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90 Cards in this Set

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What are the symptoms of neutropenia?
Bacterial and fungus infections, high fever, chill, peridontitis w/ severe pain, stomatitis, sepsis
What are the absolute levels of neutrophils to qualify for mild, moderate and severe neutropenia?
Mild: 1000-1500 /uL
Moderate: 500-1000 /uL
Severe: < 500 /uL
What is the absolute level of lymphocytes that defines lymphopenia?
< 1000 /uL
What is congenital lymphopenia called and how does it present?
Wiskoff-Aldrich Syndrome
It's an X-linked recessive trait with variable expression. It presents early in life with IgM deficiency, thrombocytopenia, atopy (eczema) and recurrent infections.
Death is usually due to infections or bleeding. Median survival is 8 years.
What is the screening and confirmation tests for HIV?
Use ELISA to diagnose, western blot to confirm
Compare and contrast Acute Lymphoblastic Leukemia with Acute Myelogenous Leukemia
ALL: peak age 3-5 years
AML: Median age of onset 65 years

ALL: Greater lymphadenopathy and bone and joint pain, more common to have CNS infiltration

AML: Auer rods in myeloblasts; less responsive to chemo

Both have anemia, splenomegaly, many blasts
Compare and contrast Chronic Lymphoblastic Leukemia with Chronic Myelogenous Leukemia
Both: leukocytosis

CLL: most common leukemia in adults; median onset > 55 years; small lymphocytes; lymphadenopathy; splenomegaly, thrombocytopenia; herpes zoster is common

CML: Philadelphia chromosome; median onset 45 years old; 3 stages.
1st - Chronic - Few blast cells, asymptomatic, normal Hct and platelets, splenomegaly, leukocytosis
2nd - Accelerated - Spenomegaly, anemia, fatigue, more blast cells
3rd - Blast crisis - Bone pain, fever, more than 30% blast cells
What is the bimodal age distribution for Hodgkin's Lymphoma?
15-34 years
> 45 years
What are the symptoms of Hodgkins Lymphoma?
First sx is painless swelling of nodes
Recurrent fevers
Night sweats
Weight loss
Pruritus
What are Reed-Sternberg cells?
Binucleate cells that look like "owl eyes". They're common in Hodgkins Lymphoma
What are the 4 types of Hodgkins Lymphoma and what are their histological appearances?
1) Nodular sclerosis - most common type; tough, gray nodes matted together in mediastinum; more common in women; good prognosis
2) Mixed cellularity type - Reed-Sternberg cells, lymphocytes, eosinophils; more common in older adults; fair prognosis
3) Lymphocyte predominance - mostly B cells, few Reed-Sternberg; uncommon, good prognosis, nodes are soft and uniform in appearance
4) Lymphocyte depletion - mostly Reed-Sternberg cells, uncommon, poor prognosis
What virus is associated with Hodgkin's lymphoma?
EBV
What are the 3 types of hepatic porphyrias? Which is the most common?
1) ALA Dehydratase Deficiency
2) Acute Intermittent Porphyria
3) Porphyria Cutanea Tarda - this is the most common
What is ALA Dehydratase Deficiency?
A hepatic porphyria that will not have PBG in the urine, but it will have ALA. It is a rare, autosomal recessive disease that presents with mostly neurological symptoms, though abdominal pain may be present. Lead poisoning will also disable this enzyme, called plumboporphyria.
What is Acute Intermittent Porphyria?
An autosomal dominant disease that is a deficiency of PBG deaminase (3rd step).

Autosomal dominant

Drugs, diet or steroids can activate disease

Sx:
Poorly localized abdominal pain and cramping, and decreased abdominal sounds and distention are common findings. No photosensitivity.
What is Porphyria Cutanea Tarda?
The most common of all porphyrias. It's a deficiency of uroporphyrinogen decarboxylase (5th step). Signs and symptoms:
Photosensitivity
Vesicles and bullae on sun-exposed areas
Liver damage
Elevated plasma porphyrins (also elevated in urine, stool and liver)
Can eventually cause cirrhosis and hepatocellular carcinoma.
What is the incidence for Non-Hodgkin's Lymphoma?
45-60 years old
7x more common than Hodgkin's
85% cases occur in B cells
What are the main differences between Burkitt's and non-Burkitt's non-Hodgkin's Lymphoma?
Burkitt's is usually in children, is associated with EBV and maxillary and mandibular tumors are common.

Non-Burkitt's is seen in adults and is not associated with EBV.

Sx common to non-Hodgkin's lymphoma:
Splenomegaly
Edema of face and neck (pressure on superior vena cava, causes edema)
Ureteral compression from pelvic nodes (can get acute renal failure)
What are the risk factors for major depression (Unipolar)?
o Heredity – first degree relative with depression - 3x higher risk
o Caretakers – increased risk for depression, added responsibility, stressful, guilty feeling when doing something for self
o History of abuse/trauma
o Stressful life events – loss of relationship, loss of loved one, loss of job
o Chronic illness, disability
What are the symptoms for unipolar depression?
o psychomotor retardation – move and speak slowly; more in adults, adolescents may have agitation instead
o severe sadness, extreme irritability in adolescents
o anhedonia – loss of pleasure; sex is first to be given up
o sleep disorder disturbances – insomnia, hypersomnia; early wake
o weight loss or gain of more than 5% in less than 1 months time
o hopelessness, fatigue
o excessive guilt; feeling like a fake or feel worthless
o suicidal preoccupation or thoughts of death (“do you wish you were the one”)
o inability to concentrate
o self-medicate – alcohol, drug use
What are the predictors of a switch to bipolar depression from unipolar?
Onset of depression < 25 y/o
Post-partum depression
Frequent episodes of depression
Quick response to tx for depression (will cycle more quickly)
Family hx of a mood disorder – especially if family hx in 3 consecutive generations
First degree relative has bipolar – 7 times more likely to get bipolar disorder
What are the symptoms of bipolar depression?
o Increase in psychomotor activity; high energy; eventually activity becomes very unproductive, though still energetic
o Pressured speech (speak quicker and quicker)
o Decreased need for sleep
o Easily distracted
o Increase in impulsive spending
o Sexual impulsivity
o May escalate to restlessness or irritability (very aggressive)
o Escalate to delusional imagination, usually grandiose delusions
At least two weeks with 5 or more symptoms of depression
Compare unipolar to bipolar depression
More disruption of social and occupational functioning with bipolar disorder
Shorter episodes (3-6 months)
Onset more abrupt with bipolar disorder
What are included in Cluster A, Cluster B and Cluster C personality disorders?
A (Odd/eccentric) - paranoid, schizoid, schizotypal
B (Dramatic/erratic) - antisocial, borderline, histrionic, narcissistic
C (Anxious) - avoidant, dependent, OCD, adjustment
What personality disorder is: a pervasive pattern of excessive emotionality?
Histrionic
What personality disorder is: a pervasive pattern of violation of the rights of others?
Antisocial
What personality disorder is: pervasive detachment from social relationships with no eccentric thoughts or behaviors?
Schizoid
What personality disorder is: pervasive, acute discomfort with and capacity for close relationships with eccentric or peculiar behavior and cognitive distortions of behavior?
Schizotypal
What personality disorder is a pervasive pattern of unstable relationships and self-image marked by impulsiveness?
Borderline
What personality disorder is: a pervasive pattern of grandiosity with a need for admiration and a lack of empathy for others?
Narcissistic
What are the suicide warning signs?
1) Pacing and agitation
2) Putting affairs in order
3) Delusions
4) Risk-taking behavior
5) Sudden mood change (either for better or worse)
What is generalized anxiety disorder and the theories of etiology?
It is characterized by 6 months or more of chronic, exaggerated or unfounded worry and tension.
Etiology:
1) Genetic predisposition
2) Increased serotonin activity in the limbic system
3) Psychoanalytical theory: conflicts against instinctive drives and internal controls
4) Cognitive/behavioral theory: they feel inadequate to cope, can be learned from parents
What is separation anxiety disorder?
Normal in toddlers when Mom or Dad leaves, parents will have it sometimes when kids leave for college. Lasts for at least one month, associated with depression symptoms, usually easy to treat.
What are the symptoms of generalized anxiety disorder?
6 months or more of chronic exaggerated or unfounded worry and tension and at least one of the following:
Fatigue
Restlessness
Sleep disturbances
Poor concentration
Irritablility
How long does acute stress disorder typically last? What are the symptoms?
2-4 weeks
Begins within one month of traumatic event
Sx of dissociation (3 or more)
1) Perceived detachment of the mind from the body
2) Sense of emotional numbing
3) Decrease awareness of someone's surroundings
4) Feeling that things are not real
5) Amnesia for the event
6) General anxiety, avoidance and flashbacks
What are the triggers and potentiators for panic disorder?
No triggers.
Potentiators include caffeine, nicotine, and alcohol
What are the symptoms of panic disorder?
1) Palpitations
2) Tachycardia
3) Can't breathe or hyperventilation
4) Chest pain
5) Choking, sweating, nausea
6) Dizziness
7) Feel like they're dying or going crazy
What are associated disorders with panic disorder?
Drug abuse
Agoraphobia
Anticipatory anxiety
What is the typical age of onset for OCD? What are the symptoms?
Childhood/adolescence
Sx:
Hand-washing
Cleaning
Counting
Continually checking things
What is the definition of personality disorders?
Severe disturbance in behavioral tendencies
Usually involve several domains
Maladaptive patterns based on interaction with a person's temperament and early life experiences
What are the different domains of personality and what do they mean?
Expressive behavior - how they appear to others
Cognitive style - how they process, organize and communicate thoughts
Interpersonal conduct - how they interact with others
Mood/temperament - how they display emotions
Self-image - how they describe themselves
Regulatory mechanisms - how they self-protect
Morphologic organization - how structured or organized the mind is, their psychic framework
Object relations - inner imprint left by very early experiences
How does a narcissistic person present with regards to domains of personality?
Expressive behavior - arrogant and haughty
Cognitive style - expansive, grandiose
Interpersonal conduct - exploitative
Mood/temperament - frigid
Self-image - admirable
Regulatory mechanisms - rationalization
Morphological organization - spurious, false
Object relations - continually changing memories of their past
What personality disorder is: pervasive distrust and suspiciousness?
Paranoid
How does a paranoid person present with regards to domains of personality?
Expressive behavior - defensive
Cognitive style - suspicious
Interpersonal conduct - provocative, testing
Mood/temperament - quick to anger
Self-image - self-important
Regulatory mechanisms - project undesirable traits onto others
Morphological organization - very rigid
Object relations - deeply held attitudes based on unwarranted fears
What personality disorder is:
Complex pattern of feelings of inadequacy, hypersensitivity, and social inhibition
Difficulty separating present from past pain
Common in children that were abused or neglected
Avoidant
What personality disorder is characterized by fear of abandonment, submissive and clingy behavior, and difficulty with daily decisions?
Dependent
What characteristics does the obsessive compulsive personality disorder have?
Pervasive preoccupation with orderliness and perfection at the expense of flexibility and efficiency; not as serious as OCD

They may horde money, be rigid and stubborn, and excessively devoted to work at the expense of other duties or friends.
What's the definition of somatization?
A chronic disorder with recurring clinically significant physical complaints. The complaints can't be explained by a physical disorder and no organic basis can be found.
What are the Dx criteria for somatization?
Onset before 30 years old

Hx of pain affecting 4 different sites

2 or more GI sx other than pain: diarrhea, constipation, bloating, dysphagia

1 sexual/reproductive disorder: dysmenorrhea, decrease in desire, dyspareunia (pain with intercourse)

1 neurological sx or deficit: HA, dizziness, numbness, tingling

No exam will diagnose symptoms
What are the symptoms of somatization?
Dramatic and emotional history - "worst pain", "unbearable"

Highly dependent on personal relationships - angry when needs are not met

Dependent personality

Exhibitionist and/or seductive

Manipulative

"Doctor shopping"
What are the traits/symptoms of Munchausens' Syndrome?
Patients report factitious symptoms that fit perfectly with a diagnosis

Recurrent feigned or induced illnesses

Borderline personality traits - self-destructive tendencies

Knowledge of the medical field

History of multiple hospitalizations

Unusual or dramatic presentation (though the symptoms usually fit the diagnosis perfectly - "classic presentation")
What is schizophrenia and what are the positive, negative and disorganized symptoms?
Def: a recurrent disorder with psychotic symptoms. Patient has a deterioration in self-care and functioning. Onset is usually late adolescence to early 20's.

Positive Sx:
Delusions
Hallucinations

Negative Sx:
Social withdraw
Absence
Poor hygiene

Disorganized Sx:
Confused speech, flight of ideas
What is the prodromal phase of schizophrenia and when does it occur?
In prodromal stage, there's social isolation, decreased attention and motivation and inability to plan for long term. 50% develop substance abuse problems.

Occurs in late teenage years; could look like drugs because they have trouble with social situations and academics.
What's the difference between abuse, dependence and addiction?
Abuse: dangerous use of substance

Dependence: abstinence results in pathologic symptoms

Addiction: inability to control use despite negative consequences
What are the physiological effects of alcohol?
Initial depressant
Followed by CNS agitation: flushing, nausea, tremors
What are the withdrawal symptoms from alcohol?
They begin within 24 hours after last drink
H/A, anxiety, muscle twitching, nausea, tremors, weakness, seizures, delirium tremum
Peak in 2-3 days and disappear after 1-2 weeks
What is the cause and symptoms of Wernicke and Korsakoff?
B1 deficiency

Wernicke (acute):
1) Confusion
2) Ataxia
3) Lateral rectus palsies (opthalmoplesia)

Korsakoff (chronic): memory loss (retrograde and anterograde, confabulation
What are the symptoms of fetal alcohol syndrome?
1) Microcephaly
2) Development delay
3) Mental retardation
4) Low birth weight
5) Facial deformities (triangular philtrum, large forehead, wide bridge of nose)
6) Behavioral problems - usually occur around puberty, can be perfectly normal in grade school
What are the short-term and long-term effects of meth?
Short term
1) Mydriasis (dilation of pupils)
2) Tachycardia
3) Hypertension
4) Hyperthermia
5) Arrhythmias

Long term
1) Paranoia
2) Formication (feeling like something is crawling on skin)
3) Mood disturbances
4) Psychosis
5) Interference with hypothalamic/pituitary axis
What is the 1/2 life of meth? What is the 1/2 life of cocaine?
1/2 life of meth: 12 hours

1/2 life of cocaine: 1 hour
What are the Sx of opiate use?
Miosis (constriction of pupils)
Hypotension
Respiratory suppression
Bradycardia
What are the diagnostic criteria for ADD/ADHD?
Some of the Sx present before 7
Objective evidence of social/academic impairment
Impairment in more than 1 setting
Sx not the result of another disorder
What are the Sx of ADD/ADHD?
6 symptoms of inattention for 6 months:
1) Repeated careless mistakes
2) Difficulty sustaining attention, easily distracted
3) Fails to finish work
4) Avoids tasks requiring mental effort
5) Loses things repeatedly
6) Forgetful in daily activities

6 symptoms of hyperactivity for 6 months
1) Fidgets, squirms
2) Leaves seat
3) Runs or climbs excessively
4) Difficulty playing quietly
5) "Driven by a motor"
6) Talks excessively, blurts out answers, loud
7) Interrupts or intrudes, can't keep hands to themselves
What is the DDx for ADD/ADHD?
Emotional trauma
Chronic OM with hearing loss
Petit-mal seizures
Learning disabilities
Conduct disorder
What are the Dx criteria for Bulimia nervosa? What's the difference between it and Anorexia nervosa?
Binging and purging at least 2x/week for 3 months

May maintain a normal weight

Laxative and diuretic abuse is common

Erosion of dental enamel

Hypokalemia

Diff from Anorexia: Bulimics are less introverted than anorexics, more likely to get help
What is the definition of Anorexia nervosa?
Refusal or inability to maintain weight for the height

Intense fear of weight gain and food

Disturbed sense of body image

Preoccupation with food/exercise
What are the complications of Anorexia nervosa?
Hypothyroidism
Hypotension
Skin is dry, yellow and covered in lanugo
Brittle nails and hair
Dehydration, menses stop
Anemia, decreased muscle mass
Arrhythmias
Brain atrophy
What is the treatment for Anorexia nervosa?
Early intervention with family and individual therapy

Address self esteem and dysfunctional relationship problems

Get them back to an acknowledgement of normal hunger cues
In anemias caused by decreased RBC production, is the reticulocyte increased or decreased?
Decreased
In anemias caused by increased RBC destruction, is the reticulocyte increased or decreased?
Increased
What is a sideroblast? What is a ring sideroblast?
Sideroblasts are seen in normal erythroblasts.

Ring sideroblasts are seen in pathological conditions and one can see more iron around the nucleus.
What is the inheritance pattern for the congenital form of sideroblastic anemia? What type of anemia does it cause?
X-linked
Autosomal recessive - rare

Congenital is usually a microcytic anemia
What are the causes of acquired sideroblastic anemia? What type of anemia does it cause?
RARS: a myelodysplastic syndrome
Lead poisoning
Excessive ETOH consumption
Zinc overdose
Copper deficiency
Hypothermia
Drugs (chemo and TB)
Idiopathic

Usually causes macrocytic anemia without megaloblasts in bone marrow
How do you diagnose sideroblastic anemia?
Bone marrow biopsy. > 10-15% ringed sideroblasts will be seen.
What are the causes of iron deficiency anemia?
Increase iron requirement (pregnancy, 1st year of life when body weight triples and Hb mass doubles)
Inadequate iron intake
Malabsorption
Blood loss (usually in GI tract)
What do the labs show in iron deficiency anemia?
1st stage: normal except for low ferritin

2nd stage: HCT normal, Hb low/normal, serum iron low, TIBC increased, % sat low/normal, FEP (free erythrocyte porphyrin) is increased

3rd stage: Hb low, HCT low; this takes 6 months to 20 months
What are the clinical findings in iron deficiency anemia?
Pallor of skin & conjunctiva
Spooning of the nails (koilonychia)
Glossitis
Tachycardia and flow murmur
Pagophagia (ice eating)
What are the Sx of Alpha-thalassemia?
If 1 or 2 genes are defective: mild anemia

If 3 genes: life-long hemolytic anemia

If 4 genes: fetal death
What is the other name for Beta-thalassemia Major?
Cooley's anemia
What are the Sx for Beta-thalassemia minor?
1 of 2 genes are missing

Sx:
Mild to moderate anemia, jaundice, splenomegaly
Prominent microcytosis and hypochromia (more than iron def anemia)
Basophilic stippling (you will also see this with lead poisoning)
What are the Sx for Beta-thalassemia major?
Presents at 4-6 months of life
Wasting, jaundice, slow growth/dev
Severe anemia
Splenomegaly
Bone marrow hyperactivity (overgrowth of maxilla, huge frontal bones, facial bones, crew-cut x-ray)
Hepatic siderosis
What do the labs show for the Thalassemias?
Microcytic anemia
Fe and ferritin increased
Reticulocyte count increased a little
TIBC decreased
What are the causes of anemia of chronic disease? What type of anemia?
Chronic infections (TB, subacute bacterial endocarditis)
Chronic inflammatory diseases (RA, SLE, IBD)
Liver and renal disease
Diabetes

Normochromic, normocytic 70% of the time
Hypochromic, microcytic 30% of the time
What do the labs show for anemia of chronic disease?
Serum Fe decreased
Serum ferritin increased
TIBC decreased
Transferrin saturation - may be normal or slightly decreased
Reticulocyte count decreased (less marrow responsiveness)
What are the causes of B12 deficiency?
Pernicious anemia
Crohn's disease and celiac sprue
Inadequate intake
What are the Sx of B12 deficiency?
Anorexia, diarrhea, weight loss
Glossitis
Numbness, ataxia, parethesias
Positive Babinski sign, loss of proprioception
Sphincter dysfunction in severe case
What do the labs show for B12 deficiency?
Low B12
MMA inceased
Increased MCV
Low reticulocyte count
Schilling test dx - where radioactive B12 absorption is measured
What is Intrinsic Hemolytic Anemia also known as? What are some examples?
Congenital
EX:
Hereditary spherocytosis
Thalassemias
Sickle Cell
G6PD
What is Extrinsic Hemolytic Anemia also known as?
Acquired
What are the causes, signs and symptoms of extrinsic hemolytic anemia?
Causes:
Infection (malaria)
Antibody mediated hemolysis (transfusion; Coomb's positive)
Mechanical trauma (prosthetic heart valves)

Signs/Sx:
May have hemolytic crisis symptoms (renal failure --> hemoglobinuria)
Fatigue, pallor, hyperdynamic CV status