a 30-50,000 count
? Treatment:
?Suppress the immune response first with
prednisone (50-75% remission rate by 3 weeks)
?High-dose RhoGAM (anti-D immune globulin) is
efficacious (can only be given to Rh+ patients)
? Is more efficacious and more expensive than
? Causes spleen to destroy antibody-coated Rh+
red cel s and destroy less platelets
?Can also give IV immune globulin (not as effective
and can have adverse side effects)

Immune Thrombocytopenic Purpura (4)
? Fear of intracranial bleeding usual y prompts
treatment (major cause of mortality due to ITP ?
especial y in the elderly)
? After suppressing the immune system about 2-3
times the calculated number of platelets need to be
given to get the count over 50,000
? General y, in adults, one platelet pack wil raise the
platelet count by 10,000
? Splenectomy is the ultimate treatment for those not
responding or having relapses
? Is not always effective (about 70% effective)
? Accessory spleens may be the cause of lack of
? Predisposes the patient to serious bacterial infections
(need al manner of immunizations)

Tests of Hemostasis (1)
? Bleeding time
?Has been replaced with the lab-performed
"Platelet Function Test"
?Measures platelet function
?Prolonged by uremia, NSAIDs, ASA, von
Wil ebrand's
?Not reliable for predicting risk of bleeding.

Tests of Hemostasis (2)
? Prothrombin time (PT)
?Measures extrinsic pathway (tissue factor) and
common pathway (prothrombin thrombin
fibrinogen fibrin)
?Reported as INR (INR=PTtest / PTnormal)
?Prolonged PT (possibly also prolonged aPTT
due to effects on final common pathway)
? Warfarin (Coumadin)
? Liver disease
? Vitamin K deficiency

Tests of Hemostasis (3)
? Activated partial thromboplastin time (aPTT)
?Measures intrinsic system and common
?Prolonged aPTT
? Heparin
? Hemophilia
? von Wil ebrand's disease
? Lupus anticoagulant

Tests of Hemostasis (4)
? Platelet count
?Decreased production: viral infections, marrow
infiltration, drugs (worst = heparin [a major
complication of this drug], gold, sulfa antibiotics,
quinine, quinidine, chronic
alcohol use)
?Increased destruction: viral infections, ITP,TTP,
DIC, HUS, heparin, protamine,
splenic sequestration, uremia, hemorrhage


Disseminated Intravascular
Coagulation (DIC) (1)
? Also known as "consumptive coagulopathy" is an
extrinsic pathway problem (vs dilutional)
? Most commonly caused by liberation of tissue
activating factor smal fibrin and blood clots
deposited in the microcirculation (consume clotting
factors; can cause tissue hypoxemia) fibrinolysis
fibrin degradation products and d-dimer
? Causes: meningococcemia (most extreme form of
DIC), trauma (especial y head), sepsis, retained
products of conception

Disseminated Intravascular
Coagulation (DIC) (2)
? Signs
? Lab Findings
? Bleeding
? Prolonged PT, aPTT (+/-)
? Thrombosis
Low platelet count
Purpura fulminans
Low fibrinogen level (but
may be normal yet DIC is
? Multisystem organ
present / In one study only
28% of DIC patients had a
low fibrinogen ? 22% in
another study)
? Elevated FDPs and d-
dimer (combo was 91%
sensitive / 94% specific in
one study
? Fragmented RBCs

Disseminated Intravascular
Coagulation (DIC) (3)
? Treatment of DIC
?Treat the precipitating problem
?If primarily bleeding manifestations, fol ow the
prothrombin (best single test in this setting)
?Give FFP replacement (10-15ml/kg) if the
prothrombin time is prolonged plus vitamin K
and folate
?May give platelets if needed as wel
?If primarily thrombosis, consider low-dose
heparin infusion

Thrombotic Thrombocytopenic Purpura (TTP)
? Similar to DIC
? Five clinical features:
? Severe decrease in platelets
? Severe microangiopathic hemolytic
anemia with red cel fragmentation
? Transient neuro deficits
? Renal failure
schistocytes = helmet cells =
? Fever
fragmented RBCs
? Systemic endothelial cel damage leads to release of von
Wil ebrand factor and consumption thrombocytopenia--smal
thrombi occlude arterials in heart, lung, kidney, pancreas,
adrenals leading to end organ ischemia
? Causes = idiopathic, drug-induced, pregnancy, infection
? Treatment: steroids, plasmapheresis, FFP
? Avoid platelet transfusion (can aggravate thrombosis)

Anemia and
Anemia and
thrombocytopenia thrombocytopenia
Predominantly Predominantly
Reflects the underlying
il ness
Heparin and blood
General y poor
*MAHA = microangiopathic hemolytic anemia

? Inhibits clotting factor activity
? Doesn't cross placenta (even LMWH doesn't)
? Complications: bleeding, decreased platelets
(aggregation / splenic sequestration /
antibody formation [can immediately reduce
platelets if previous exposure to heparin,
otherwise takes 6-10 days to occur])
Reversed with protamine sulfate
(1mg reverses 100 units)

Low Molecular Weight Heparin
? At least as efficacious as standard heparin
? Doesn't cross placenta
? Short chains derived from standard heparin
? Once or twice daily dosing
? Less likely to cause thrombocytopenia
? Reliable dose/response curve = no monitoring
?Does not affect the aPTT
? Each LMWH product is produced differently
and has different doses and frequency of

Warfarin (1)
? Inhibits liver synthesis of vitamin K-dependent
clotting factors (II, VII, IX, and X)
? Monitored by PT (INR)
? Over-anticoagulation wil also cause prolonged
aPTT because both the PT and aPTT measure
the function of the final common coagulation
? Multiple drugs (particularly oral antibiotics
? inhibit gut bacteria needed for vitamin K
production) can increase the effects of


Warfarin (2)
? Treatment of warfarin-induced over
anticoagulation depends on the urgency of the
clinical situation:
?Stop the drug (2.5 day half-life)
?Parenteral vitamin K (takes 12-24 hours)
? IV may be associated with anaphylactic
reactions ? IM safer
? May last up to 2 weeks making
reanticoagulation problematic
?Fresh frozen plasma (10-15ml/kg, may result in
fluid overload, requires defrosting, may require
significant time for infusion)

Warfarin (3)
? Treatment of warfarin-induced over
anticoagulation depends on the
urgency of the clinical situation:
? Prothrombin complex concentrates
? Concentrates of II, VII, IX and X
? No defrosting required, smal volume injected IV (30ml)
? Works immediately and is advised by the American
Col ege of Chest Physicians in their 2008 guidelines* and
the British Committee for Standards in Haematology
? Very costly
? *The ACCP statement regarding the urgent reversal of
warfarin anticoagulation: "Although fresh frozen plasma
can be given in this situation, immediate and ful
correction can only be achieved by the use of factor
concentrates because of the amount of FFP required to
ful y correct the INR is considerable and may take hours
to infuse."

Newer Anticoagulants
Direct Thrombin Inhibitors
? Attributes of both direct thrombin inhibitors and Xa
inhibitors - oral dosing / predictable effects / fewer food
and drug interactions / shorter plasma half-life /
improved efficacy-safety ratio / once or twice a day
? Primary indication ? non-valvular atrial fibril ation to
prevent strokes
? Direct thrombin inhibitors
? Dabigatran (Pradaxa) / 80% excreted by the kidneys
? Thrombin is a component of the final common pathway
of the clotting cascade
? A aPPT more than twice the upper limit of normal
suggests excess bleeding risk
? An INR cannot be used to assess bleeding risk

Newer Anticoagulants
Factor Xa Inhibitors
? Factor Xa inhibitors
?Rivararoxiban (Xarelto) / 35% renal excretion
?Apixaban (Eliquis) / 37% renal excretion
?Edoxaban (Lixiana) / 50% renal excretion
?Xa is a component of the final common pathway
of the clotting cascade
?aPTT tests are not appropriate for factor Xa
inhibitor assessment
?An INR cannot be used, but a prolonged PT
may indicate excess risk but direct quantitation
is not reliable

Bleeding and Newer Anticoagulants
? Maximum effect on coagulation tests occur about 3 hours
after ingestion ? therefore it is important to know when the
drug was taken
? Plasma abundance of the drugs may block the effects of newly
administered clotting factors
? Time is the most important antidote in the setting of non-life-
threatening bleeding due to the relatively short elimination half
lives of these drugs
? Normalization of hemostasis is about 12-24 hours with the Xa
inhibitors and the direct thrombin inhibitors (assuming normal
renal function in DTI patients) (consider dialysis with DTIs)
? Local hemostatic measures / fluid replacement / RBCs if
needed / FFP as a volume expander (doesn`t reverse these
? Consider tranexamic acid and desmopressin (transports Factor
8 and simulates release of von Wil ebrand factor)
? If life-threatening bleeding, consider adding prothrombin
complex concentrate (PCC) (no clinical evidence of its efficacy)
and activated factor VIIa

Sickle Cell Anemia
? A genetical y based chronic hemolytic anemia
? Baseline hemoglobin = 6-9 g/dL
? 5-15% reticulocyte count
? Cardiac and respiratory dysfunction is
? Routinely icteric
? Splenomegaly
splenic infarction

Sickle Cell Anemia

Sickle Cell Vasoocclusive Crisis (1)
? Cause: sludging of sickled RBCs causing
microcirculation obstruction, viscosity,
ischemic pain, infarction
? Precipitants: infection / cold exposure /
dehydration / high altitude / exertion ? common
theme, increased need for cel ular
oxygenation / trigger is unknown in more than
half of the cases
? Manifestations
? Musculoskeletal pain: most common presentation (arm,
leg, low back)
? Abdominal pain: second most common presentation /
diffuse, no peritoneal signs, poorly localized, rather
sudden in onset

Sickle Cell Vasoocclusive Crisis (1)

Sickle Cell Vasoocclusive Crisis (2)
? Acute chest syndrome
?The leading cause of sickler death and 2nd most
common cause of hospitalization (pain is first) /
most cases less than 21 years old
?Clinical syndrome:
? New pulmonary infiltrate involving at least one
complete lung segment (usual y lower lobes)
? Chest pain
? Fever (more than 38.5C)
? Concomitant tachypnea, wheezing or cough
?In about half the cases the initial cause for
admission is a reason other than acute chest
syndrome (mostly with vaso-occlusive pain crisis)
?In these cases, the syndrome develops on average
2.5 days into the hospitalization

Sickle Cell Vasoocclusive Crisis (3)
? Acute chest syndrome etiology
?Most common causes:
? Pulmonary infections (usual y chlamydia / mycoplasma)
? Pulmonary infarctions
? Fat embolism
? Can be a combination of the above
?Heavy emphasis on empiric treatment
? Oxygen (hypoxemia out of proportion to CXR findings
is the rule)(also is noted in PCP pneumonia)
? Antibiotics (always include a macrolide)
? Mechanical ventilation (13% in one large study)
? Incentive spirometry
? Pain management
? Bronchodilator therapy (even if not wheezing)
? Transfusions (especial y if at high risk)

Sickle Cell Vasoocclusive Crisis (4)
? CNS crisis: painless, cerebral infarction in
children / hemorrhage in adults
?Other CNS problems: TIAs, strokes, seizures,
? Renal crisis: infarction, hematuria, flank pain,
papil ary necrosis
? Hand-Foot Syndrome: in first two years of age,
swel ing of hands or feet due to avascular
necrosis due to vasoocclusion - may be first
sign of sickle cel disease
? Priapism ? exchange transfusion / corporal epi
and aspiration

Hematologic Sickle Cell Crisis
? Splenic sequestration
?2nd most common cause of death in SCD
children (infections are first, mostly pulmonary)
?Sickled blood blocks splenic outflow =
hypovolemic shock, painful
?Treat with RBCs and exchange transfusion
? Aplastic crisis
?Failure of bone marrow erythropoiesis
?Reticulocyte count low
?Precipitants: infection, folate
?Usual y self-limited

Infectious Sickle Cell Crisis
? Infection is the leading cause of death
? Especial y in children under 5
? Have functional asplenia as a predisposer to infection
Prone to infection by encapsulated organisms
e.g. Pneumococcus
Also prone to Salmonel a (bone infection),
H. influenzae, Staph, E. coli and Mycoplasma
Also watch for influenza and parvovirus
? Need immunizations to decrease risk of preventable
? Low threshold for antibiotics if infection suspected

Treatment of Sickle Cell Crisis
? Hydration
? Analgesics
? Oxygen
? Transfusions if indicated
? Emergent exchange transfusion for serious
sickle crisis (CNS infarction, sequestration)
? Antibiotics if indicated

Malaria (1)
? Patients with sickle cel disease are less
susceptible to malaria (means "bad air" ?
thought to come from fetid marshes)
? WHO says 219 mil ion documented cases 2010
? 600,000 to 1.2 mil ion deaths per year (90% in
sub-Saharan Africa)
? Transmitted by the female anopheles mosquito
(needs blood to nurture her eggs)
? Causes = five species of Plasmodium ? the worst
two = falciparum and vivax
? Diagnosis via blood smear, antigen-based rapid
diagnostic tests, PCR to detect DNA of the bug. 52

Malaria (2)
? Onset 8-25 days after bite (prophylactic
antimalarials may delay the onset of symptoms)
? Initial symptoms mimic the flu ? fever, headache,
shivering, joint pain, vomiting, hemolytic anemia,
hemoglobin in the urine
? Classical y ? paroxysms of sudden coldness
fol owed by rigor then fever, sweating every two
days (vivax and ovale) and every 3 days for
? Can progress to respiratory distress and renal
failure (blackwater fever - hemoglobin in urine)
? Always consider Dx. in equatorial travelers

Malaria (3)

Dengue Fever (1)
? 50-100 mil ion cases / yr ? endemic > 110
countries / viral tropical il ness / symptomatic Rx.
? Transmitted by mosquitos (Aedes aegypti)
? Initial y symptoms flu-like ? sudden onset fever
(returns every 1-2 days), headache (typical y
behind the eyes), muscle / joint pain (source of
the name breakbone fever) ? skin rash like
? 80% asymptomatic or mild symptoms
? 3-14 day incubation (usual y 4-7)
? Worst form ? dengue hemorrhagic fever ?
bleeding, low platelets, plasma leakage

Dengue Fever (2)

Dengue Fever Rash

Hemophilia (1)

Hemophilia (2)
? Hemophilia A is the most common variant (factor
VIII deficiency) (85% of cases)
?1 per 10,000 live male births (60% have severe
disease [less than 1% of normal factor VIII])
?Females are carriers and have 50% of the normal
clotting factor (Both A / B are x-linked recessive
? Hemophilia B (factor IX) (15% of cases)
?1 per 25,000 ? 35,000 males
? Bleeding sites
?Joints: destruction with time
?Soft tissue: neck, airway, retroperitoneal (usual y
?Extremities: compartment syndromes (5 "p" ?
pain, pal or, paresthesias, uselessness, paralysis)

Hemophilia (3)
? Bleeding sites (cont'd)
?Mucocutaneous - mouth, GI, nose
?CNS - IC bleeds are the most common cause of
death from bleeding - can be spontaneous
?Genitourinary: hematuria, common but not usual y
?Bone cysts from resolved hematomas
? High incidence of HIV with older treatments
(leading cause of death), also hepatitis B and C
? Tests - usual y normal PT, increased aPTT,
decreased factor VIII or IX

Hemophilia (4)
? Low threshold for factor r eplacement or
desmopressin (DDAVP is a trade name) (a synthetic
replacement for vasopressin) with any CNS
? Desmopressin causes release of VWB factor from
endothelial storage sites ? the increased amounts of
VWB factor al ows extra factor VIII to be carried in the
? Desmopressin raised VIII levels 3x within an hour
? Factor VIII replacement can be done with:
? Recombinant products (2-3x more costly than plasma-
derived products)
? Plasma-derived (human or porcine) purified products ?
very smal risk of viral transmission

Von Willebrand's Disease
? The most common inherited coagulation disorder (1%
of population)
? Multiple variants of the disorder exist and most patients
with it do not have a clinical bleeding disorder (about 1
in 10,000 do)
? Von Wil ebrand factor facilitates platelet activation and
adhesion and carries factor VIII in the plasma. The
trigger to this process is exposure of platelets to
subendothelial tissue
? PT usual y normal. Bleeding time prolonged, aPTT
increased in about half the cases
? Treatment
? DDAVP (induces release of VWF from storage sites
within the endothelium)
? Factor VIII concentrate has large amounts of VWF

HIV-Related Emergencies (1)
? Lactic Acidosis
? Certain anti-HIV drugs (nucleoside reverse transcriptase
inhibitors-NRTIs) can cause mitochondrial damage inhibiting
cel ular energy production resulting in lactic acidosis
? May start suddenly or gradual y
? Symptoms include abdominal pain, weight loss, malaise,
fatigue, rapid breathing, tachycardia
? Stop the drugs, bicarbonate, glucose, riboflavin
? Mortality can be as high as 60%
? Immune Reconstitution Syndrome
? Highly active antiretroviral therapy (HAART) can be
associated with a reconstitution of the immune system and
exaggerated immune responses to occult opportunistic
infections characterized by severe inflammation.
? Can be seen with subclinical TB (fever, SOB, enlarged
lymph nodes, cerebral masses [tuberculomas], CMV,
hepatitis C

HIV-Related Emergencies (2)
? Medication-Related Problems
? Hypersensitivity reactions to certain NRTIs
? Abacavir reaction = 4% = fever, skin rash, N/V, diarrhea,
abdominal pain, malaise, lethargy
? First six weeks, stop the drug, symptomatic treatment
? Nephrolithiasis
? Indinavir, a protease inhibitor, is wel -known to crystal ize in the
kidney and form stones (4-22%)
? Stones don't show up on plain x-rays or non-contrast CT
? Rashes / Stevens-Johnson syndrome
? NNRTIs can cause maculopapular rashes (25-35%) that can
progress to Stevens-Johnson syndrome (TEN) (1-8%
depending on which NNRTI given)
? Pentamidine-related hypoglycemia

HIV-Related Emergencies (3)
? Eye Emergencies
?Cytomegalovirus retinitis
? The most common vision-threatening
conditions in HIV
? T-cel counts typical y below 50
? Blind spots, visual field losses, flashing lights, floaters,
decreased VA
? Treatment with anti-CMV meds (ganciclovir, foscarnet,
?Varicel a zoster
? Second most common eye condition in HIV (shingles,
3-4% of patients
? Can involve the retina with retinitis and necrosis (70% get
retinal detachment with the necrosis)
? Treatment is acyclovir for 10-14 days

HIV-Related Emergencies (4)
? Pulmonary Emergencies
? Pneumocystis carini pneumonia (a yeast-like fungus)
? The leading AIDS-defining condition in the U.S.
? Typical y T-cel count is less than 200
? Often accompanied by moniliasis of the mouth (thrush)
? Fever, dry cough, disproportionate dyspnea compared
to chest x-ray findings, extreme fatigue
? Rapidly progressive fever, chil s and rigors are
UNCOMMON with PCP pneumonia as is purulent sputum
and suggest a bacterial pneumonia
? Diagnosis ? CXR and sputum exam
? Trimethoprim/sulfamethoxazole and steroids
? Bacterial pneumonias
? Findings similar to those without HIV

Pneumocystis Carinii

HIV-Related Emergencies (5)
? CNS-related emergencies
?Cerebral toxoplasmosis
? A parasite (hosted by cats) infection usual y caused by
ingestion of undercooked meats (lamb and pork) that
causes focal brain lesions (be careful with cat litter!!)
? Most cases occur with T-cel s less than 50
? Headache, confusion, altered mental status, fever,
seizures (up to 50%) and strokes (exceed seizures)
? Diagnosis by CT (ring-enhancing lesions) and positive
IgG antibodies to Toxoplasma
?Cryptococcal meningitis
? Most common systemic HIV fungal infection
? Typical signs and symptoms of meningitis
but may be slower in presenting and more indolent
? LP looking for cryptococcal antigen and fungal culture 68

"Ring Enhanced" Lesions of
Toxoplasma Gondii

HIV-Related Emergencies (6)
? GI-related Emergencies
? The most common cause of an HIV-related ED visit
? Diarrhea and abdominal pain can be related to HIV-
associated infections or non-HIV-associated infections and
noninfectious causes (often due to HIV medications)
? Opportunistic infections are unlikely with T-cel counts over
200 (therefore this number is important to know)
? Oropharyngeal ? consider Group A strep, GC, HSV, CMV,
C. albicans
? Intestinal ? consider C. difficile, Salmonel a, Shigel a,
Campylobacter, CMV, rotovirus, Giardia, E. histolytica
? Anorectal ? consider GC, Chlamydia, T. pal idu,
? Hepatobiliary/Pancreas ? E. coli, Klebsiel a, Proteus,
hepatitis C, B, A, CMV, E. histolytica, Microsporidia

Oncologic Emergencies

Cancer-Related Emergencies (1)
? Local tumor compression
?Spinal cord
?Pericardial effusion with tamponade
?Superior vena cava syndrome
? Biochemical derangements
?Hyperviscosity syndrome
?Adrenocortical insufficiency
?Tumor lysis syndrome

Cancer-Related Emergencies (2)
? Myelosuppression
?Granulocytopenia and sepsis
?Immunosuppression and opportunistic
?Thrombocytopenia and hemorrhage
?Anaphylaxis and transfusion reactions

Cancer-Related Spinal
Cord Compression
? Cause:
?Big three are lung, breast and
prostate (combined = 60%)
?Also - multiple myeloma, lymphoma,
renal cel carcinoma
? Incidence = clinical y develops in 5% of CA patients
? Etiology: bleeding, infection, fracture, tumor mass
? 60% thoracic, 30% lumbosacral, 10% cervical
? Manifestations: local pain, neuro deficits, urinary
retention, decreased anal sphincter tone
? Management: CT/MRI (MRI of the whole spine is best),
preparation for surgery, high-dose steroids

Cancer-Related Upper
Airway Obstruction
? General y slow and insidious and associated
with voice changes
? May be accelerated by infection, bleeding or
obstructing secretions
? Most often associate with
?Carcinoma of the larynx
?Thyroid carcinoma
?Metastatic lung cancer
? Treatment: airway

Pericardial Effusion (1)
? Can cause cardiac tamponade
? Most common cause is lung / breast cancer
? Malignant melanoma has a special predilection
for the heart
? May be related to:
? Speed of development
and volume of fluid determine effects

Pericardial Effusion (2)
? Classic findings
? Hypotension / narrow pulse pressure
? Jugular venous distention
? Diminished heart sounds
? Pulsus paradoxus greater than 10 mmHg (an
exaggeration of the normal physiologic response)
? Low QRS voltage
? Cardiomegaly on x-ray without evidence of CHF
? Definitive diagnosis: ultrasound
? Treatment: pericardiocentesis, pericardial window,
radiation, intrapericardial chemotherapy

Cancer-Related Superior Vena
Cava Syndrome (1)
? Due to obstruction of blood flow in the SVC
causing elevated venous pressure in the arms,
neck, face and head
? Malignant causes:
? Lung cancer (70%)
? Lymphoma (12%)
? Non-malignant causes:
? Goiter
? Pericardial constriction
? Thrombosis
? TB
? Radiation
? Central lines

Cancer-Related Superior Vena
Cava Syndrome (2)
? Findings
? Edema of face (82%), arms (46%)
? Head congestion / ful ness / headache (9%)
? ICP increase (may cause syncope [10%])
? Dyspnea (54%), Cough (54%), Hoarseness (17%)
? Papil edema
? Neck (63%) and upper chest vein congestion (53%)
? Facial plethora / telangiectasia (20%)
? Occasional y a palpable supraclavicular tumor mass
(sentinel node)
? Enlarged mediastinum on CXR
? Emergency treatment: diuretics and steroids,

Cancer-Related Hypercalcemia (1)
? Primary hyperparathyroidism accounts for
90% of al cases of hypercalcemia
? Cancer-related hypercalcemia causes 60% of
admits (most common cause is squamous cel
carcinoma of the lungs)
? Hypercalcemia effects 10-30% of CA patients.
Poor prognostic sign ? 80% die within a year
(median survival = 3-4 months

Cancer-Related Hypercalcemia (2)
? Secretion of parathyroid hormone-related protein
(mimics parathyroid hormone) accounts for 80% of
cases in cancer patients (increases bone resorption,
increased renal reabsorption of calcium)
? Osteolytic bone metastases account for 20% of
cases (usual causes: renal cel cancer, multiple
myeloma, lymphoma, bony metastases (lung, breast
[occur in up to 70%], prostate)
? Suggestive findings: dehydration, altered
sensorium, constipation, hypertension, back
pain, QT shortening, polyuria, muscle weakness,
abdominal pain. Renal failure, pancreatitis and
coma may be seen in those with life-threatening

Cancer-Related Hypercalcemia (3)
? Calcium and albumin
?Most calcium is bound to albumin
?Low serum albumin = low serum calcium
?Biological y active calcium is the ionized
unbound component
? Calcium and phosphate
?Inverse relationship: when one declines the
other increases
? Calcium and pH
?Alkalosis causes a decrease in ionized calcium
and an increase in bound calcium = functional

Cancer-Related Hypercalcemia (4)

Short QT: Consider Hypercalcemia

Cancer-Related Syndrome
of Inappropriate ADH
? Usual causes -- cancer of the brain, lung,
pancreas, duodenum, thymus, prostate and
lymphosarcoma (think of midline cancers)
? Ectopic secretion of ADH
? Manifestations
? Hyponatremia / low osmolality / normovolemia
? Less than maximal y dilute urine
? Excessive urinary sodium excretion
? Treatment
? Find and eliminate cause
? Fluid restriction
? Hypertonic saline if seizures or cardiac arrhythmias

Cancer-Related Hyperviscosity
Syndrome (1)
? Usual causes - macroglobulinemia, multiple
myeloma, chronic myelocytic leukemia
? Due to marked increase in serum proteins
(usual y immune globulins)
? Symptoms due to sludging of blood flow and
reduced perfusion and microthromboses
? Fatigue, headache, anorexia and somnolence
are early findings

Cancer-Related Hyperviscosity
Syndrome (2)
? Consider hyperviscosity if
?Altered consciousness
?Rouleau formation on peripheral RBC smear
?"Sausage-linked" retinal vessels on fundus
?Factitious hyponatremia / low osmolality /
? Treatment: hydration, phlebotomy and

"Sausage-Linked" Retinal Vessels

Cancer-Related Adrenal
Insufficiency (1)
? May occur from tumor replacement of adrenals
or adrenocortical suppression
? May be precipitated by infection, dehydration,
surgery, trauma
? Consider in al cancer patients with fever,
dehydration, hypotension and shock
? Empirical y treat cancer patients with steroids
who are dependent on them and have the
above findings / obtain blood for a cortisol level
before empiric treatment

Cancer-Related Adrenal
Insufficiency (2)
Lab clues to adrenal insufficiency
? Hypoglycemia
? Hyponatremia
? Hyperkalemia
? Eosinophilia
? Patients without adrenal function need about
35-40 mg of hydrocortisone per day (250-500
mg as an emergency dose to treat adrenal
insufficiency crisis)

Tumor Lysis Syndrome (1)
? Usual y is caused by the massive lysis of cancer cel s
from chemotherapy / 12-72 hrs post treatment
? Blood-based cancers are particularly prone to causing
TLS ? non-Hodgkin's lymphoma, acute or chronic
lymphocytic or myelogenous leukemia / breast, testicular,
smal cel lung
? Release of intracel ular electrolytes with elevations of K
(the most life-threatening component of TLS), Mg, PO4
and decreases of calcium (from combining with
phosphorus and precipitating)
? Extensive DNA / RNA breakdown causes uric acid
precipitation in the tubules causing acute nephropathy
? LDH elevations indicate extensive cel lysis
? Manifestations can be very quick and life-threatening.
? Dehydration and renal insufficiency predispose

Tumor Lysis Syndrome (2)

Tumor Lysis Syndrome (3)
? Elevated PO4 -- hydration and forced diuresis / oral
phosphate binders (aluminum hydroxide) / hemodialysis
? Elevated K ? hydration, furosemide, oral potassium
binders (sodium polystyrene) / aerosolized adrenergics /
glucose-insulin infusions
? Decrease uric acid build-up by alkalinization of the urine
(somewhat controversial but is likely safe with
aggressive hydration)
? Calcium supplementation only if evidence of CV
instability (Q-T prolongation on the EKG) or
neuromuscular irritability / otherwise avoid ? may cause
precipitation of calcium phosphate

Tumor Lysis Syndrome (4)

Cancer-Related Emergencies Due to
Myelosuppression and Infection
? Granulocytopenia and sepsis
? Nadir of WBC = 7-10 days post chemotherapy
?Febrile (38C or higher) neutropenia (absolute
neutrophil count of 500 or less)
? Associated with a 50% chance of having an
established or occult infection
? When infected, bacterial pathogens are the rule
? Empiric coverage with a broad spectrum
antipseudomonal penicil in plus an aminoglycoside is
general y recommended
? Immunosuppression and opportunistic


Joint Fluid Analysis (1)
? Most useful test: Gram's stain, culture, crystal exam,
and cel count.
? Cel count helps distinguish between
? Non-inflammatory (osteoarthritis, trauma)
? Inflammatory (rheumatoid, gout, SLE, etc.)
? Septic arthritis
? Synovial glucose
? Normal y close to serum glucose
? Decreased in septic arthritis
? May be extremely low in rheumatoid arthritis

Joint Fluid Analysis (2)
transparent transparent
Yel ow
Yel ow
Yel ow
>50% pos.
Multiple or None
Trauma Rheum- RA,SLE,Spondylo-
Staph. 98
atic fever

Septic Arthritis
? Hematogenous origin
? Newborn = Staph., Enterobacter, Group B strep.,
? Children under 15 = Staph., Strep pyogenes,
Pneumococcus, H. influenzae, Gram - bacil i
? Young sexual y active adults = Neisseria (females 3-4x
more common), Staph, Strep
? Special circumstances:
? Salmonella with sickle cell disease and SLE
? IV drug users - often involves axial skeleton (ribs,
vertebrae, sternoclavicular and SI joints), Often Gram ?
? Trauma / skin infection
? Staph. aureus is the most common cause overal of al
septic arthritis (and most virulent)
? Joint prosthesis ? early after surg. = Staph. / late =
Gram -

Septic Arthritis

Gout vs. Pseudogout (1)
?Uric acid crystal
?Calcium pyro-

phosphate crystal


(myelo- or
?Can be associated

lymphoproliferative with hyper-

disease) or
calcemia; most

decreased renal

excretion (diuretics) cases idiopathic

?Great toe (most
common), tarsals, ?Knee, wrist, ankle,
ankle, knee, males elbow / both sexes
equal y / elderly 101

Gout vs. Pseudogout (2)
? X-ray = negative
? X-ray = calcification of
? Needle-shaped

joint cartilages

? Rhomboid crystals

? NSAIDs, colchicine,
NSAIDS, colchicine,

? Allopurinol (decrease
production /
probenecid increase

Gout of the Great Toe

Chondrocalcinosis (Pseudogout)
Radiographic densities in
areas of cartilage are
consistent with deposition of
calcium pyrophosphate ? the
cause of pseudogout

Gonococcal Arthritis
? More common in females (4:1)
? Symptoms often start during menses
? Begins with fever, chil s, migratory
tenosynovitis, arthritis (knee, ankle, wrist)
? Characteristic rash in 2/3 - hemorrhagic,
necrotic pustules with surrounding erythema
(usual y occurs first on distal extremities)
? Blood and joint cultures are frequently
negative for GC
? Treat empirical y in young female patients with
fever, migratory polyarthritis and polytendonitis

Disseminated Gonorrhea

Lyme Disease (1)
? Caused by spirochete
Borrelia burgdorferi
? Vector = deer tick =
Ixodes dammini
(primarily on east coast)
? Erythema migrans = skin lesions early after
infection (from local spread of the organisms
in the skin [annular lesions with bright red
borders and cleared centers]), occur in
majority of cases (75%)

Lyme Disease (2)
? Multiple somatic symptoms
?Arthralgias / myalgias / frank arthritis
?Fever / fatigue
?Neurologic abnormalities (headache, facial
palsy, meningitis, radiculoneuritis)
?Cardiac manifestations
(dysrhythmias, heart block)
? Differential: GC, septic arthritis, rheumatic
fever, rheumatoid arthritis, Reiter's syndrome
? Treatment: doxycycline, amoxicil in,
erythromycin and others

Lyme Disease Rash / Erythema Migrans

Antiphospholipid Syndrome
? A noninflammatory autoimmune syndrome
whose primary pathologic process is thrombosis
(both arterial and venous)
? Multiple potential manifestations:
?Stroke / MI / retinal vessel thrombosis / PE /
thrombocytopenia / placental ischemia and fetal
?Catastrophic APL syndrome:
? Sudden, diffuse vascular occlusion leading to multi-
organ failure
? Treatment
?Life-long anticoagulation with warfarin

Rheumatoid Arthritis Emergencies
? Increased joint pain, edema and dysfunction =
rheumatoid flare
?Elevated acute phase reactants, NSAIDS,
? Felty syndrome ? triad of RA, neutropenia and
?Prone to serious bacterial infections
? Cervical instability (esp. C1-C2) ? can be
produced by minor trauma or occur unprovoked
? DMARDS (disease-modifying antirheumatic
drugs) treatment (e.g., anti-TFN) are
anti nflammatory drugs / predisposes to serious
infections, cancer

Systemic Lupus Erythematosus (1)
? Multiorgan autoimmune disease / most are females (esp
African-Americans, age 16-55 / exacerbations and
remissions are typical / runs in families
? Fever, joint pain and rash in susceptible age group -
think lupus
? Multiple potential manifestations
1. Constitutional symptoms: fever, fatigue, malaise, anorexia
2. Rheumatologic: can mimic RA, arthralgias (>90%),
myalgias, tenosynovitis
3. Dermatologic: butterfly facial rash (55-90%) / intermittent
? Discoid lupus: scaly, raised plaques primarily on face, head,
neck; most patients with discoid lupus do not have SLE
4. Renal: nephritis (persistent proteinuria), nephrotic
syndrome, CRF

Systemic Lupus Erythematosus (2)
? Multiple potential manifestation (cont'd)
5. Cardiac: pericarditis (20-30%, most common heart
involvement, myocarditis, effusions, tamponade
6. Pulmonary: effusions, pleurisy, pulmonary infarcts,
pneumonitis, cough dyspnea, fever
7. GI: oral & nasal ulcerations, intestinal vasculitis, with
possible perforation, gangrene
8. Hematologic: anemia, thrombocytopenia,
autosplenectomy, thrombosis (often due to
antiphospholipid syndrome)
9. Neurologic: seizures, strokes, psychosis (may be due
to SLE or steroid treatment of SLE), migraines,
neuropathy (neuro events often occur when SLE is
active in other organs)
? Most life-threatening inflammatory manifestations of
SLE are steroid-responsive

Malar rash of SLE

Seronegative Spondyloarthropathies (1)
? What are they?
? A group of disorders that share certain clinical features
and an association with the histocompatibility antigen
? A shared pathogenic mechanism is suggested and al are
negative for rheumatoid factor
? The primary examples
? Ankylosing spondylitis (SI joints first, progressive
inflammation and fusion of the spine)
? Reactive arthritis (e.g., Reiter's syndrome)
? Psoriatic arthritis
? Enteropathic arthritis (Crohn's / ulcerative colitis.)
? All of these conditions can be associated with
inflammation of the eyes, skin, mouth and a variety of

Seronegative Spondyloarthropathies (2)
? Reactive arthritis (formerly cal ed Reiter syndrome)
?Genetic predisposition, mostly white males, 15-35
?Triggered by infection, either urethritis / cervicitis
(Chlamydia) or dysentery (Salmonel a, Shigel a
[most common], Campylobacter etc.)
?Classic triad ? urethritis, conjunctivitis (may evolve
into iritis / uveitis), polyarticular, asymmetric arthritis
-- particularly
? Arthritis of the heel ("lover's heel")
? Sausage digits
? Psoriatic arthritis
? Inflammatory bowel disease
? Arthritis is often associated with flare-up

Rheumatic Fever
? A noninfectious immune disease, occurring in
genetical y predisposed individuals caused by
sensitization to certain types of Group A beta-
hemolytic Streptococcus
? Occurs 3-4 weeks after infection
? Jones Criteria (2 major or 1 major and 2 minor)
? Major criteria
? Minor criteria
? Migratory polyarthritis
? Fever
? Carditis*
? Arthralgias
? Chorea* (rapid face and
? Prior history of RF
arm movements ?late)
? Prolonged PR interval
? Erythema marginatum*
? Elevated acute phase
? Subcutaneous
reactants (CRP)
nodules*(back of wrist,
? Evidence of Group A strep.
elbow, front of knees

Erythema Marginatum /
Carditis / Nodules


A patient receives a massive blood
transfusion following an MVA. Which
is a known complication of this

A. Hypothermia
B. ABO incompatibility
C. Coagulation factor depletion
D. Thrombocytopenia
E. All of the above
HE 1

What would you expect the PaO2 to
be in a patient with carbon monoxide

A. Unable to determine from this information
B. Below normal
C. Normal
D. Substantial y different than that of a patient
with methemoglobinemia
E. Too complicated ? ask a pulmonologist

HE 2

A patient presents with fever in
association with a transfusion. The
Coombs test is positive. What is the
most likely cause of the fever?

A. Autoantibodies causing hemolysis of RBCs
B. Transmission of infection via the transfusion
C. An al ergic reaction to the blood
D. Administration of the blood too rapidly
E. Inadequate information to say
HE 3

Which of the following is true regarding
packed red blood cell (PRBC)

A. Stays unclotted in the bag because of the
phosphate additive
B. AB + is the universal recipient
C. The adult human blood volume is about 40ml/kg
D. One unit of PRBCs should raise the Hgb 3.5 g/dl
E. PRBCs contain many platelets
HE 4

Which of the following statements is
true regarding dengue fever?

A. It is transmitted by a mosquito bite
B. It is caused by a parasite
C. Periodic fevers are characteristic of this
D. The incubation periods for dengue fever and
malaria are similar
E. Dengue is treated with antiparasitic agents
HE 5

A 5 y/o male patient developed a recent onset
of diffuse bruising and petechiae. He had a
URI 2 weeks prior. Which of the following is
true regarding the most likely diagnosis?

A. Suppression of the immune system should
precede platelet transfusions
B. Is usual y associated with depressed WBC
C. The immune trigger to this pathology is
general y easily identified
D. Treatment should be initiated with PLT
counts of 50,000.
E. Anti-D immune globulin has no proven
HE 6

Regarding systemic anticoagulation,
which of the following is true?
A. Unlike unfractionated heparin, due to its smal er
molecular size, fractionated heparin crosses the
B. Heparin blocks the extrinsic limb of the clotting
cascade by blocking the effects of tissue
C. Measuring both the PT and aPTT are essential to
assess the effect of warfarin
D. Use of FFP to rapidly reverse warfarin-induced over
anticoagulation may result in fluid overload and
requires time for defrosting
E. Parenteral vitamin K reverses warfarin in 2-4 hours
HE 7

A 9 y/o sickle cell patient presents with
tachypnea, fever (39.0), chest pain and a right
lower lobe infiltrate on CXR. Which is true
regarding the patient
's most likely
A. It is among the least common causes of death
in sicklers
B. Requires a pulmonary infiltrate, chest pain,
fever and tachypnea, wheezing or cough
C. Atypical organisms are rarely associated with
infectious etiologies
D. Empiric treatment is specifical y not advised
HE 8

A 12 y/o has hemophilia A. Which
statement is correct regarding this

A. Factor VIII carries von Wil ebrand factor
B. Worse in females than males
C. Associated with an elevated PT
D. GI bleeding is the most common cause of
bleeding-related death
E. Give desmopressin before getting a CT scan
in patients presenting with a severe
HE 9

An 39 Y/O AIDs patient with a CD-4
(T-4) count of 35 presents with
decreased vision over 24 hours. Which
of the following is a true statement?
A. The most likely cause has findings general y
inconsistent with a retinal detachment
B. The most likely diagnosis is cytomegalovirus
C. Treatment is acyclovir
D. Varicel a retinitis is the most likely diagnosis
E. Most cases are irreversible
HE 10

Which of the following is true regarding
cancer-related cord compressions?

A. Most are lumbosacral in location
B. Cauda equina syndrome is associated with
increased anal sphincter tone and urinary
C. Lung, breast and prostate cancer are the
main causes
D. Low-dose steroids usual y resolve the
E. Assuming that both modalities are available,
CT is preferred over MRI
HE 11

Which of the following is true of
cancer-related superior vena cava

A. Typical y is associated with a normal chest x-ray
B. Is associated with many findings consistent with
decreased intracranial pressure
C. Is usual y caused by lung cancer
D. Is rarely associated with central venous lines
E. Emergency treatment is surgery
HE 12

A 40 y/o patient presents with a swollen,
red knee that is warm to the touch. Which
of the following synovial fluid findings is
consistent with septic arthritis?

A. Clear appearance
B. Color = bloody
C. PMNs = 50%
D. GLC = 30% of serum glucose
E. WBC = 36,000
HE 13

A 70 Y/O patient with squamous cell
cancer of the lung is noted to have a short
QT on his EKG. What other findings may
be expected in this patient?

A. Dehydration, altered sensorium, polyuria
B. Diarrhea, hypotension, hyperactivity
C. Muscle hyperreflexia, splenomegaly
D. Elevated blood glucose, elevated WBC count
E. Eventual development of a sine wave EKG
HE 14

A 36 y/o patient is evaluated for an
erythematous rash with areas of central
clearing. She gives a history of recent flu
symptoms with fever and headache. Which of
the following statements is true regarding
this disease?

A. TMP/SMZ is the drug of choice
B. Gram-negative bacteria is the cause
C. Can result in arthritis
D. Associated with a petechial rash on the
palms and soles
E. Is transmitted by mosquito bites
HE 15

A patient with myelogenous leukemia
presents 3 days after her last
chemotherapy treatment. She has
prominent T waves and a prolonged QT
interval. What entity is this patient likely
to have?

A. Antiphospholipid syndrome
B. Inappropriate antidiuretic hormone syndrome
C. Tumor lysis syndrome
D. Cancer-related hypercalcemia
E. Felty's syndrome
HE 16

A 24 y/o African American female presents
with fever, joint pain and a malar rash.
Which of the following is associated with
the most likely cause of her symptoms?
A. Proteinuria
B. Monarticular arthritis
C. Thrombocytosis
D. Vil ous adenoma
E. Polycythemia
HE 17

A 34 y/o female presents with a purulent vaginal
discharge, fever, chills, pain in her right knee,
that moved to her ankle and a hemorrhagic,
pustular rash. What findings are consistent
with her most likely diagnosis?
A. Blood and synovial fluid usual y isolate the
B. The onset is often associated with menses
C. The patient wil report a history of a painless
genital ulcer with spontaneous resolution
D. The 1st metatarsal phalangeal joint is the
most common location of arthritis
E. Most can be treated as outpatients
HE 18

What is a unique characteristic that tends
to be consistent with septic arthritis
caused by IV drug use?

A. Large extremity joint involvement is the rule
B. Salmonel a is disproportionately common
C. Rarely involve the axial skeleton
D. Are often Gram negative in origin
E. Are not hematogenous in origin
HE 19

Which of the following is not a potential
concern in certain patients with
rheumatoid arthritis?

A. Felty syndrome-related infections
B. DMARDS-related infections
C. Cervical instability particularly at C1-C2
D. Bukata syndrome (frequently misspel ed as
Brugada syndrome)
E. Steroid-responsive flares of joint pain, edema
and dysfunction
HE 20

Hematology-Oncology Answer Key
1. E
2. C
3. A
4. B
5. A
6. A
7. D
8. B
9. E
10. B

This post was last modified on 24 July 2021