Internal Medicine Flashcards
The first step in evaluating a patient presenting with chest pain is a ——— to look for ———
12-lead ECG
ST segment elevation
Chest pain observed with MI/angina
Substernal or left-sided chest heaviness, pressure, or pain, typically radiating to the left arm, shoulder, or jaw. Often described as “an elephant sitting on my chest.” Commonly accompanied by diaphoresis or dyspnea.
(A key question to ask is does the chest pain worsen with exertion and get better with rest.)
Chest pain observed with pericarditis
Chest pain radiating to the shoulder, neck, or back; worse with deep breathing or cough (pleuritic); relieved by sitting up and leaning forward.
Chest pain observed with aortic dissection
Severe chest pain radiating to the back, can be associated with unequal pulses or unequal blood pressure in right and left arms. Often described as a “tearing” pain.
Chest pain observed with Thoracic abscess or mass
Often sharp, localized pain; can be pleuritic.
Chest pain associated with Pulmonary embolism
Often pleuritic. Frequently associated with tachypnea and tachycardia.
Chest pain associated with Pneumonia
Pleuritic, frequently associated with cough, sputum, and hypoxia if severe.
Chest pain observed with GERD/esophageal spasm/tear:
Burning pain, midline, substernal; may be associated with dysphagia. Pain made worse with lying flat, certain foods, accompanied by a bitter taste in the mouth known as “water brash.” May be similar to pain of MI.
Chest pain observed with Costochondritis/musculoskeletal:
Sharp, localized pain with reproducible tenderness (touch chest wall and feel the pain); often exacerbated by exercise (second or third costochondral junction inflammation, aka Tietze syndrome).
Chest pain isn’t the only presentation of MI—women often only complain of (2) as their “anginal equivalent.”
nausea or dizziness
MI can be silent, particularly in people with ———, due to ——— or ———, due to ———.
diabetes, due to neuropathy
transplanted hearts, due to denervation
Stable angina:
A chronic, episodic, predictable pain syndrome due to temporary myocardial ischemia. The pattern of pain is similar to that of acute MI, but resolves with rest or medication. Doesn’t change (i.e., it’s stable).
Stable angina: Treatment
Beta blocker (reduces myocardial oxygen demand), aspirin, nitroglycerin.
Patients with angina can benefit from ——— such as ——— to help control symptoms.
long-acting nitrates
isosorbide mononitrate
Prinzmetal angina (variant angina) due to:
coronary vasospasm, not linked to exertion
Prinzmetal angina (variant angina) Treatment
Calcium channel blockers and nitrates to reduce vasospasm.
Prinzmetal angina (variant angina)
Distinguished from unstable angina by
chronic, intermittent nature
Prinzmetal angina (variant angina)
pain usually occurs
at a specific hour in the early morning.
With Prinzmetal angina (variant angina), coronary vessels are
normal (no stenosis or plaques)
With Prinzmetal angina (variant angina), ECG may show
transient ST elevations
Coronary vasospasm can be seen in oncology patients being treated with
5-fluorouracil
A 62-year-old smoker presents complaining of 3 episodes of severe chest heaviness this morning. Each episode lasted 3 to 5 minutes, but he has no pain now. He has never had this type of pain before.
Think: Unstable angina.
A middle-aged woman comes to the emergency room (ER) with severe chest pain and ST elevations on ECG. She is rushed for a cardiac catheterization, which shows no obstruction in her coronary arteries.
Think: Variant angina (aka Prinzmetal angina/vasospasm).
For Vasospastic (formerly Prinzmetal or variant) angina, ——— is a risk factor; (2) are not.
Tobacco smoking
hypertension and hypercholesterolemia
For Vasospastic (formerly Prinzmetal or variant) angina,
Triggers include (3)
cocaine, alcohol, and triptans
Acute coronary syndrome (ACS) refers to patients in whom there is suspicion or confirmation of
acute myocardial ischemia (lack of blood flow to the heart) or MI (which is ischemia lasting long enough or severe enough that it leads to heart muscle damage)
ACS events are classified according to changes on the ECG:
- Non-ST-elevation changes: This category includes non-ST-elevation myocardial infarction (NSTEMI) and unstable angina.
- ST-elevation changes
Contrast the two types of ACS with Non-ST-elevation changes:
Both NSTEMI and unstable angina have chest pain and ECG changes, but unstable angina has no troponin elevation. Unstable angina is associated with increased frequency and/or severity of symptoms, symptoms at rest, or new onset of symptoms compared to stable angina, which has stable chronic symptoms.
Family history for coronary artery disease is a ——— in men, and ——— in women.
first-degree relative with an MI before age 40 in men, and before age 55 in women.
Stable angina vs Unstable angina vs NSTEMI vs STEMI: Pain occurrence
Stable angina: on exertion
Unstable angina: mild exertion or at rest
NSTEMI: at rest
STEMI: at rest
Stable angina vs Unstable angina vs NSTEMI vs STEMI: troponin level
Stable angina: no elevation
Unstable angina: no elevation
NSTEMI: elevated
STEMI: elevated
Stable angina vs Unstable angina vs NSTEMI vs STEMI: infarction
Stable angina: none
Unstable angina: none
NSTEMI: Subendocardial
STEMI: Transmural
Stable angina vs Unstable angina vs NSTEMI vs STEMI: ecg changes
Stable angina: none
Unstable angina: Possible ST depression and/or T-wave inversion
NSTEMI: ST depression and/or T-wave inversion
STEMI: ST elevation, pathologic Q waves
——— is the cardiac biomarker most commonly used clinically given its specificity for myocardium.
Troponin I
If you suspect ACS in patients, all patients should have “serial enzymes” drawn, which consists of
sending cardiac biomarkers every 6 to 8 hours for a 24-hour period
Myoglobin vs CK-MB vs Troponin T/I: onset (hrs)
Myoglobin: 1-4
CK-MB: 3-12
Troponin T/I: 3-12
Myoglobin vs CK-MB vs Troponin T/I: peak (hrs)
Myoglobin: 6-8
CK-MB: 18-24
Troponin T/I: 18-24
Myoglobin vs CK-MB vs Troponin T/I: duration:
Myoglobin: 24 hours
CK-MB: 3-4 days
Troponin T/I: 7-10 days
Myoglobin vs CK-MB vs Troponin T/I: specificity/utility
Myoglobin: nonspecific
CK-MB: Its onset and peak are similar to troponin but it clears in 3 to 4 days as opposed to 7 to 10 seconds with troponin. Therefore, it is extremely useful in patients where reinfarction after an MI occurs or for restent thrombosis.
Troponin T/I: Very sensitive and specific markers for cardiac muscle injury.
Criteria for STEMI on EKG
2mm ST elevation or new LBBB (wide flat QRS)
Patients presenting with ACS should be given ——— (unless contraindicated):
SNAP
Statin – high-intensity statin
Nitrate – sublingual or IV to control pain
Aspirin – non-enteric-coated, preferably chewed, 162 to 325 mg
P2Y12 inhibitors – clopidogrel or ticagrelor
Morphine
For patients presenting with ACS, ——— should be the last medication given. It may ——— and should only be given when a diagnosis of ACS is certain and the patient still has severe pain.
Morphine
mask angina
Troponin may also be slightly chronically elevated in
congestive heart failure (CHF)
Troponin is ———, so levels may be elevated in the setting of ———
renally cleared
renal insufficiency
Supplemental oxygen will ——— in nonhypoxic patients with ACS.
not affect outcomes
The symptoms associated with chest pain that have the highest relative risk for MI are (4)
radiation to upper extremity and association with diaphoresis, nausea, or vomiting
Typical symptoms for any angina/MI:
Left-sided/substernal chest pressure (not usually pain) with radiation to the left shoulder, arm, or jaw. The patient may characterize this by putting a closed fist over the chest (Levine sign), shortness of breath, diaphoresis, nausea, or vomiting. Chest pressure is worse with activity and improves with rest or nitroglycerin sublingually.
Atypical presentation of ACS: (list 6 sxs)
Up to one-third of patients with MI have no chest pain on presentation.
Other symptoms patients will complain about are dyspnea, weakness, nausea, epigastric pain, palpitations, or syncope.
Patients at risk for atypical ACS presentations are (2)
diabetics (may have neuropathy that blunts the sensation of pain)
women
Heparin effect on clotting:
does not dissolve already-present clots; rather, it prevents future ones from forming
Prescription of Beta blockers after presentation for ACS?
Contraindications? (3)
do not need to be given immediately, but rather, within the first 24 hours of ACS presentation
acute heart failure, cardiogenic shock, and right ventricular MI
Thrombolytics are not used in ———because 60% to 80% of the time the infarcted artery is not occluded.
unstable angina or NSTEMI
Patients with ST elevations have better outcomes when ——— happens within ——— minutes of arrival. This is known as ———
revascularization
60 minutes
door-to-balloon (DTB) time
Patients treated with thrombolytics should still be transferred to a ———capable hospital, for ——— and in case ———
PCI
angiography
the thrombus reforms!
Causes of ST elevation: (4)
• MI
• Pericarditis
• Left ventricular (LV) aneurysm
• Early repolarization (young people)
A 47-year-old man presents to the ER with left-sided chest pain at rest.
He is a chronic smoker with a family history of diabetes and hypertension. On examination, he is found to have stable vitals. ECG showed new ST depressions in II, III, and aVF. ASA, Plavix, and nitrate were given, and the patient still complained of pain. Next step:
Start heparin drip and send patient for cardiac catheterization
STEMI is typically caused by
complete occlusion of a coronary artery
Txt STEMI:
Need urgent opening of the blockage in the coronary artery (revascularization). This can be done with 1) thrombolytics (medicines that break up clots: tenecteplase, alteplase, and reteplase) and/or 2) cardiac catheterization and stenting (PCI)
In txt STEMI, ——— is superior to ———
Cardiac catheterization with PCI
thrombolytics
Contrindications for STEMI txt (cardiac catheterization with PCI/thrombolytics):
risks of bleeding, particularly in the brain, prior intracranial hemorrhage, stroke within 1 year, brain tumor, active internal bleeding, or suspected aortic dissection
Unstable angina and NSTEM have similar pathogenesis, typically due to an ———. NSTEMI differs
from unstable angina in that ———
incomplete occlusion of a coronary artery
the lack of oxygen is severe enough to cause myocardial damage and enzyme leakage (unlike unstable angina, where there is no enzyme leakage)
Treatment NSTEMI/unstable angina:
SNAP. Can treat with invasive treatment (cardiac catheterization) or conservatively (medications) based on risk factors (TIMI score). Ongoing chest pain and signs of cardiac dysfunction should prompt intervention.
What is a TIMI score?
A scoring system used to evaluate the risks of patients with NSTEMI and determine if early invasive management (i.e., cardiac catheterization) is warranted.
Each item is worth a point:
• Age>65
• ≥ 3 CAD risk factors
• Prior coronary stenosis > 50%
• ST changes on ECG
• ≥ 2 anginal episodes in 24 hours
• Use of aspirin in prior week
• Positive serum markers
TIMI > 4: Patient should get invasive management.
Cocaine can cause ——— changes with elevated ——— and ——— pain due to ———. Vasoconstriction, in addition to ——— and ———, can lead to a mismatch between oxygen supply and myocardial demand (do a urine drug screen if suspicious).
ECG
cardiac enzymes
chest
severe vasoconstriction without plaque rupture or thrombus formation
tachycardia
hypertension
If location of MI is anterior, involved artery and leads with ST elevation or q waves will be:
LAD
V1-V4
If location of MI is lateral, involved artery and leads with ST elevation or q waves will be:
Circumflex
I, aVL, V4-V6
(Note: Lateral includes aVL)
If location of MI is inferior, involved artery and leads with ST elevation or q waves will be:
RCA
II, III, avF
(Note: inferior includes aVF)
If location of MI is right ventricle, involved artery and leads with ST elevation or q waves will be:
RCA
V4 on R-sided EKG is 100% specific
Lead ——— is the only standard ECG lead that looks directly at the right ventricle.
V1
Significant timing elements for STEMI ECG changes over time:
ST elevation occurs “pretty much immediately” (minutes to hours)
T wave inversion occurs “a little later”“6hrs-years”
Pathological q waves markers of an old infarct “last pretty much forever”
Txt STEMI to restore blood flow (Emergency reperfusion)
Cath lab (best) or *thrombolytics if no contraindications (and if catch within 6 hours or if can’t get them to cath lab)
Contraindications for thrombolytic use in STEMI: (4)
Bleeding
“Ever ever had” hemorrhagic stroke
Recent ischemic stroke
Recent closed head trauma (if fell off a horse and then had a MI - no thrombolytics!)
Lead V1 is predominantly ——— in a normal ECG.
negative
A tall R wave in V1 has its own differential: (7)
• RBBB
• Posterior MI
• Dextrocardia
• Wolff-Parkinson-White
• Duchenne muscular dystrophy
• Right ventricular hypertrophy
• And, of course, a normal variant
——— is commonly referred to as dual antiplatelet therapy (DAPT). The duration of DAPT after ACS is still being investigated.
Aspirin with a P2Y12 inhibitor
A 65-year-old woman with a history of CAD and drug-eluting stent (DES) placed 6 months ago presents with acute crushing chest pain. She says that she had stopped taking her Plavix 1 week ago for a dental procedure.
Think: Acute in-stent thrombosis.
Next step: ECG (may show ST elevation; send for urgent cardiac catheterization).
Medications to be continued indefinitely after ACS: (5)
Aspirin
Beta blocker
Angiotensin-converting enzyme (ACE) inhibitor in patients with resulting low cardiac function (decreased left ventricular ejection fraction [LVEF]).
High-intensity statin (HMG CoA reductase inhibitor)
Clopidogrel, prasugrel, or ticagrelor if a stent was placed. (Clopidogrel or ticagrelor for 12 months if no stent was placed.)
Postinfarction complications: Ruptures usually occur within ———of a large MI; These can be
in the —(3)—. These are very rare in the era of angiography with PCI
4 to 5 days
free wall of the heart, the intraventricular septum, or the papillary muscle, which causes acute mitral regurgitation
Postinfarction complications
(Arrhythmias): (3)
VT usually within 48 hours (when the myocardium reperfuses)
bradycardia (usually from inferior wall MI)
atrioventricular block (can be from anterior wall or inferior wall MI)
Postinfarction complications: Dressler syndrome usually occurs ——— after cardiac injury (MI or cardiac surgery). It is associated with —(3 sxs)—; likely a ——— process. Treat with —(2)—.
1 or 2 weeks
fever, pericarditis, and sometimes pericardial or pleural effusions
hypersensitivity
NSAIDs and steroids
A 58-year-old man who was discharged from the hospital after an MI 2 weeks ago presents with fever, chest pain, and generalized malaise. ECG shows diffuse ST-T wave changes.
Think: Dressler syndrome. Treat with NSAIDs.
——— is the most common cause of anemia worldwide.
IDA
IDA occurs due to decreased
iron stores resulting in decreased hemoglobin synthesis
The most common causes of IDA are: (5)
blood loss
menorrhagia in premenopausal women
gastrointestinal blood loss
hookworm infection in tropical areas
malnutrition/malabsorption (e.g., bariatric surgery, Helicobacter pylori gastritis, celiac sprue)
pregnancy
Symptoms of IDA are: (7)
fatigue
exertional dyspnea
decreased exercise tolerance
Koilonychias (spoon nails)
pica (ingestion of clay or ice)
glossitis (red, beefy, glossy, swollen tongue without papillae)
cheilosis (crusting of angle of the mouth)
A young man or postmenopausal woman with IDA needs
aggressive workup with colonoscopy and endoscopy to evaluate for colon cancer.
Differential for low MCV includes: (4)
IDA
thalassemia
anemia of chronic diseases sideroblastic anemia
For IDA, serum iron, transferrin or TIBC, ferritin, % transferrin saturation (TSAT) (serum iron/TIBC):
serum iron: decreased
transferrin or TIBC: increased
ferritin: decreased
% transferrin saturation (TSAT) (serum iron/TIBC): very decreased
Both ferritin and transferrin saturation are decreased in ———; however, ferritin is increased in ———
IDA
anemia of chronic diseases
For IDA, MCV and RDW:
- Microcytosis, that is, low mean corpuscular volume (MCV) less than 80
- High red cell distribution width (RDW) more than 15% (Anisocytosis-increased variation in red cell size)
Management with IV iron formulations
can be dangerous! Make sure the patient is in a setting where nursing can monitor closely for reactions.
HIV patients on highly active antiretroviral therapy (HAART) can have ——— anemia due to ———
macrocytic
azathioprine (AZT)
Causes of megaloblastic macrocytic anemia (2) vs non-megaloblastic macrocytic anemia (4):
Megaloblastic macrocytic anemia: folate deficiency
vitamin B12 deficiency
Non-megaloblastic macrocytic anemia:
liver dysfunction
hypothyroidism
drugs
bone marrow disorders
Causes of folate-deficiency anemia: (4)
Pregnant MAN:
Pregnancy and lactation
Malabsorption (celiac and Crohn disease)
Alcoholism
Nutritional (toast and tea elderly diet)
Causes of macrocytosis:
FEeD THeM
Folate and vitamin B12 deficiency
Ethanol and liver disease
Drugs (AZT, methotrexate)
Thyroid (hypothyroidism)
Hemolysis (reticulocytosis)
Myelodysplastic syndrome
Folate deficiency can be differentiated from vitamin B12 deficiency by the lack of
neurologic abnormalities
Folic acid is absorbed in the ———, so conditions like ——— will lead to folate deficiency.
upper third of the small intestine
short gut syndrome
Lab findings in folate (vit b9) deficiency: (5)
High MCV
Hypersegmented neutrophils seen on peripheral smear
High homocysteine levels
Low red blood cells (RBCs)
Low serum folate levels
Most common causes of vitamin B12 deficiency include: (4)
pernicious anemia (lack of intrinsic factor)
lack dietary B12 intake (vegan diet or no-dairy diets)
fish tapeworm (Diphyllobothrium latum)
malabsorption (celiac, Crohn disease, bacterial overgrowth, and ileal resection)
Average age of diagnosis of pernicious anemia is
60 or older in adults
Causes of vitamin B12 deficiency
VITAMIN B
Vegan diet
Ileal resection
Tapeworm
Autoimmune (pernicious anemia-lack of intrinsic factor)
Megaloblastic anemia
Inflammation of terminal ileum Nitrous oxide
Bacterial overgrowth
Causes of normocytic normochromic (normal size, normal shape) anemia include: (3)
• Bone marrow problems (aplastic bone marrow, leukemia, myelodysplastic syndromes, etc.)
• Destruction (hemolytic anemias)
• Early nutritional anemias (iron, B12, folate)
Methylmelonic acid levels in vit b12 deficiency vs folate deficiency:
vit b12 deficiency: high
folate deficiency: normal
A hallmark of vitamin B12 deficiency is ——— symptoms that present after the anemia; may include:
neurologic
dementia or “megaloblastic madness”:
(Subacute combined degeneration of the dorsal (sensory) and lateral
(upper motor neuron) spinal cord columns from myelin deficiency; Symmetrical, affecting the legs and beginning with paresthesias and
ataxia (loss of vibration and position sense) and progressing to weakness, spasticity, clonus, and hyperreflexia (upper motor neuron symptoms))
Lab findings with vit B12 deficiency: (5)
high MCV
hypersegmented neutrophils
high homocysteine levels
high methylmalonate in urine
low serum B12 levels
Pernicious anemia pathogenesis:
- The parietal cell in the stomach produces hydrochloric acid (HCl) and intrinsic factor (IF). Vitamin B12 needs hydrogen ions from HCl to bind to IF, which carries vitamin B12 to the terminal ileum, where it is absorbed.
- In pernicious anemia, autoantibodies are made against the IF and/or the parietal cells, thus impairing the absorption of vitamin B12. As the parietal cells get destroyed, chronic gastritis and eventually gastric cancer can ensue.
Pernicious anemia associated with: (3)
chronic gastritis, vitiligo (another autoimmune process), and gastric cancer
In G6PD deficiency, causes of Hb precipitation within cells and hemolysis: (4)
infections
diabetic ketoacidosis
medications such as sulfa drugs (trimethoprim-sulfamethoxazole, primaquine), quinolones
fava beans
G6PD deficiency is an —(mode of inheritance)— that is more common in those with ——— ancestry
X-linked disease
Mediterranean (Italians, Greeks, Arabs) and African
ABCs of G6PD deficiency:
ABCDEFG
Antimalarials
Bactrim/bite cells
Ciprofloxacin
DKA
InfEction
Fava beans (can be Fatal without transfusion)
G6PD deficiency
Key finding on smear for G6PD deficiency:
reticulocytes, bite cells, and Heinz bodies
(Cells with precipitated Hb have Heinz bodies, which are removed by the spleen, resulting in bite cells)
Clinical features G6PD deficiency: (3)
- Acute hemolysis causing anemia, with sudden onset of jaundice (high indirect bilirubin)
- Dark urine (hemoglobinuria) and abdominal and back pain can occur
-Acute tubular necrosis can occur due to hemoglobinuria
G6PD deficiency: lab findings for bilirubin, haptoglobin, in urine, on smear, and for Coombs test
(Evidence of intravascular hemolysis)
increased indirect bilirubin
decreased haptoglobin
Urine: hemoglobinuria
Smear: reticulocytes, bite cells, and Heinz bodies
Negative Coombs tests (nonautoimmune).
For G6PD deficiency: G6PD assay timing
Check the G6PD assay 3 weeks after an acute episode because in acute situations younger RBCs that still have the G6PD enzyme reveal a falsely high level. The G6PD deficiency causes a decreased half-life of enzyme, so older RBCs without G6PD die.
A 31-year-old Italian male with back pain, dark urine, jaundice, and anemia after 2 days of ciprofloxacin. Dx and next steps?
Think: G6PD deficiency.
Next step: Check peripheral smear, looking for “bite cells”; transfuse if severe anemia; and check renal function.
Aplastic anemia is a:
Marrow failure resulting in pancytopenia from stem cell defect, which is usually from immune-mediated injury (idiopathic or after exposure to radiation, drugs, infections, or certain chemicals).
Common causative viral infection for aplastic anemia:
parvovirus B19
(Also viral hepatitis)
Drug causes for aplastic anemia:
chloramphenicol
( Also, benzene and dichlorodiphenyltrichloroethane (DDT))
Pure red cell aplasia (anemia only) can be seen in ——— patients after ——— infection
sickle cell patients
parvovirus B19 (erythema infectiosum/fifth disease)
Key clinical presentation features of aplastic anemia:
- Anemia: Weakness, fatigue, and pallor
- Thrombocytopenia and symptoms such as of mucosal bleeding and
spontaneous bleeding - Neutropenia can lead to fevers and infections with typical organisms
Findings for Diagnostic Workup of aplastic anemia:
- Low reticulocyte count and normal MCV
- Bone marrow biopsy will show hypocellular marrow with lots of white fat cells
Lab findings for anemia of chronic disease for RBCs, EPO, serum iron, transferrin/TIBC, ferritin, % transferrin saturation (serum iron/TIBC):
RBCs: microcytic or normocytic anemia
EPO: normal
serum iron: decreased
transferrin/TIBC: decreased (unlike IDA; Evolutionary reasoning—pathogens use circulating iron to thrive. The body has adapted a system in which iron is stored within the cells of the body and prevents pathogens from acquiring circulating iron)
ferritin: increased (acute phase reactant)
% transferrin saturation (serum iron/TIBC): normal or decreased
Lab findings for hematochromatosis for serum iron, transferrin/TIBC, ferritin, % transferrin saturation (serum iron/TIBC):
serum iron: increased
transferrin/TIBC: decreased
ferritin: increased
% transferrin saturation (serum iron/TIBC): very increased
Lab findings for pregnancy/OCP use for serum iron, transferrin/TIBC, ferritin, % transferrin saturation (serum iron/TIBC):
serum iron: normal
transferrin/TIBC: increased
ferritin: normal
% transferrin saturation (serum iron/TIBC): decreased
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin: what are we worried about?
Hemolysis
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin: Sickle cell kid w/ sudden drop in Hct?
Aplastic Crisis.
Sickle Crisis from hypoxia, dehydration or acidosis
(In SCD, aplastic crisis can be caused by infection with Parvovirus B-19. Fifth disease, a normally benign childhood disorder associated with fever, malaise, and a mild rash. This virus infects RBC progenitors in bone marrow, resulting in impaired cell division for a few days. Healthy people experience, at most, a slight drop in hematocrit, since the half-life of normal erythrocytes in the circulation is 40-60 days. In people with SCD, however, the RBC lifespan is greatly shortened (usually 10-20 days), and a very rapid drop in Hb occurs. The condition is self-limited, with bone marrow recovery occurring in 7-10 days, followed by brisk reticulocytosis.)
Sickle cell patients should always be on ———! Chronic hemolysis causes ——— loss and deficiency, ——— infection.
folate
folate
B19 infection (erythema infectiosum/fifth disease)
In sickle cell disease, Hb S will polymerize in ——— conditions, which ——— causing:
hypoxic
distorts the red cell into the classic crescent or sickle shape
decreased deformability can cause hemolysis and vascular occlusion, causing crisis
In SCD, acute vasoocclusive pain crisis (VOC) is caused by and typically manifests as:
Caused by: vascular sludging and thrombosis, which can lead to organ failure secondary to infarction, dehydration, fever, and leukocytosis
Manifests as: acute-onset generalized pain in the back, extremities, chest and abdomen, and joints
In SCD, acute chest syndrome: cause, presentation, and txt:
Caused by: occlusion of pulmonary vasculature by sickled cells and/or infection.
Presentation: Hypoxia, chest pain, shortness of breath, infiltrates
Txt: It is the most common cause of death in sickle patients and is an emergency requiring exchange transfusion with normal red cells. Treat with exchange transfusion.
In SCD, key genitourinary emergency:
Priapism can occur acutely or chronically. This is a medical emergency, as permanent damage can occur.
Chronic disease manifestations of SCD include: (7)
aseptic necrosis of the femoral head
pigmented gallstones (increased bilirubin)
hematuria
renal papillary necrosis
pulmonary hypertension
high-output cardiac failure
secondary hemochromatosis
In SCD, there is an increased susceptibility to infections due to ——— This carries a higher risk of sepsis with ———
functional asplenism (due to repeated infarction)
encapsulated organisms (Streptococcus pneumoniae, Salmonella osteomyelitis, and Haemophilus influenzae)
In SCA, hemoglobin ——— will show Hb S
electrophoresis
Blood smear of SCD will show: (2)
Howell–Jolly bodies (cytoplasmic remnants of nuclear chromatin that are normally removed by the spleen)
sickled cells
Blood tests in SCD will show: (2)
anemia
evidence of hemolysis:
Increased reticulocyte count, increased indirect bilirubin, and leukocytosis
Indications for exchange transfusion in sickle cell disease: (5)
Stroke/TIA
Acute chest syndrome
Priapism
Third-term pregnancy
Intractable vasoocclusive crisis
Signs of SCA:
SICKLE
Splenomegaly/Sludging
Infection
Cholelithiasis/acute Chest syndrome
Kidney (hematuria, papillary necrosis)
Liver congestion/Leg ulcers
Eye changes
Management of acute crisis in SCD: (3)
Analgesia, hydration, folate (and antibiotics if signs of infection) (assess for transfusion need)
Management of acute chest syndrome in SCD: (3)
Respiratory support, exchange transfusion, and empiric antibiotics for pneumonia.
Long term management of SCD: (2)
Hydroxyurea acts by increasing the amount of fetal hemoglobin; may increase frequency of crisis
H. influenzae and pneumococcal vaccines for prophylaxis
A patient walks in with macrocytic
anemia: MVC = 100 (nl 80-100), ↓retics, ↑homocysteine, nl methylmelonic acid
Folate deficiency anemia
A patient walks in with macrocytic
anemia: arrow shows?
Arrow: hypersegmented neutrophil
A patient walks in with macrocytic
anemia: MVC = 100 (nl 80-100), ↓retics, ↑homocysteine, ↑ methylmelonic acid
Vitamin B12 deficiency anemia
(And might also give neurologic sxs)
A patient walks in with macrocytic
anemia: MVC = 100 (nl 80-100). Arrow? Dx?
Arrow: Acanthocytes (“spur cells”)
Dx: Liver disease (another cause of macrocytic anemia)
A patient walks in with microcytic
anemia… MCV = 70 (nl 80-100), ↓Fe, ↑TIBC, ↓retic, ↑RDW, ↓ferritin: Dx?
Iron deficiency anemia
A patient walks in with microcytic
anemia… MCV = 70 (nl 80-100), ↓Fe, ↓TIBC, ↓retic, ↑RDW, nl or ↑ ferritin: Dx?
Anemia of chronic disease
A patient walks in with microcytic
anemia… MCV = 60 (nl 80-100): Dx?
thalassemia
(MCV super low - thalassemia characterized by really low MCV)
(Also note low RDW (little variation) - if genetic defect, all RBC affected by thalassemia; whereas, in another cause might be more retics, so greater variation in size of RBCs)
——— infection is a significant cause of morbidity in patients with thalassemia and other iron overload syndromes (cirrhosis and hereditary hemochromatosis)
Yersinia enterocolitica
A patient walks in with microcytic
anemia… MCV = 70, ↑Fe, ↑ferritin, ↓TIBC: Dx?
Sideroblastic anemia
(Isoniazid (inh) can cause sideroblastic anemia; isoniazid used for TB)
(Will see ringed sideroblasts in bone marrow and basophilic stippling in peripheral smear)
Lab findings in immune-mediated hemolytic anemia: antibodies, retics, MCV, indirect bilirubin, haptoglobin, LDH, Coomb’s test
- Presence of autoantibodies to one’s RBCs, resulting in hemolysis
- Reticulocytosis (bone marrow pushes out immature RBCs)
- normal MCV
- Increased indirect bilirubin (by-product of lysed cells [globin])
- increased LDH (by-product of lysed cells)
- Decreased haptoglobin (binds free hemoglobin released from lysed cells)
- Direct Coombs test (presence of antibody on RBC surface)
(Also Splenomegaly (site of clearance))
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin… Sudden onset after penicillins, ceph, sulfas, rifampin or Cancer: Dx? Destruction location? Antibody? Tx?
Dx: Warm Agglutinins.
Destruction in spleen.
Antibody: IgG.
Tx w/ steroids 1st, then splenectomy.
Warm hemolytic anemia: Immunoglobin, temperature, tx, seen with?
- IgG antibodies to different RBC antigens (e.g., Rh) (Do not usually fix complement (IgG))(“Warm weather is Good.”)
- Active at body temperature
- Treat with steroids. Transfuse if severe anemia. Splenectomy for steroid resistant.
- Seen with chronic lymphocytic leukemia (CLL), leukemias, SLE, and other autoimmune diseases, and drugs (penicillin, sulfas, and antimalarials); Sixty percent of cases are idiopathic.
PT—tests function of ———
common and extrinsic pathway (factors I, II, V, VII, and X).
defect results in increased PT (Play Tennis outside [extrinsic pathway]).
INR (international normalized ratio) =
patient PT/control PT.
1 = normal, > 1 = prolonged.
(Most common test used to follow patients on warfarin, which prolongs INR.)
PTT—tests function of ———
common and intrinsic pathway (all factors except VII and XIII)
Defect increases PTT (Play Table Tennis inside).
TT—measures ———. Prolonged by —(4)—
the rate of conversion of fibrinogen to fibrin
anticoagulants, hypofibrinogenemia, DIC, liver disease.
Coagulation disorders can be due to clotting factor deficiencies or acquired factor inhibitors (most commonly against factor VIII). Diagnosed with a ———, in which ———
mixing study
normal plasma is added to patient’s plasma. Clotting factor deficiencies should correct (the PT or PTT returns to within the appropriate normal range), whereas factor inhibitors will not correct.
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin… Cyanosis of fingers, ears, nose + recent Mycoplasma infx (walking pneumonia): Dx? Destruction location? Antibody?
Dx: Cold Agglutinins.
Destruction occurs in the liver.
Antibody: IgM mediated.
Cold hemolytic anemia: Immunoglobin, temperature, tx, seen with?
- Immunoglobulin M (IgM) antibodies. (Fixes complement (IgM))
- Active at cool temperatures (dissociate at 30°C) such as in distal body parts (blue fingers and toes).
- Treatment includes keeping warm; corticosteroids don’t work well.
- Seen acutely with Mycoplasma and infectious mononucleosis (resolve spontaneously) and chronically with lymphomas and Waldenström macroglobulinemia.
(Degree of hemolysis is variable.)
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin… Splenomegaly, +FH, bilirubin gallstones, ↑MCHC (mean cell hemoglobin concentration): Dx? Tx?
Dx Hereditary spherocytosis (AD loss of spectrin).
Tx w/ splenectomy
(Small, round RBCs with no central pallor. decreased surface area/dehydration -> increased MCHC -> premature removal by spleen (extravascular hemolysis).
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin… Dark (pink/red) urine in AM, Budd-Chiari syndrome (venous thrombosis, “clots in IVC”): Dx? Defect in? Increased risk for?
Dx: Paroxysmal Nocturnal Hemoglobinuria.
Defect in PIG-A. Lysis by complement.
Incr risk for aplastic anemia (and acute leukemias)
Normal MCV, ↑LDH, ↑indirect bilirubin, ↓haptoglobin… Sudden onset after primiquine, sulfas, fava beans: Dx? Lab findings? Avoid?
Dx: G6PDH def.
Finding: Heinz bodies, Bite cells.
Avoid oxidant stress.
A patient walks in with thrombocytopenia…. 30 y/o F recurrent epistaxis, heavy menses & petechiae. ↓plts only: Dx? Tx?
Dx: ITP.
Tx w/ prednisone 1st. Then splenectomy. IVIG if <10K. Rituximab
Clinical features ITP: (2)
- Petechiae and purpura over the trunk and limbs can occur spontaneously and with minimal trauma
- Mucosal bleeding can occur as well.
Cause and work up of ITP:
Cause: Immune-mediated thrombocytopenia of unknown etiology. (Antibodies against a platelet surface antigen are developed, and the antibody–antigen complexes effectively decrease platelet count by being removed from circulation.)
Work up: CBC will show low platelet counts. It is a diagnosis of exclusion; thus, absence of other factors to explain
thrombocytopenia is also key. (Antiplatelet antibodies are not useful for the diagnosis)
Tx ITP:
- Corticosteroids acutely to improve platelet count.
- IVIG for severe cases.
- Platelet transfusion if significant bleeding present.
- splenectomy can be performed electively to reduce recurrence.
Thrombocytopenia presents
as ——— vs Factor deficiencies (e.g., hemophilia) present with ———
oozing from mucosal sites (gums) and petechiae (Petechiae = Platelet Problem)
large bleeds (typically hemarthrosis)
Differentiate between ITP, aplastic anemia, and MDS:
• ITP has normal RBCs, normal WBCs, only low platelets with giant (young) platelets on
the peripheral smear. Usually don’t give platelets – treat with steroids or IVIG.
• Aplastic anemia has low RBCs, low WBCs, and low platelets (all cell lines low). No dysmorphic cells on smear.
• Myelodysplastic syndrome (MDS) can have any one or all lineages low. The smear can show dysplastic neutrophils (hypolobulated), and RBCs with ringed sideroblasts.
Causes of thrombocytopenia, think PLATELETS:
Platelet disorders: TTP, ITP, DIC Leukemia
Anemia
Trauma
Enlarged spleen
Liver disease
EtOH (alcohol)
Toxins (benzene, heparins, aspirin, chemotherapy agents, etc.)
Sepsis
And RBCs with ringed
Classic pentad for TTP—
RAT FaN
Renal dysfunction
Anemia (hemolytic) Thrombocytopenia
Fever
Neurologic dysfunction
A patient walks in with thrombocytopenia and this smear… what is in smear?
Schistocytes (“that’s scary”)
A patient walks in with thrombocytopenia and this smear… If PT and PTT are ↑, fibrinogen ↓, D-dimer and fibrin split products ↑: Dx? Causes? Tx?
Dx: DIC
Causes: Sepsis (LPS from gram - sepsis), rhabdo, adenocarcinoma, heatstroke, pancreatitis, snake bites (“don’t get bit by a snake”), OB stuff, Tx of M3 AML (Auer rods)
Tx: FFP, platelet transfusion, correct underlying d/o (“not much works right? You gotta correct underlying factor. You can give FFP to replace fibrinogen. You can transfuse platelets. But unless you correct what started this whole thing, you are kinda toast”)
A patient walks in with thrombocytopenia and this smear… If PT and PTT are nl: Dx? Causes? Tx?
Dx: HUS or TTP
Causes: O157H7 (causes HUS), ticlopidine, quinine, cyclosporine, (some drug associated with TTP-most important ticlopidine), HIV, cancer
Tx: Plasmapheresis. NO PLATELETS!
47 yo F with a history of “relentless DM” presents with a 1 week history of a necrotic, erythematous skin lesion. Diagnosis? Skin lesion is?
Dx: glucagonoma
(Skin lesion is Necrolytic migratory erythema)
MEN 2B: key features and association
- Medullary thyroid carcinoma: neoplasm of parafollicular C cells; secretes calcitonin
- Pheochromocytoma (secretes catecholamines)
- Mucosal neuromas (oral/intestinal ganglioneuromatosis)
Associated with marfanoid habitus; mutation in RET gene
Patients may experience ——— nails from excess thyroid hormone
onycholysis (Plummer nails) where the nail raises up away from the nail bed
Tx thyroid storm:
Sxs thyroid storm
A 38-year-old woman came to the office due to anxiety and palpitations for the past 2 months. She also notes a 6 lbs (2.7 kg) weight loss over this time period without changes in her diet. The patient is generally healthy; medical history is unremarkable. She does not consume caffeine, alcohol or illicit drugs. Temperature is 37.0°C (98.6°F), pulse is 115/min, and blood pressure is 125/85 mmHg. On physical examination, the patient’s skin is moist, and a fine hand tremor is noted. The thyroid gland is diffusely enlarged and nontender. Laboratory tests are obtained and shown below:
Which of the following is the most likely diagnosis?
- TSH-secreting pituitary adenoma
- Ingestion of exogenous thyroid hormone
- Toxic adenoma
- Graves disease
- Subacute thyroiditis
TSH-secreting pituitary adenoma
TSH-secreting pituitary adenoma is a rare cause of secondary hyperthyroidism. Patients may present with classic signs of hyperthyroidism, in addition to visual field defects, menstrual disturbances, galactorrhea, and headaches. Laboratory tests typically show normal/high serum TSH concentrations and elevated serum total and free T4 and T3 concentrations, as well as elevated alpha subunit of glycoprotein hormones.
A 31-year-old man comes to the physician complaining of neck pain for the past week. The pain is located mainly in the neck, but states that it travels to the jaw at times. He experienced episodes of palpitations and anxiety-attacks for several weeks, which resolved spontaneously one week ago. Past medical history is significant for Crohn disease, which is well-managed with diet. The patient’s father had thyroid cancer at the age of 77. Temperature is 37.0°C (98.6°F), pulse is 82/min, blood pressure is 124/88 mm Hg, and BMI is 20 kg/m2. Palpation of the neck causes severe pain for the patient. The thyroid gland is asymmetrically enlarged. Laboratory studies show the following:
ESR high
TSH high
Anti-thyroid peroxidase Negative
Anti-thyroglobulin antibody Negative
A biopsy of the thyroid gland is performed and shows diffuse infiltration with lymphocytes and giant cells forming noncaseating granulomas, as well as disruption and collapse of thyroid follicles. Further evaluation of this patient will most likely reveal which of the following?
- Family history of thyroid cancer
- Recent flare-up of Crohn disease
- A recent viral infection
- Biopsy showing vasculitic disease
- Positive serology for thyroid-peroxidase autoantibodies
A recent viral infection
Subacute granulomatous thyroiditis (de Quervain thyroiditis) is an inflammatory condition that occurs after a viral infection. The inflammation damages thyroid follicles, resulting in transient hyperthyroidism, followed by hypothyroidism, before returning to a euthyroid state.
This patient had a previous episode of hyperthyroidism (e.g., palpitations, anxiety attacks), followed by an extremely tender neck mass, elevated ESR) signs and symptoms of hypothyroidism, and biopsy findings showing granulomatous inflammation of the thyroid. These findings are suggestive of subacute granulomatous thyroiditis (de Quervain thyroiditis).
Although the exact mechanism is not yet established, subacute granulomatous thyroiditis is presumed to be caused by a viral infection. There are classically 3 stages to this condition, which are: 1) hyperthyroidism due to the extra thyroid hormones released by the damaged follicles, 2) hypothyroidism, and 3) return to a euthyroid state. Biopsy findings in the early phases will show neutrophils and destruction of follicles with colloid depletion, followed by formation of granulomas that surround the follicles.
On clinical examination, the most common and consistent finding is severe neck pain, which can often radiate to the lower face, causing jaw pain. Since the underlying cause of this condition is inflammation, laboratory studies typically show an elevated ESR. The majority of cases resolve spontaneously after several weeks.
Key causes of left sided HF
Key causes of right HF
New York Heart Association (NYHA) Functional Classification of Heart Failure
Class I: No limitation
Class II: Slight limitations (symptoms with ordinary
efforts)
Class III: Marked limitation (comfortable at rest,
symptoms with minimal efforts)
A 6-year-old boy is brought to his pediatrician’s office by his parents because of blister formation on his buttocks for the past 3 days. His mother reports seeing enlarged blisters on his buttocks that appear to be filled with fluid. His temperature is 37.1°C (98.8°F); pulse is 72/min; respirations are 18/min, and blood pressure is 95/60 mm Hg. Physical examination shows the following image: Which is the following toxins is most likely responsible for this patient’s condition?
- Toxic shock syndrome toxin-1
- Streptolysin S
- Exfoliative toxin A
- Pyrogenic exotoxin
- Streptolysin O
Exfoliative toxin A
Bullous impetigo is caused by exfoliative toxin A released from Staphylococcus aureus. Bullous impetigo should be treated with oral antibiotics.
A 60-year-old man comes to the emergency department because of severe shortness of breath. He has become progressively dyspneic over the past month and he can no longer walk to the bathroom without stopping to catch his breath. Patient says he stopped some of his home medications because he “couldn’t stand going to the bathroom so much”. Patient’s medical history is contributory for congestive heart failure. His temperature is 37.8°C (100°F), pulse is 120/min, respirations are 28/min, and blood pressure is 145/90 mm Hg. Physical examination shows jugular venous pulsations on the ear and well perfused lower extremities, with 3+ pitting edema bilaterally. An S3 sound and wheezes in all lung fields are heard on auscultation. ECG shows sinus tachycardia and chest x-ray is shown below. Which of the following is the most appropriate next step in management?
-Ipratroprium
-Metoprolol
-Nitroglycerin
-Furosemide
-Dobutamine
Metoprolol
Nitroglycerin is used to decrease preload and alleviate symptoms in acute decompensated congestive heart failure (CHF) exacerbation. CHF is related with pulmonary edema (PE), which is fluid accumulation in the air spaces and parenchyma of the lungs that leads to impaired gas exchange causing respiratory failure.
Pulmonary edema (PE) is fluid accumulation in the air spaces and parenchyma of the lungs. It leads to impaired gas exchange and causes respiratory failure. It can be a cardiogenic pulmonary edema (CPE) or a noncardiogenic pulmonary edema (NCPE). PE leads to fatal respiratory distress or cardiac arrest due to hypoxia, and is characterized by dyspnea, hemoptysis, hyperhidrosis, anxiety, and pallor. Diagnose is made with low oxygen saturation, disturbed arterial blood gas readings, and chest X-ray, which shows fluid in the alveolar walls, Kerley B lines (short lines perpendicular to the pleural surface that extend one to two centimeters into the lung and represent the thickening of the pulmonary interstitium), increased vascular shadowing, upper lobe diversion, and pleural effusions, among others. Managment is with tracheal intubation, mechanical ventilation, supplementary oxygen, and treatment of the underlying cause. Loop diuretics are administered, often together with morphine or diamorphine to reduce respiratory distress. Specific vasodilators are used (intravenous glyceryl trinitrate or ISDN), continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BIPAP/NIPPV), for reducing the need of mechanical ventilation. In acute decompensated congestive heart failure (CHF) exacerbation, the underlying cause is treated. Nitroglycerin is used to decrease preload and alleviate symptoms.
(Furosemide is a loop diuretic that reduces a person’s total volume status over the course of hours to days. However, in the absence of prior preload or afterload reduction, furosemide is associated with worsening hemodynamic function.)
(Dobutamine is a catecholamine inotropic agent which is used to increase cardiac output in patients who have congestive heart failure and signs of poor organ perfusion. However, inotropic therapies are also associated with tachyarrhythmias, increased myocardial oxygen demand, and increased rates of mortality.)
