A child presents to the ED with bloody

diarrhea and fever. He has elevated BUN on

labs and schistocytes on smear. What is the
diagnosis?
A patient with a mechanical heart valve has a
slightly elevated reticulocyte count and LDH.
Blood smear is abnormal. Explain these
findings.
acanthocyte
Basophilic stippling
Bite cell
elliptocyte
Macro-ovalocyte
Ringed siderblast
A woman with lupus presents with anemia,
spherocytosis on blood smear, and positive
direct Coombs test. What caused the anemia?
Target cell
A teenager with sickle cell anemia presents to
the ED in pain crisis. What other RBC
pathology will you likely see on smear?
A patient who underwent a splenectomy now
has basophilic nuclear remnants in his RBCs.
What are these?
What stain is used to visualize Heinz bodies?

How are bite cells formed?

What do mean corpuscular volume (MCV)
values help diagnose in an anemia work-up?
Normocytic anemias are classified as ____ or
____.
How are normocytic, normochromic hemolytic
anemias further classified?
What are the two types of macrocytic anemia?

Name five causes of microcytic anemia.

Which two disease states miight first appear
as normocytic anemia but then become
microcytic anemia?
Name six causes of hemolytic normocytic
anemia intrinsic to the red blood cell.

Hemolytic uremic syndrome caused by

enterohemorrhagic E. coli(dic,ttp/hus, traumatic
hemolysis-mechanical heart valve prosthesis)
The findings are a result of microangiopathic
hemolytic anemia (heart valve causes
fragmentation of RBCs and labs reflect mild
hemolysis)
Liver disease, abetalipoproteinemia(cholesterol
dysregulation)
Anemia of chronis disease, alcohol abuse, lead
poisoning, thalassemias
G6pd def
hereditary
Megablastic anemia
Excess iron in mitochondria

Autoimmune hemolysis(or hereditary)

HALT-HbC, asplenia, liver, thalassemia
Howell-Jolly bodies (this patient has most likely
autoinfarcted her spleen by this age due to her
sickle cell disease) basophilic nuclear remnants in
rbc
These are Howell-Jolly bodies, which are normally
removed by splenic macrophages
Crystal violet(g6pd def, a-thala- Heinz body-like)
Sulfhydryl groups in hemoglobin are oxidized to
form denatured precipitation and cause phagocytic
damage to the membrane of RBCs
MCV <80 fL is microcytic anemia, MCV >100 fL is
macrocytic anemia, and MCV between those values
is a normocytic anemia
Nonhemolytic; hemolytic
Intrinsic vs. extrinsic to the red blood cell
Megaloblastic and nonmegaloblastic
Iron deficiency (late), anemia of chronic disease,
thalassemia, lead poisoning, and sideroblastic
anemia
Anemia of chronic disease and iron deficiency
anemia
Hereditary spherocytosis, hemoglobin C defect,
G6PD, pyruvate kinase deficiency, sickle cell

Name four causes of hemolytic normocytic

anemia extrinsic to the red blood cell (red
blood cell is not defective).
Name three causes of nonmegaloblastic
macrocytic anemia.
Name three causes of megaloblastic
macrocytic anemia.
A patient who is deficient in copper can have
what sort of anemia?
Name four causes of nonhemolytic normocytic
anemia.
A woman presents with tongue inflammation
and atrophy, dysphagia for solids, and a
hemoglobin of 9 g/dL. What is the cause of her
anemia?
What disease, common in Asia and Africa, is
caused by deletions of the hemoglobin- chain
gene?
What would you see on hemoglobin
electrophoresis in a fetus with deletions of all
four -globin genes?
What are three etiologies of iron deficiency
anemia, and how does iron deficiency affect
red blood cells?
What condition would you expect to see in a
patient with a deletion of three of the four globin genes?
A mother is concerned that her child has
deletions of two -globin genes. What
symptoms will likely manifest?
A man complains of changes in his bowel
habits and bright-red blood in his stools. His
MCV is 68 fL. What is the most likely
diagnosis?
A fetus undergoes amniocentesis and is found
to have deletions of all four -globin genes.
What is the likely outcome?

What are the main treatments for lead

poisoning in adults?
A man who restores old houses presents with
memory loss, abdominal pain, and foot drop.
What is the likely cause of his symptoms?
A new patient states that he has been

anemia, and paroxysmal nocturnal hemoglobinuria

Autoimmune hemolytic anemia, microangiopathic
hemolytic anemia, macroangiopathic hemolytic
anemia, and infections
Liver disease, alcoholism, and reticulocytosis
Folate deficiency, B12 deficiency, and orotic
aciduria
Microcytic sideroblastic anemia
Anemia of chronic disease, aplastic anemia, chronic
kidney disease, and iron deficiency (early)

Plummer-Vinson syndrome (the triad of esophageal

webs, atrophic glossitis, and iron deficiency anemia)
-thalassemia (cis deletion in Asian populations,
and trans deletions in African populations)
Hemoglobin Barts (4)this is a lethal condition
that results in hydrops fetalis
Chronic bleeding, malnutrition or poor absorption,
and high demand (such as pregnancy); iron
deficiency decreases heme synthesis(dec-iron, incr
TIBC, dec- ferritin, fatigue, conjunctival pallor)
Hemoglobin H disease (HbH) in which excess globin forms (4)
Deletion of one or two -globin genes is not
associated with clinically significant symptoms

Iron deficiency anemia (microcytic anemia)this

patient could have a chronic bleed from his colon
Hydrops fetalis (deletion of four genes is
incompatible with life)
Dimercaprol or EDTA are first-line treatments (for
wrist/foot drops); inhibits ferrochelatase and ALA
dehydratase- dec heme synthesis and inc RBC
protoporphyrin(succimer for kids)
Exposure to lead-based paint (encephalopathy,
abdominal pain, and peripheral neuropathy are
signs of lead poisoning)
Lead (lead lines (gingiva-burton lines/long bone

diagnosed with lead poisoning. What are

associated signs/symptoms?
What is the cause of hereditary sideroblastic
anemia, and what is its inheritance pattern?
What is the treatment for sideroblastic anemia
due to a mutation in -ALA synthase?
A child has abdominal pain and gray-blue lines
on his gingivae, MCV is 50 fL, and blood smear
shows basophilic stippling. Diagnosis?
Name reversible causes of sideroblastic
anemia
What disease, seen in people who live in the
Mediterranean region, is caused by point
mutations in hemoglobin- chain genes?
What type of mutation is present in a patient
with -thalassemia minor?
What symptoms and laboratory findings are
consistent with -thalassemia minor?
A two-month-old presents with severe
hemolysis. Hemoglobin electrophoresis shows
increased fetal hemoglobin. What is the likely
diagnosis?
What are the treatment for -thalassemia
major and the possible long-term effects of
that treatment?
A child has -thalassemia major. What
physical exam and x-ray findings do you
expect?
What disease do you expect to see in the child
of a father homozygous for hemoglobin S and
a mother with -thalassemia major?
A patient with -thalassemia major has
increased risk of infection by what bacteria,
leading to aplastic crisis?
What is the cause of basophilic stippling
associated with lead poisoning?
A girl's growth is declining. You suspect lead
poisoning, which disrupts what two enzymes
involved in the formation of hemoglobin?
What treatment is indicated in a child who has
lead poisoning?
Why is the mean corpuscular volume
increased in megaloblastic macrocytic

metaphyses); encephalopathy,erythrocyte
stippling; abdomen pain, sideroblastic anemia;
wrist/foot drop
Defect in the -aminolevulinic acid synthase gene;
X-linked(inc iron, normal TIBC, inc ferritin)
Pyridoxine (vitamin B6) is a cofactor for -ALA
synthase

Sideroblastic anemia from lead poisoning

Alcohol use (most common), lead poisoning, vitamin
B6 deficiency, copper deficiency, isoniazid( genetic,
acquired-myelodysplastic syndromes)
-thalassemia (these mutations are at splicing sites
and promoter regions leading to decreased
synthesis of -globins)
There is a mutation in one gene (heterozygote) in a
promoter or splice site, causing underproduction of
-globin
This condition usually is asymptomatic, and it is
diagnosed by an increased HbA2 (>3.5% on
electrophoresis)
-thalassemia major (HbF [22] is protective in
infants, & infants become symptomatic >6 months
of age)
Treatment is multiple blood transfusions resulting in
secondary hemochromatosis (both genes are
mutated, so no chains are produced)
Crew-cut on skull x-ray (marrow expansion), skeletal
deformities, and chipmunk facies, extramedullary
hematopoiesis(leads to hepatosplenomegaly
Mild-to-moderate sickle cell disease based on
chain production (he will be a hemoglobin S/thalassemia heterozygote)

Parvovirus B19
Lead disrupts the degradation of rRNA, which builds
up and stains with basic dyes
Ferrochelatase and ALA dehydratase (decrease the
synthesis of heme and increase the RBC
protoporphyrin)
Succimer (it ""sucks"" to be a kid who eats lead)
A defect in DNA synthesis causes nucleus to take
longer to mature than the cytoplasm, causing

anemia?
What is the treatment for orotic aciduria?
An anemic man has an MCV of 110 fL and
hypersegmented polys on blood smear. Name
two likely causes and a lab test that
differentiates them. (glossitis)
What is the homocysteine level in patients
with vitamin B12 and folate deficiency?
An 85-year-old cachectic female presents to
ED after a fall. She has megaloblastic anemia.
What two vitamin deficiencies can explain
this?
A 30-year-old man with Crohn disease
presents with chronic low energy. Why is he
susceptible to macrocytic anemia?
Name three drugs than can cause a folate
deficiency and macrocytic anemia.

A student is forgetful and lethargic a few

months after becoming a vegan. What vitamin
deficiency could be causing her symptoms?
Name two conditions in which an increased
demand for folate can result in a macrocytic
anemia.
What autoimmune disorder can result in a
macrocytic anemia?
A man who loves the new sushi bar in town
presents with weight loss and fatigue. Labs
show macrocytic anemia. What is the likely
cause?
An alcoholic with cirrhosis is fatigued,
hemoglobin is 9 g/dL, and MCV is 125 fL. What
is the most likely cause and mechanism of his
anemia?
What change in mean corpuscular volume
(MCV) would you expect in an otherwise
healthy man who has had moderate-to-severe
blood loss?
A woman with HIV has been taking
zidovudine. What type of anemia does this
drug predispose her to?
What three medications can cause
nonmegaloblastic macrocytic anemia?
A 50-year-old man is prescribed PPIs for GERD.

excess cytoplasm and a megaloblast, abnormal cell

divison-pancytopenia
Uridine monophosphate
Vitamin B12 (cobalamin) or folate deficiency; test
for methylmalonic acid, which is elevated only in
vitamin B12 deficiency
Both have elevated homocysteine levels
Vitamin B12 and folate deficiency both cause
megaloblastic anemia (poor diet, malnutritionalcoholics, malabsorption) (vitamin B12 deficiency
causes neurologic symptoms [the fall])
Crohn disease often affects the terminal ileum,
which is where vitamin B12 is absorbed
Methotrexate, trimethoprim, amd phenytoin all
interfere with folate metabolism
Vitamin B12 deficiency causes megaloblastic
anemia and subacute neurologic symptoms(fatty
acid pathway and myelin synthesis- peripheral
neuropathy with sensorimotor dysfunction, dorsal
columns-vibration/proprioception, lateral
corticospinal- spasticity, dementia)
Pregnancy (folate is needed for fetal neural tube
development) and hemolytic anemia (folate is
needed to replenish hemolyzed blood)
Pernicious anemia results in vitamin B12 deficiency
Eating raw fish may lead to infection
with Diphyllobothrium latum, a tapeworm found in
fish/sushi that absorbs vitamin B12
Nonmegaloblastic macrocytic anemia from liver
disease or direct EtOH bone marrow toxicity; low
B12/folate can cause megaloblastic anemia
Increased MCV (the body will compensate with
reticulocytosis, and reticulocytes have higher MCV
than mature RBCs)
Nonmegaloblastic macrocytic anemia (liver disease
and alcohol)
5-fluorouracil, zidovudine, and hydroxyurea
PPIs can cause B12 deficiency, which, in addition to

He later has difficulty walking; physical exam

reveals poor vibration sense. Cause?
How does vitamin B12 cause neurologic
symptoms?
How is does nonmegaloblastic anemia differ
from megaloblastic anemia?
Deficiencies in what enzyme will result in
failure to convert orotic acid to uridine
monophosphate?
Which synthetic pathway utilizes the
conversion of orotic acid to uridine
monophosphate?
What is the inheritance pattern of orotic
aciduria?
What diagnosis is possible in a small child with
megaloblastic anemia unresponsive to B12 or
folate and a normal ammonia level?
A patient presents with vomiting and
confusion; serum shows hyperammonemia
and increased urine orotic acid. What is a
likely diagnosis?
What kind of anemia is associated with
congenital deficiencies in pyrimidine
synthesis?
A man with a prosthetic heart valve has
chronic fatigue. Labs show ___
(increased/decreased) haptoglobin and ___
(increased/decreased) LDH.
A pt presents w/jaundice. Peripheral smear
shows schistocytes. There is increased LDH
and high unconjugated bilirubin. What is the
cause?
Blood smear has schistocytes, increased
reticulocytes. Labs show low haptoglobin, high
LDH, and urobilinogen in urine. What are
causes?
How are normocytic, normochromic anemias
classified?
How do chronic inflammatory processes cause
anemia?
What are the iron and ferritin levels and total
iron-binding capacity in a patient with anemia
of chronic disease?
What are four general etiologies of aplastic

symptoms listed, causes proprioception defect,

spasticity, dementia
B12 is involved in fatty acid pathways and myelin
synthesis
DNA synthesis is not impaired in nonmegaloblastic
macrocytic anemia, as opposed to megaloblastic
anemia, in which DNA synthesis is impaired

Uridine monophosphate synthase

The de novo pyrimidine synthesis pathway

Autosomal recessive
Orotic aciduria (it can also have hypersegmented
neutrophils, glossitis, and orotic acid in urine)

Ornithine transcarbamylase deficiency (orotic

aciduria does not have hyperammonemia)

Orotic aciduria, which results in macrocytic anemia

Decreased; increased (a prosthetic valve causes
mechanical destruction of RBCs and intravascular
normocytic, normochromic hemolytic anemia)
Extravascular hemolysis (i.e., hereditary
spherocytosis)RBCs are cleared by macrophages
in the spleen
PNH, mechanical destruction (aortic
stenosis/prosthetic valve), microangiopathic
hemolysis (he has intravascular hemolysis)
First as hemolytic vs. nonhemolytic, and then by
cause of hemolysis (intrinsic vs. extrinsic) and
location (intravascular vs. extravascular)
The inflammation increases hepcidin levels, which
cause macrophages to retain iron, preventing heme
from forming
Decreased iron level and total iron-binding capacity,
with increased ferritin level
Radiation/drugs, viral infections, Fanconi

anemia?
Name four drugs that can result in aplastic
anemia.
A woman is on a chemotherapy regimen.
What type of anemia is she predisposed to if
she is taking alkylating agents?
A patient with rheumatoid arthritis will at first
have normocytic anemia that can become
____.
What are two possible explanations for
aplastic anemia that is considered idiopathic?
You suspect that a patient has aplastic anemia
due to an autoimmune process. What
treatment can you pursue?
Other than withdrawal of offending agent
(e.g., drugs) or immunosuppressive regimens,
what three treatments for aplastic anemia are
there?
Why do patients with chronic kidney disease
have normocytic, normochromic anemia?
A 47y/o woman w/ recent infection complains
of malaise, mucosal bleeding. On exam, she is
pale and skin is dotted w/petechia. Diagnosis?
What are four viral infections that can cause
aplastic anemia?
A young boy with sickle cells presents with
sudden arm pain and splenomegaly. What
type of anemia is he predisposed to?
Why will a newborn with sickle cell disease
initially be asymptomatic?

What is the pathogenesis of sickle cell

disease?
What evolutionary advantage may explain the
prevalence of sickle cell trait in the AfricanAmerican population?
A patient who is homozygous for sickle cell
disease is found to have microhematuria.
What renal condition is the most likely culprit?
An asplenic sickle cell teenager presents with
meningococcemia. Why?

anemia(dna repair defect), or idiopathic aplastic

anemia (fatigue, malaise, pallor, purpura, mucosal
bleeding, petechiae, infection)
Benzene, chloramphenicol, antimetabolites, and
alkylating agents (chemotherapy)
Aplastic anemia- pancytopenia-anemia, leukopenia,
thrombocytopenia; hypocellular bone marrow with
fatty infiltration-dry bone marrow tap
Anemia of chronic disease (although at first
normocytic, it can become microcytic,
hypochromic)There may be an autoimmune etiology or a primary
defect in the stem cell itself, may fallow acute
hepatitis
Immune suppression using antithymocyte globulin
or cyclosporine
Red blood cell/platelet transfusion,
granulocyte/granulocyte macrophage colonystimulating factor, & allogeneic bone marrow
transplantation
Kidneys produce erythropoietinin renal failure, the
loss of erythropoietin causes anemia
Aplastic anemia likely due to a viral agent
(parvovirus B19, EBV, HIV, HCV)
Parvovirus B19, HIV, Epstein-Barr virus, and viral
hepatitis

Hemolytic normocytic anemia

The newborn still has increased levels of
hemoglobin F and will be asymptomatic until his
hemoglobin F is replaced by hemoglobin S
Low oxygen, dehydration, or acidosis causes
hemoglobin S to polymerize (sickle), leading to
anemia and vaso-occlusive disease-dactylistis
painful hand swelling; point mutation in beta chainglutamic acid to valine position 6
Patients with sickle cell trait (heterozygotes) are
resistant to malaria8% of African-American
population carry this trait
Microhematuria from medullary infarcts (can also
have renal papillary necrosis from decreased O2 in
papilla)
Sickle cell pts are at increased risk for infection by
encapsulated organisms, secondary to splenic

What treatment options are available for

patients with sickle cell disease?
What manifestation of sickle cell anemia is
also seen in patients with thalassemia?
A 19y/o man with sickle cell disease has his
blood drawn. What would you expect to see
on blood smear considering his splenic
dysfunction?
A patient with known sickle cell disease
presents with swelling and tenderness about
the femur. What do you suspect and which
organism?
What is the most common cause of death in
adults with sickle cell disease?
Hereditary spherocytosis, pyruvate kinase
deficiency, HbC,and sickle cell are ____
(intravascular/extravascular) forms of
hemolytic anemias.
Paroxysmal nocturnal hemoglobinuria is an
____ (intravascular/extravascular) form of
hemolytic anemia
Which X-linked recessive disorder of RBCs is
both intravascular and extravascular type of
hemolytic anemia?
What organ is responsible for the anemia seen
in hereditary spherocytosis and why?
A woman with hereditary spherocytosis has a
parvovirus B19 infection. Why is this
important?
What lab test do you order to confirm the
diagnosis in a patient who has spherocytes on
peripheral smear and splenomegaly on exam?
The peripheral smear of a patient shows
small, round RBCs w/o central pallor, and
positive osmotic fragility test. What is the
treatment?
What mutations are responsible for the red
blood cell morphology in hereditary
spherocytosis?
What are the RBC indices that define
hereditary spherocytosis?

dysfunction and eventual autosplenectomy; aplastic

cris parvo b19, splenic sequestration crisis
Patients can be treated with hydroxyurea, which
increases hemoglobin F concentration, or can
undergo bone marrow transplantation; diagnosed
with hemoglobin electrophoresis
Crew cut on skull x-ray from increased
erythropoiesis and bone marrow expansion,
crescent shaped rbc
Howell-Jolly bodies (early splenic dysfunction occurs
in childhood) incre risk of infection with
encapsulated organisms

The patient likely has osteomyelitis

from Salmonella infection
Acute chest syndrome (other manifestations include
avascular necrosis and stroke)

Extravascular

Intravascular

G6PD deficiency
The spleen; the small, inflexible membrane causes
the spleen to remove affected red blood cells
prematurely
Parvovirus B19 can cause aplastic crisis in a patient
with hereditary spherocytosis
Osmotic fragility test (eosin-5-maleimide binding
test is useful for screening)

Treat with splenectomy (this patient has hereditary

spherocytosis)
Ankyrin, band 3, protein 4.2, spectrin (these
proteins interact with the RBC skeleton and plasma
membrane to preserve biconcavity)
Increased MCHC and RDW, normal-to-low MCV

In G6PD deficiency, what is the mechanism for

pathology?
You prescribe TMP-SMX for a urinary tract
infection; the patient develops back pain,
jaundice, and hemoglobinuria. What did you
overlook?
What is the most common enzymatic disorder
of RBCs and its mode of inheritance?
A patient is newly diagnosed with G6PD
deficiency. What clues would his blood smear
contain?
A newborn has kernicterus, and metabolic
screening is abnormal. What error in
metabolism may be the cause, and what is its
inheritance?
What mutation produces hemoglobin C, and
what type of anemia does it cause?
What is the significance of the hemoglobin C
mutation?
What is the mechanism of hemolysis in
paroxysmal nocturnal hemoglobinuria?
A patient complains of dark urine on waking.
Flow cytometry shows no CD55 or CD59
markers on RBCs. She is more predisposed to
develop what?
What is the triad associated with paroxysmal
nocturnal hemoglobinuria, and treatment?
What is the mutation that causes sickle cell
disease?
An adolescent with atypical pneumonia and
fatigue has a hemoglobin of 11 g/dL. What is
the likely cause?
How is a direct Coombs test performed and
what is a positive test?
A patient with a mechanical mitral valve and
symptoms of anemia has an abnormal blood
smear. What do you see and why?
You note schistocytes, or helmet cells, on a
peripheral smear. What mechanism causes
this deformity?
Name four conditions that may cause helmet
cells, or schistocytes, on peripheral blood

The depletion of glutathione increases susceptibility

to oxidant stress leading to hemolytic anemia
Diagnosis of G6PD deficiency; sulfa drugs, some
antimalarials, fava beans, and infections can cause
oxidant stress, triggering hemolysis
G6PD deficiency; X-linked recessive
Heinz bodies and bite cells (results from oxidant
stress [fava beans, sulda drugs, antimalarial,
infections]think stress makes me eat bites of fava
beans with Heinz ketchup) back pain
Pyruvate kinase deficiency (autosomal recessive)
causes low ATP in RBCs, leading to rigidity of the
membranes and destruction by the spleen
Point mutation at position 6 of the -globin chain
substituting glutamic acid with lysine; intrinsic
normocytic hemolytic anemia
Patients with hemoglobin S and C mutations have
milder sickle cell disease than have homozygotes
for hemoglobin S
Intravascular hemolysis due to defective decayaccelerating factor GPI anchor, which normally
prevents complement-mediated destruction of RBCs
She has paroxysmal nocturnal hemoglobinuria, an
acquired mutation in a hematopoietic stem cell with
a higher incidence of acute leukemias
Nnegative Coombs test, pancytopenia, and venous
thrombosis; eculizumab
Point mutation at position 6 of the -globin chain
substituting glutamic acid with valine
Mycoplasma pneumonia infection can cause cold
agglutinin, IgM-mediated acute hemolytic anemia
(cold ice creamyummm)
Anti-IgG antibodies (Coombs reagent) are added to
the RBCs, and if they are coated with antibodies,
they will agglutinate (positive test)
Schistocytes; prosthetic heart valves can cause
macroangiopathic anemia due to traumatic
mechanical destruction
Damage to red blood cells as they pass through a
narrow or obstructed vessel lumen (seen in
microangiopathic hemolytic anemias)
Disseminated intravascular coagulation, thrombotic
thrombocytopenic purpurahemolytic-uremic

smear.
A patient with aortic stenosis may suffer from
____ (microangiopathic/macroangiopathic)
anemia.
Name two infections that cause hemolysis but
are not associated with agglutinins.
How is an indirect Coombs test performed and
what is a positive test?
Name three conditions that are associated
with warm agglutinin (immunoglobulin G
mediated) autoimmune hemolysis.
What are two infections that can present with
hemolytic anemia due to cold agglutinins?
A woman with systemic lupus erythematosus
has fatigue; hemoglobin is 9 g/dL and MCV is
92. What is a likely cause of her anemia?
What is the role of ferritin in the body?
What is the relationship between transferrin
and total iron-binding capacity?
What is the evolutionary theory behind the
etiology of anemia of chronic disease?
Pregnancy and oral contraceptive pills cause
what changes to iron handling in the body?
A woman with menorrhagia has fatigue and
dizziness. Her hemoglobin is 10 g/dL and MCV
is 82. What is her diagnosis and other relevant
labs?
A middle-aged man has a bronze skin color
and cirrhosis. What labs will reflect his
disease?
An asymptomatic man with Crohn disease has
a routine exam. Hemoglobin is 12 g/dL, and
MCV is 90. What other lab values are relevant?
What causes the absolute neutrophil count to
be <1500 cells/mm3(neutropenia)
HIV, DiGeorge syndrome, SCID, lupus,
radiation, sepsis, and operations cause what
sort of leukopenia?
What two causes lead to a low eosinophil
count?
How do corticosteroids lead to neutropenia,
eosinopenia, and lymphopenia?

syndrome, lupus, & malignant hypertension

Macroangiopathic
Intracellular pathogens, like malaria and babesia,
can lyse the erythrocyte and cause hemolytic
anemia
Normal RBCs added to serum agglutinate if antiRBC surface Ig exists when the anti-Ig antibodies
(Coombs reagent) are added (positive test)
Systemic lupus erythematosus, chronic lymphocytic
leukemia, & drug reactions (e.g., -methyldopa)
(warm weather is great"")
Mycoplasma pneumoniae infection and
infectious mononucleosis; these are IgM-mediated
(""cold ice cream yummm"") CLL
Warm agglutinin autoimmune hemolytic anemia, a
chronic anemia that is IgG mediated (warm weather
is great) sle cll, drugs-alpha-methyldopa
It is the primary iron storage protein of the body
Total iron-binding capacity is an indirect measure of
transferrin, which transports iron in the blood
Pathogens need iron to thrive, and sequestration of
iron in macrophages may prevent pathogens from
reproducing
Normal serum iron/ferritin, TIBC is increased
(estrogen stimulates production of binding
proteins), transferrin saturation percentage is low
Serum iron and ferritin are low, TIBC high,
transferrin saturation percentage very low (this is
iron-deficiency anemia)
High serum iron and ferritin, transferrin saturation
percentage is very high in patients with
hemochromatosis, TIBC is low
Serum iron is low, TIBC/transferrin low, ferritin high,
transferrin saturation percentage normal (this is
anemia of chronic disease)
Sepsis/infection, drugs (e.g., chemotherapy),
aplastic anemia, lupus, radiation
Lymphopenia in which the absolute lymphocyte
count is <1500 cells/mm3, or in children, <3000
cells/mm3
Cushing syndrome and use of corticosteroids
Prevent PMN adhesion molecule activation (no
migration to inflamed sites), keep eosinophils in

lymph nodes, cause lymphocyte apoptosis

A man with abdominal striae and moon facies
is diagnosed with Cushing syndrome. What
abnormality in his leukocyte count would be
expected?
What enzyme is defective in acute
intermittent porphyria?
What environmental exposures are common
sources of lead poisoning in adults? In
children?
In porphyria cutanea tarda, there is a defect in
the enzyme ____, resulting in an accumulation
of ____, causing ____-colored urine.
What cofactor is necessary for the ratelimiting step in heme synthesis and why?
Which step in heme synthesis requires iron?
How does heme provide negative feedback for
its own synthesis?
A woman who works in an ammunition factory
experiences headaches and memory loss.
What is the diagnosis? What is the mechanism
of disease?
Lead poisoning leads to the accumulation of
____ and ____ in the heme synthesis pathway.
A patient recently started phenobarbital and
has abdominal pain, altered mental status,
and urine that turns pink after standing.
Diagnosis?
A man presents with abdominal pain and red
winecolored urine. What treatments are
available for his condition?
A man with anemia has MCV of 82 and rings
around the nucleus of the erythrocyte. What
enzyme is affected in this anemia?

What are the 5P's that stand for the symptoms

associated with acute intermittent porphyria?
You study the first step of heme synthesis.
Adding sugar stalls the reaction. Name the
enzyme(s), substrate(s), cofactor(s), and
product(s).
You add -aminolevulinic acid to cell cultures.
Hours later, porphobilinogen levels rise in the
cells. What happened? Did a reaction occur?
Radiolabeled porphobilinogen is added to cell

Eosinopenia
Porphobilinogen deaminase
Adults are exposed to lead in the workplace, such
as battery, ammunition, radiator factories; children
are exposed via lead paint

Uroporphyrinogen decarboxylase; uroporphyrin; tea

Vitamin B6; needed to convert glycine and succinylCoA to -aminolevulinic acid
The final step, where protoporphyrin is converted to
heme by ferrochelatase
Heme inhibits ALA dehydratase, the second step in
heme synthesis
Lead poisoning; lead inhibits ferrochelatase and ALA
dehydratase, enzymes of the heme pathway, and
substrates accumulate
Protoporphyrin and -ALA (in blood)
Acute intermittent porphyria (symptoms are
abdominal pain, wine-colored urine,
polyneuropathy, & psych issues, often precipitated
by drugs)
He has acute intermittent porphyria and needs
glucose or heme, both of which inhibit ALA synthase
to decrease buildup of heme precursors
-ALA synthase, which is the rate-limiting step of
heme synthesis, converting glycine and succinylCoA to aminolevulinic acid
Painful abdomen, port wine-colored
urine, polyneuropathy,psychological
disturbances, precipitation by drugs, alcohol, and
starvation

-aminolevulinic acid synthase; glycine and

succinyl-CoA; vitamin B6; -aminolevulinic acid
In heme synthesis, -ALA exits mitochondria and is
converted into porphobilinogen by -ALA
dehydratase; yes
Heme synthesisporphobilinogen converts to

cytoplasms. Later, you also find radiolabeled

uroporphyrinogen III there. What happened?
You infuse a new, synthetic derivative of
uroporphyrinogen decarboxylase into a
patient with porphyria cutanea tarda
blistering cutaneous photosensitivity. Will this
cure the pt?
You reverse a woman's porphyria cutanea
tarda and note increased cytosolic
coproporphyrinogen III. How is this molecule
converted into heme?
What pathologic process occurs if there is a
defect in one of the steps of heme synthesis?
What are the differences in neurologic
outcomes between adults and children with
lead poisoning?
What three substances accumulate in acute
intermittent porphyria?
What coagulation factors are evaluated when
you order a prothrombin time test?
What coagulation factors are evaluated when
you order a partial thromboplastin time test?
Hemophilia A is caused by a deficiency of ____;
hemophilia B is caused by a deficiency of ____.
What is the treatment for a patient with
hemophilia A?
A man with a history of easy bruising develops
a large swollen joint. Arthrocentesis shows
blood. What underlying disease is responsible?
How does vitamin K deficiency affect
prothrombin time (PT) and partial
thromboplastin time (PTT)? What are the
changes in their values?
What are the prothrombin time (PT) and
partial thromboplastin time (PTT) results in a
patient with hemophilia (A or B)?
A patient comes in with fever, hallucinations,
and altered mental status. Labs show anemia,
proteinuria, and thrombocytopenia. Diagnosis?
What is the role that von Willebrand factor
multimers play in the development of
thrombotic thrombocytopenic purpura?
A patient has petechiae and recurrent
epistaxis. Blood smear shows no platelet
clumping. What will bleeding time and platelet

hydroxymethylbilane via porphobilinogen

deaminase, which then converts to
uroporphyrinogen III

Yes, because this enzyme may help to convert

uroporphyrinogen III (which builds up in porphyria
cutanea tarda) into coproporphyrinogen III
Coproporphyrinogen III enters mitochondria,
converts into protoporphyrin, then converts into
heme via ferrochelatase (with Fe2+cofactor)
Porphyria, in which heme precursors accumulate
Adults typically have headaches, memory loss, and
demyelination, but children have mental
deterioration
Porphobilinogen, -ALA, and coporphobilinogen (in
urine)
Factors I, II, V, VII, and X, the extrinsic pathway
All coagulation factors except VII and XIII, the
intrinsic pathway
Factor VIII; factor IX
Recombinant factor VIII
Hemophilia, which causes spontaneous
hemarthrosis, a macrohemorrhage
Both PT and PTT are elevatedvitamin K is
necessary for the synthesis of factors II, VII, IX, and
X and proteins C and S
PT is normal and PTT is elevated, because
hemophilia affects the intrinsic pathway
Thrombotic thrombocytopenic purpura (symptoms
are pentad of neurologic & renal, fever,
thrombocytopenia, microangiopathic hemolytic
anemia)
The large vWF multimers cause increased platelet
aggregation and thrombosis
Bleeding time is increased and platelet count is
normal (this is Glanzmann thrombasthenia)

count show?
A patient w/purpura and menorrhagia has low
platelets. Antiplatelet antibodies are found.
What is the diagnosis, and what may trigger
it?
A woman with schistocytes on blood smear
and high LDH and bleeding time is diagnosed
with thrombotic thrombocytopenic purpura.
Treatment?
What enzyme is deficient in patients with
thrombotic thrombocytopenic purpura?
A patient has bleeding from the gums,
epistaxis, petechiae, and purpura. What is the
likely defect in coagulation?
What is the mechanism by which BernardSoulier disease causes a defect in the
interaction of platelets?
Glanzmann thrombasthenia causes a defect in
what platelet interaction?
What is the antibody target in immune
thrombocytopenia? What is characteristically
elevated on labs?
What coagulation test will indicate a problem
with platelet function?
Explain how desmopressin works to treat von
Willebrand disease.
What is the most common inherited bleeding
disorder? Is it autosomal dominant or
recessive? How is it diagnosed and treated?
A mnemonic for the seven most common
causes of DIC is stop makingnew thrombi.
What does it stand for?
In DIC, there is ____ (increased/decreased) Ddimer, ____ (increased/decreased) fibrinogen,
____ (increased/decreased) factors V and VIII.
How is a bleeding state brought about in
disseminated intravascular coagulation?
A woman develops severe postpartum
bleeding. Labs show increased PT, PTT, and
bleeding time and decreased platelet count.
Diagnosis?
Von Willebrand factor carries/protects what
factor of the coagulation pathway?
In von Willebrand disease, how is platelet plug
formation defective?
A patient with lifelong bleeding problems has

This is immune thrombocytopenia, involving

decreased platelet survival and increased marrow
megakaryocytes; viral illnesses may trigger it

Exchange transfusion, steroids

ADAMTS 13, a metalloprotease that degrades von
Willebrand factor multimers
These describe the microhemorrhages associated
with a platelet disorder in which there may or may
not be a decreased number of platelets
A decrease in the molecule glycoprotein Ib for
platelet-to-vWF adhesion results in defective
formation of platelet plugs
Poor platelet-to-platelet aggregation in the platelet
plug due to decreased glycoprotein IIb/IIIa
Antibodies for glycoprotein IIb/IIIa causing plateletantibody complex destruction by splenic
macrophages; megakaryocytes are elevated
Bleeding time, which will be increased
It helps release von Willebrand factor stored in
endothelium
Von Willebrand disease; dominant (varying severity,
typically mild); ristocetin cofactor assay; DDAVP
(desmopressin)
Sepsis (gram negative), trauma, obstetric problems,
acute pancreatitis,malignancy, nephrotic syndrome,
and transfusion

Increased; decreased; decreased

There is a deficiency in clotting factors from
widespread activation of clotting, resulting in a
state of bleeding

Disseminated intravascular coagulation

Factor VIII
There is decreased vWF available for platelet-tovWF adhesion
The treatment for her condition (von Willebrand

an elevated PTT and bleeding time. How does

the treatment of choice for her condition
work?

What is the pathogenesis of factor V Leiden?

Name the most common cause of inherited
hypercoagulability.
Why does protein C or S deficiency lead to a
hypercoagulable state?
In what states can antithrombin deficiency be
acquired?
A woman with hypercoagulability started on
heparin continues to have a normal PTT. What
is a possible cause of her lack of response?
A woman develops purpura and bullae on her
legs after 10 days of warfarin. What is she at
risk for with this reaction to warfarin?
Why are venous clots formed in prothrombin
gene mutation?
A trauma patient has lost a significant amount
of blood. What is the most appropriate blood
product to administer? What is its effect?
A patient with an uncontrollable nosebleed is
found to have a platelet count of
<15,000/mm, What blood product would be
therapeutic?
A patient has numbness and tingling in his
fingers and toes after receiving multiple blood
transfusions during surgery. What is the
cause?
What transfusion therapy is used to treat
fibrinogen and factor VIII deficiencies? Why?
What are the risks of blood transfusion?
A patient with liver cirrhosis has increased
bleeding following a surgical procedure. What
blood product would be therapeutic?
How does tumor location differ between
leukemia and lymphoma?

What is a leukemoid reaction?

disease) is DDAVP (desmopressin), which promotes

vWF release from endothelium
There is a mutation in factor V, making it
undegradable by protein C, leading to a
hypercoagulable state
Factor V Leiden
There is an inability to inactivate clotting factors V
and VIII
When a patient has renal failure or nephrotic
syndrome, antithrombin may be lost in urine,
allowing factors II and X levels to rise
She has antithrombin (AT) III deficiency and will not
have the expected increase in PTT, because the
mechanism of heparin depends on AT III

Protein C deficiency (protein C cancels coagulation)

There is a mutation in the 3 untranslated region
leading to increased prothrombin production and
thus venous clots
Packed red blood cells; they increase hemoglobin
and oxygen-carrying capacity
Platelets, transfusion of which increases platelet
count in thrombocytopenia and in qualitative
defects
Citrate, an anticoagulant in stored blood, is a
calcium chelator and can lead to hypocalcemia after
multiple transfusions
Cryoprecipitate; because it contains fibrinogen,
factor VIII, and factor XIII
Infection, transfusion reactions, hypocalcemia,
hyperkalemia, iron overload
Fresh frozen plasma, which will increase the
coagulation factors that are deficient in cirrhosis (as
these are produced in the liver)-DIC, warfarin
overdose
Leukemia cells usually are found in blood & bone
marrow, whereas lymphoma cells are lymphoid
masses (presentation may blur definitions)
An increase in white blood cells with left shift and
increased leukocyte alkaline phosphatase, usually
due to infections

A man with CML has ___ (increased/decreased)

leukocyte ALP; a patient with a leukemoid
reaction has ___ (increased/decreased)
leukocyte ALP.
A man has pneumonia, abnormal complete
blood count (CBC), and increased leukocyte
alkaline phosphatase. What cell type is high
on the CBC?
A patient with night sweats, low-grade fevers,
weight loss, and new palpable lymph nodes
likely has what type of lymphoma?
Which viruses are associated with Hodgkin
and non-Hodgkin lymphomas, respectively?
How do the demographics of Hodgkin's and
non-Hodgkin's lymphoma differ?
How does the pattern of lymph node
involvement differ between Hodgkin and nonHodgkin lymphomas?
A 57-year-old man has night sweats and
weight loss. Reed-Sternberg cells are found
under microscopy. How do you predict
prognosis?
Typically, non-Hodgkin lymphoma is a tumor
derived from what type of cell?
What is characteristically found on biopsy to
help diagnose Hodgkin lymphoma?
What is the CD profile of Reed-Sternberg cells?
A patient with Hodgkin lymphoma with strong
stromal or lymphocytic reaction against ReedSternberg cells has ____ (better/worse)
prognosis.
Of the Hodgkin lymphoma subtypes, which
type is most common, which has the best
prognosis, and which has a poor prognosis?
Which variant of non-Hodgkin lymphoma is
associated with t(14;18)/bcl-2 expression?
What is the expected course in a patient who
has follicular lymphoma?
Which variant of non-Hodgkin lymphoma is
associated with t(8;14)/c-myc expression?
What is the gene product of the translocation
associated with Burkitt lymphoma?

Decreased; increased
Band forms of polymorphonuclear leukocytes (the
increased leukocyte alkaline phosphatase suggests
leukemoid reaction)
Hodgkin (the patient has B symptoms and localized
nodal involvement)
Epstein-Barr virus is associated with 50% of Hodgkin
lymphoma cases; HIV and immunosuppression are
associated with non-Hodgkin
Hodgkin has bimodal distribution (young adults,
>55y/o), men > women (except nodular sclerosing
type); some non-Hodgkin peak at 2040y/o
Hodgkin involvement is localized, rarely extranodal,
contiguous spread; non-Hodgkin, multiple,
peripheral, extranodal, noncontiguous spread
Stage is the strongest predictor of prognosis
(Hodgkin lymphomas have better prognoses than
non-Hodgkin lymphomas0
B lymphocytes, except for the few that have
lymphoblastic T-cell origin
Reed-Sternberg cells
CD30+ and CD15+ (2 owl eyes [CD's]
15 = 30)binucleate and bilobed

Better
Nodular sclerosing form is most common; best
prognosis in lymphocyte-rich form; poor prognosis
in lymphocyte-mixed or -depleted forms
Follicular lymphomathere is a translocation of
heavy-chain Ig (14) and bcl-2 that inhibits apoptosis
in this B-cell lymphoma
Indolent course (patients experience painless
waxing and waning lymphadenopathy)
Burkitt lymphoma, a B-cell lymphomathere is a
translocation of c-myc(8) and heavy-chain Ig (14)
The c-myc oncogene moves next to heavy-chain
immunoglobulin gene promoter and is produced in

large quantities, starry sky-sheets of lymphocytes

with interspersed macrophages; jaw lesions in
endemic form Africa and peliv or abdomen in
sporadic form
A young boy presents with recent fatigue.
What infectious etiology is associated with
Burkitt lymphoma?
An 80-year-old man presents with fevers and
weight loss. What type of non-Hodgkin
lymphoma usually affects older men?
A patient with back pains is diagnosed with
non-Hodgkin lymphoma. A t(14;18) anomaly
was detected. Which lymphoma type is the
diagnosis?
A patient's lymphoma immunohistochemistry
reveals t(11:14) and CD5 positivity. What is
the diagnosis?
Name two non-Hodgkin lymphoma subtypes
that are associated with mature T cells.
A patient presents with multiple cutaneous
lesions, and a biopsy is consistent with
mycosis fungoides. What is the prognosis?
A Caribbean patient presents with multiple
skin lesions; lab tests reveal HTLV-1 infection.
What malignancy is probable?
Which non-Hodgkin lymphoma of mature Bcell origin is the most common in adults?
Name four non-Hodgkin lymphoma subtypes
that are associated with mature B cells.
A patient presents with lower back pain. He
has anemia, elevated creatinine, and elevated
calcium. What diagnosis do you consider?
Name a characteristic finding of multiple
myeloma that can be seen on peripheral blood
smear.
Bone marrow biopsy of a 55-year-old man
shows proliferation of plasma cells with a
fried-egg appearance. What is the likely
diagnosis?
What is the difference between the
immunoglobulins (Ig) involved in Waldenstrm
macroglobulinemia vs. those in multiple
myeloma?
A 65-year-old man has an M spike on serum
protein electrophoresis. He is asymptomatic.
What is the likely diagnosis?

Epstein-Barr virus

Mantle cell lymphoma

Diffuse large B-cell lymphoma (patients have lytic

bone lesions and hypercalcemia)
Mantle cell lymphoma, a type of non-Hodgkin
lymphoma having a translocation of heavy-chain Ig
(14) and cyclin D1 (11)
Adult T-cell lymphoma and mycosis
fungoides/Szary syndrome
Mycosis fungoides, or Szary syndrome, is a T-cell
lymphoma seen in adults that has an indolent
course, CD4+; cutaneous pathches/palques/tumors
Adult T-cell lymphoma, a neoplasm associated with
IV drug abuse and seen in Japan, West Africa, the
Caribbean- lytic bone lesions, hypercalcemia
Diffuse large B-cell lymphoma, only 20% of which
are found in children
Burkitt lymphoma, diffuse large B-cell lymphoma,
mantle cell lymphoma, and follicular lymphoma
Multiple myeloma (crab = hypercalcemia, renal
insufficiency, anemia,bone/back pain)
Rouleaux formation, in which red blood cells appear
to be stacked like poker chips
Multiple myeloma, a plasma cell neoplasm and the
most common primary bone tumor in patients older
than 4050 years of age
M spike of Waldenstrm macroglobulinemia &
overproduction of IgM; in multiple myeloma,
increased IgG (55%) or IgA (25%) occurs
Monoclonal gammopathy of undetermined
significance (MGUS); the condition may progress to
multiple myeloma (1%2% per year) serum
monoclonal protein <3g/dl and bone maroow with

What noninvasive tests are ordered in a

patient suspected to have multiple myeloma?
Waldenstrm macroglobulinemia and multiple
myeloma both have an M spike on protein
electrophoresis. What differs in their
presentation?
A patient with multiple myeloma presents with
a fever. Why is this significant?

What does MGUS stand for and what is its

significance?
What is the most common etiology of renal
failure in multiple myeloma?
A patient diagnosed with breast cancer
receives chemotherapy and radiation after
surgery. What is she at risk of developing?
A pt has finished his final round of
chemotherapy. Blood smear shows neutrophils
with 2 nuclei connected by chromatin. What is
this called?
A patient has unregulated growth of
leukocytes in the bone marrow, anemia, and
thrombocytopenia. What is a possible
diagnosis?
An adolescent male with a mediastinal mass
(leukemic infiltration of the thymus) and
immunohistochemistry of bone marrow shows
TdT+ and CD10+ cells. What is the diagnosis?
What are the common sites for metastases in
acute lymphoblastic leukemia/lymphoma?
A patient is diagnosed with a mature B-cell
tumor that is TRAP-stain positive (now flow
cytometry). If bone marrow aspiration is
ordered, what would be found?
Which of the leukemias is most responsive to
treatment? What are some cell markers that
can be used to identify it?
Which organs do leukemic cell infiltrates
affect?
A 63-year-old man is newly diagnosed with a

<10% monoclonal plasma cells

Serum and urine protein electrophoresis, peripheral
blood smear-numerous plasma cells with clock face
chromatin and intracytoplasmic inclusions
containing immunoglobulin, radiographs
Hyperviscosity symptoms are more common in
Waldenstrm macroglobulinemialytic lesions are
characteristic of multiple myeloma
Multiple myeloma produces nonfunctional
immunoglobulins, increasing the possibility of
opportunistic infections due to immunocompromise
Monoclonal gammopathy of undetermined
significance; it is a monoclonal plasma cell
expansion and asymptomatic precursor to multiple
myeloma
Bence Jones proteins (immunoglobulin light chains)
in the urine are directly toxic to the tubules (primary
amyloidosis)
Possibly ineffective hematopoiesis, defective cells in
nonlymphoid line, and risk of transformation to AML;
benzene

PseudoPelger-Huet anomaly
Leukemia (patients have marrow failure and
variable amounts of WBCs and RBCs in the blood,
leading to anemia, infections, and low platelets)
Acute lymphoblastic leukemia/lymphoma, both
associated with Down syndrome (TdT is a marker of
pre-B and pre-T cells, CD10 of pre-B cells)increase
lymphoblasts age less than 15
Central nervous system and testes
This patient has hairy cell leukemia, which causes
bone marrow fibrosis and, therefore, dry tap on
aspiration; filamentous, hair like projections; tx:
cladribine (2CDA), an adenosine analog (inhibits
adenosine deaminase)
Acute lymphoblastic leukemia/lymphoma; cells may
be TdT+ (a marker of pre-T and pre-B cells) and
CD10+ (pre-B cells)
Liver, spleen, lymph nodes
Small lymphocytic lymphoma/chronic lymphocytic

B-cell neoplasm that is CD20+, CD5+.

Diagnosis? How does this typically present?
What is the difference between small
lymphocytic lymphoma and chronic
lymphocytic leukemia?
Blasts predominate in ____ (acute/chronic)
leukemias.
What diagnosis is confirmed by the presence
of the Philadelphia chromosome (t[9;22])
& bcr-abl)?
What lab test can help distinguish between
chronic myelogenous leukemia (CML) and a
leukemoid reaction in a patient with elevated
WBCs?
A woman with chronic myelogenous leukemia
(CML) suddenly becomes severely ill. How has
her disease course changed?
What translocation is associated with acute
myelogenous leukemia M3 subtype and what
does it respond to?
How does the epidemiology of acute
myelogenous leukemia (AML) differ from
chronic myelogenous leukemia (CML)?
What are Auer rods?
What are risk factors for developing acute
myelogenous leukemia?
What drug is effective for chronic
myelogenous leukemia, and what is its
mechanism of action?
Name the abnormal protein and the
chromosomal translocation that are
associated with chronic myelogenous
leukemia.
What chromosomal translocation is seen in
subtype M3 of acute myelogenous leukemia?
A lymphoma patient has a translocation of
chromosomes 8 and 14, with activation of cmyc. Diagnosis?
What chromosomal translocation is seen in
mantle cell lymphoma?
What chromosomal translocation is seen in
follicular lymphoma?
In Langerhans cell histiocytosis, immature
cells are unable to stimulate ____ via antigen

leukemia; an autoimmune hemolytic anemia that is

often asymptomatic and progresses slowly; smudge
cells
Chronic lymphocytic leukemia has increased
peripheral blood lymphocytosis, or bone marrow
involvement
Acute
Chronic myelogenous leukemia; increase
neutrophils and metamyelocytes and basophils,
spenomegaly
Leukocyte alkaline phosphatase, or LAP score, will
be low in CML and high in leukemoid reaction due to
the maturity of the cells
CML can accelerate and transform to acute
lymphoblastic leukemia/lymphoma or acute
myelogenous leukemia in a blast crisis
t(15;17); responds to all-trans-retinoic acid (vitamin
A) by inducing myeloblast differentiation; DIC is
common and induced by chemotherapy due to
release of auer rods
Median age of onset of AML is 65 years, whereas
median age in CML is 64 years and peak is at 4585
years
Peroxidase-positive cytoplasmic inclusions seen in
M3 AML
Alkylating chemotherapy, radiation,
myeloproliferative disorders, Down syndrome
Imatinib; a small-molecule inhibitor of the bcrabl tyrosine kinase

bcr-abl protein; t(9;22), the Philadelphia

chromosome (Philadelphiacream cheese)
t(15;17) all trans reinoic acid

Burkitt lymphoma
t(11;14), with activation of cyclin D1
t(14;18), with activation of bcl-2
Primary T lymphocytes, child with lytic bone lesions
and skin rash or as recurrent otitis media with a

presentation.
From what cell lineage do the cells that cause
Langerhans cell histiocytosis originate? What
surface proteins do they express?

What is the hematologic profile for

polycythemia vera in terms of RBC, WBC, and
platelet counts?
What three myeloproliferative disorders are
associated with the JAK2mutation (involved in
hematopoietic signaling)?
What is a cause of secondary polycythemia
vera?
A patient with leukocytosis, thrombocytosis,
anemia is found to be positive for Philadelphia
chromosome. What is the treatment?
Which of the myeloproliferative disorders does
not involve hematopoietic growth factor
signaling in JAK2? What is its mechanism?
In what condition(s) do you expect to see
appropriate absolute polycythemia?
In what condition(s) do you expect to see
inappropriate absolute polycythemia?
A patient with polycythemia vera would have
what changes to plasma volume, RBC mass,
O2 saturation, and EPO level?
A child with Wilms tumor has polycythemia.
What changes in plasma volume, RBC mass,
O2 saturation, and EPO do you expect?
A patient with relative polycythemia would be
expected to have what changes in plasma
volume, RBC mass, O2 saturation, and EPO?
A patient moves to Denver. What changes in
plasma volume, RBC mass, O2 saturation, and
EPO do you expect in a few weeks?

mass involving mastoid bone

The cells are dendritic from monocyte lineage; they
express S-100 and CD1a, tennis racket birbeck
granules
All three (RBC, WBC, platelet) are increased.
Hct>55, intense itching after hot shower;
erythromelalgia(severe, burning pain and reddish or
blusih coloration) due to episodic clots in vessels of
the extremities
Polycythemia vera, essential
thrombocytosis(enlarged megakaryocytes in bone
marrow), and myelofibrosis (fibrotic obliteration of
bone marrow,teardrops and immature myeloid line)
Natural or artificial increases in EPO levels

Imatinib (Gleevec) (this patient has CML)

Chronic myelogenous leukemia; bcrabl transformation increases cell division and
inhibits apoptosis
Lung disease, congenital heart disease, high
altitude
Renal cell carcinoma, Wilms tumor, cyst,
hepatocellular carcinoma, hydronephrosis (all
pathologies are due to ectopic erythropoietin)
Increased plasma volume and RBC mass, normal
O2 saturation, decreased EPO due to negative
feedback
Normal plasma volume, increased RBC mass and
EPO levels, normal O2saturation (this response is
due to ectopic erythropoietin)
Decreased plasma volume only (apparent rise in
RBC mass is caused by reduced plasma volume due
to blood loss, dehydration, or burns)
No change in plasma volume, increased RBC mass
and EPO levels, decreased O2 saturation