Hematology- Jaynstein Flashcards
What is the cut off for macrocytic anemia
>100fl
What is the cut off for microcytic anemia
<80fl
What does a high retic indicate
Hemolysis
Acute blood loss
What is the NR for Plt count
150,000-450,000/mcL
What is the Hgb of a mild anemia
8-10
What is the Hgb level in a moderate anemia
6-8
What is the Hgb in a severe anemia
<6
What is the severity of an anemia largely dependent on
symptoms
What are the s/s of anemia
Fatigue
Tachycardia (due to compensation)
HSM
DOE
Pallor
Bone Tenderness (if applicable to underlying cause)
What do the MCH and MCHC determine
They describe the Hgb concentration- varying levels will tell you if an RBC is normochromic, hypochromic, or hyperchromic
What are the microcytic anemias
IDA
Thalassemia
Anemia of chronic disease (if they have had it long enough)
Sideroblastic anemia
What are the normocytic anemias
anemia of chronic disease (early on)
aplastic anemia
hemolytic anemia
What are the macrocytic anemias (megaloblastic)
B12 deficiency
Folate deficiency
Where is the majority of iron absorbed
duodenum
How is iron transported in the body
Transferrin
What is the storage form of iron
ferritin
Where is iron stored
Liver
Spleen
Bone marrow
Muscle
What is the leading cause of anemia world wide
IDA
What causes IDA
Insufficient dietary intake
Poor absorption of iron
Chronic blood loss
What pts are more prone to IDA
women
What should you always do if a pt presents with IDA
search for evidence of blood loss (don’t forget DRE)
What are the sx of IDA
- Tachycardia
- DOE
- Fatigue
- Pale skin and mucosa
- Brittle nails
- Angular cheilitis
- Pruritis
- Pica
- Anxiety/tingling/numbness
What are the iron studies in order of most to least sensitive for detecting IDA
Ferritin
TIBC
Transferrin Sat
Serum Iron
What does the Ferritin test look for
stored iron
What is the first lab to decrease in IDA
Ferritin
If a pt has a Ferritin of <30mcg/dL with a decrease H/H, what is the diagnosis
IDA
What does the TIBC measure
capacity to bind iron
What are the NR for TIBC
240-450 mcg/dL
Is TIBC increased, decreased, or normal in IDA
increased (less free floating Iron to bind)
Will Transferrin Sat % be high, low, or normal in IDA
Low
What will serum iron levels be in IDA
normal to possibly slightly decreased
Why is serum iron not an ideal test for dx IDA
It can have considerable daily variation so it isn’t that useful
What are the treatments for IDA
PO→Diet or ferrous sulfate tablets
Parental→IV
Why do you want to start a pt on low dose of ferrous sulfate
It is poorly tolerated and upsets GI tract
How long does it take for Hct to get halfway to normal when tx IDA with ferrous sulfate
about 3 weeks
When will tx of IDA with ferrous sulfate return a pt to normal HCT levels
2 months
When would you admin ferrous sulfate IV
If pt is super intolerant PO or has a GI disease inhibiting absorption
Why is it important to ask fhx in a pt with new dx of anemia
To r/o thalassemias
What kind of anemia is a ild to moderate thalassemia
microcytic/hypochromic
What is the retic of a pt with a thalassemia
elevated- bone marrow is attempting to compensate
What are the iron studies for a pt with thalassemia
normal
What disorder is known to show target cells on a smear
Thalassemia
What is the definitive lab to dx thalassemia
Electrophoresis
What are the s/s of a severe thalassemia
Pallor
HSM
Growth failure
Bone deformities
Jaundice
What is the tx for mild thalassemia
No specific tx just monitoring
Folate supplements (to try and aid in synthesis)
Avoid iron supplements and sulfonamides
What is the tx for major thalassemias
May require transfusion
splenectomy
bone marrow transplants
avoid iron/sulfonamides
What is the cure for thalassemia major
allogenic stem cell transplant
What are the complications associated with thalassemias
iron overload
increased risk of infection (especially if tx with splenectomy)
Bone deformities
How do you tx iron overload
chelating agents
What is sideroblastic anemia
Anemia cuased by reduced Hgb synthesis because of failure to incorporate iron
What causes sideroblastic anemia
Alcoholism
lead
copper
zinc
dietary B6 or Cu deficiency
OCP
What is a characteristic sign of lead poisoning associated with sideroblastic anemia
basophilic stippling
What is characteristic on a periph smear for sideroblastic anemia
ringed sideroblasts
and if caused by lead poisoning, basophilic stippling
What is the tx for sideroblastic anemia
rarely requires tx, just tx underlying cause
on rare occasion can transfuse if necessary
What are the causes of megaloblastic anemias
B12 or folate deficiency
What is the most common cause of B12 deficiency
Pernicious anemia
What leads to pernicious anemia
Hereditary loss of gastric cells that help absorb B12
or
GI surgery that leads to loss of gastric cells (bariatric)
What pt population is prone to B12 deficiency and why
Vegans, it is found in foods of animal origin
What are the s/s of vit B12 deficiency
Fatigue
Pallor
CNS complaints- loss of vibratory sense, parasthesias, loss of balance
What labs will be out of range for vit b12 deficiency
elevated MCV
Low B12
What is the tx for B12 deficiency anemia
B12 IM injections
or
cobalamin PO
What does the body use folic acid for
DNA synthesis and repair
What foods contain folate
fruits and veggies
Why is folic acid so important to supplement
you can’t synthesize it and must come from your diet
What drugs can decrease folate absorption
phenytoin
sulfonamides
methotrexate
What are the s/s of folic acid deficiency
regular anemia sx
What labs will be out of NR for folic acid deficiency
Elevated MCV
Low folate
What is the tx for folic acid deficiency
Folic acid supplementation- 1mg PO daily
What is B9
folic acid
What normocytic anemias have low retic counts
anemia of chronic disease
anaplastic anemia
What normocytic anemia(s) have high retics
hemolytic anemia (there is attempted compensation by the marrow)
What are the most common causes of anemia of chronic disease
Chronic infxn/inflammation/cancer/liver disease
or
Renal failure leading to decreased epo production/reduced RBC production
What age group is more prone to anemia of chronic disease
>85 years old
How do you tx anemia of chronic disease
Tx the comorbidity
ir bad enough can tx with transfusion or EPO
What causes aplastic anemia
Bone marrow failure due to injury or abnormal expression of stem cells
What are the autoimmune cause(s) of aplastic anemia
SLE
What kind of trauma leads to aplastic anemia
radiation
chemo
toxins
What kinds of drugs lead to aplastic anemia
sulfonamides
phenytoin
carbamazepine
What are the s/s of aplastic anemia
fatigue
pallor
freq infxn
bleeding
purpura
petechiae
HSM
What are the lab changes associated with aplastic anemia
Pancytopenia
normocytic early on
decreased retic (no compensation)
normal smear just decreased numbers
bone marrow is hypocellular
What is the tx of mild aplastic anemia
blood and plt transfusions
What is the tx of severe aplastic anemia
bone marrow transplant
immunosuppression
What is the prognosis of severe aplastic anemia
typically live about 6 months if not aggresively tx
What do pts with aplastic anemia usually die from
infxn
What are the most common hemolytic anemias
G6PD deficiency
Sickle Cell
Why are sx of hemolytic anemias commonly delayed
Bone marrow production of erythrocytes will increase initially to compensate
What are the sx specific to hemolytic anemia
jaundice
splenomegaly
What will labs show for hemolytic anemias
elevated retic
elevated bilirubin
elevated LDH
urobilinogen
What is the tx for hemolytic anemias
transfusions
What is G6PD deficiency anemia
hereditary enzyme defect leading to episodic hemolytic anemia because RBCs cannot cope with oxidative stress
What is a characteristic of RBCs that is specific to G6PD deficiency
Heinz bodies
What are heinz bodies
markers for RBC destruction as a result of the body’s inability to handle oxidation
What pt population does G6PD def. commonly affect
african american men
What meds/foods can put a pt with G6PD def. into anemia
dapsone
quinine
primaquine
quinidine
sulfonamide
nitrofurantoin
fava beans
What are the s/s of G6PD def
fatigue
splenomegaly
What lab changes will be seen with G6PD Def
elevated bilirubin
elevated retic
heinz bodies
How do you tx G6PD def
avoid known oxidative drugs
How common is sickle cell and who does it affect
1:400 american blacks
What provokes sickle cell crisis
infxn
dehydration
hypoxia
can be spontaneous
What is the typical cause of pain for a pt in sickle cell crisis
ischemia to organs
What are the s/s specifically for sickle cell anemia
chronically ill appearing
jaundice
splenomegaly
systolic murmur
non-healing ulcers
When do s/s of sickle cell usually manifest in a pt
within 6 months of life
What lab changes can be seen in pts with sickle cell
anemia
elevated retic
sickled cells on smear
howell-jolly bodies
target cells
How do you confirm dx of sickle cell
Hgb S on electrophoresis
What is the tx for sickle cell
prophylaxsis with hydroxyurea (stimulates epo)
UTD on vaccines
folic acid supplements
blood transfusions when necessary
Is there a cure for sickle cell
no
How do you tx sickle cell in acute crisis
tx pain
identify and remove ppt
tx infxn and ischemia
admin exchange transfusions
What are the complications associated with sickle cell disease
strokes, PE, DVTs
increased infxn
leg ulcers
osteo-necrosis
gallstones
kidney damage
priapism
splenic squestration/rupture
eye damage
delayed growth
acute chest syndrome
What occurs in primary hemostasis
plt adhesion
activation
aggregation into hemostatic plug
What occurs in secondary hemostasis
clotting factors are activated to form a fibrin complex that then stabilizes the plt plug
What is the only test that evaluates plts
bleeding time
What does the aPTT test
Evaluates the intrinsic pathway
What can prolong the aPTT
heparin
hemophilia
DIC
APS
What does the PT evaluate
The extrinsic pathway
What can prolong the PT
warfarin
liver dysfunction
vit k def
DIC
What does fibrinogen test measure
protein precursor to fibrin
What does a decrease in fibrinogen indicate
greater bleeding risk
What does the d-dimer measure
measures a product of clot breakdown
When would you start to be concerned about a pts plt level
when it gets below 50,000/mL
At what plt level does spontaneously bleeding start to be a concern
about 20,000/mL
What are the ss of thrombocytopenia
vary based on cause but commonly there is:
petechia (<3mm)
purpura (3-10mm)
Ecchymosis (>10m)
Long bleeding time or easy bruising
mild gingival and nasal bleeds
What are the most common causes of thrombocytopenia
decreased plt production
increased plt destruction
sepsis/blood loss
What labs will be abnormal if there is thrombocytopenia due to decreased plt production
bleeding time will be increased
How do you tx thrombocytopenia due to decreased plt production
address underlying cause (remove offending med etc)
bone marrow stimulation
How do you dx thrombocytopenia of decreasd plt production if there is no obvious cause
CA eval
bone marrow asp.
What is idiopathic thrombocytopenic purpura
Autoimmune disease characterized by an abnormal decrease in the number of plts→IgG tagged plts
What pt population is it common and unconcerning to see idiopathic thrombocytopenic purpura in?
children post-viral infxn
it is usually self limited
When is Idiopathic Thrombocytopenic purpura (ITP) concerning
When acquired as an adult- this is less likely to resolve
What is the pt presentation for ITP
rash
mucosal bleeding
easy bruising
How do you dx ITP
Made by an isolated low plt count of less than 10k- dx of exclusion and you have ruled out all other causes
What labs will be altered with ITP
thrmobocytopenia
prolonged bleed time
What is the tx for ITP
in kids- only monitor
BUT
in adults-
if plt is >20k and there is no bleed, dont treat
If plt is <20k or there is a bleed admin prednisone, IVIG and splenectomy if persistent
When would a pt with ITP get a plt transfusion
If they are at severe bleed risk, otherwise it is a pointless tx
What is TTP (thrombotic thrombocytopenic purpura)
Autoimmune process characterized by extensive microscopic intravascular clotting, leading to plt clumping, thrombocytopenia, and easy bleeding
Why does TTP lead to RBC lysis
The thrmobosis leads to shearing of RBCs
What disease can be caused by an ADAMTS13 enzyme defect
TTP
What can trigger TTP
meds
bacterial infxn
pregnancy
SLE
bone marrow transplants
What is the pt demo that commonly gets TTP
90% occur in adulthood
women
What labs will be altered with TTP
Plt count <50,000
Anemia
unconjugated bili
LDH elevation
evidence of renal failure
urine with hematuria and proteinuria
smear with frag RBCs
What is the tx for TTP
EMERGENT-
admit
corticosteroids
pRBCs
plasmapharesis (to get rid of vWF)
ASA
Splenectomy
What usually causes HUS
GI infections of E. coli, shigella, or salmonella
What is the disease process of HUS
bacterial toxins damage vascular endothelium leading to thrombus formation and eventually thrombocytopenia
What organ does HUS usually attack
kidney
What pt population is commonly affect by HUS
kids
What disease should you suspect if a child has renal failure associated with diarrhea
HUS
What is the sx triad of HUS
thrombocytopenia
renal failure
hemolytic anemia
What are the lab changes in HUS
plt count <50k
anemia
uncon. bili
LDH elevated
renal failure signs
blood and protein in urine
frag RBCs
How do you tx HUS
Kids: Admit for observation and give IVF
Adults: tx like TTP
What meds do you want to avoid when tx HUS
Abx- they increase bacterial lysis leading to increase release of toxin and increase clotting
What does HELLP stand for
Hemolysis
Elevated Liver enzymes
Low PLT count
What causes HELLP
complication of pregnancy that usually accompanies preeclampsia or eclampsia
What are the s/s of HELLP
nonspecific:
fatigue
n/v
HA
RUQ abd pain
blurry vision
epistaxis
What are the lab changes associated with HELLP
thrmobocytopenia
low h/h
elevated bili
elevated LDH
elevated LFTs
What complications are associated with HELLP for the mother
1% mortality risk
DIC
renal failure
liver damage
What complications are associated with pregnancy with HELLP
placental abruption
iatrogenic prematurity
How do you tx HELLP
delivery (if 34 weeks or more)
What is DIC
An “acquired bleeding disorder” caused by abnormal acceleration of the coag cascade leading to thrombosis and depletion of clotting factors leading to simulatenous bleeding
bleed and clot happening at once
What are the s/s of DIC
Bleeding from multiple sites
ecchymossis/petechiae/purpura
cool and/or mottled extremities
dyspnea
hematuria
What lab changes are present in DIC
All the bleeding and clotting tests will be abnormal, if you see everything is abnormal think DIC
What is the tx for DIC
supportive care (ABC mgmt, cardiopulm support)
tx underlying d/o
transfuse only if absolutely necessary for bleed
heparin may or may not be helpful (case by case)
What is HIT
The development of thrombocytopenia after the admin of heparin
When does HIT usually form
after about 5 days of admin of heparin
How do you dx HIT
Decrease of plt count by 50% or more with admin of heparin
What are the sx of HIT
Pt are rarely symptomatic but if they display symptoms it is more likely to be clotting symptoms than bleeding
What is the tx for HIT
Stop heparin and initiate another anticoag NOT WARFARIN
Avoid heparin indefinitely
plt tranfusion is not usually necessary
Why do only males get hemophilia
It is a sex linked trait so women are only carriers
What factor deficiency leads to hemophilia A
Factor 8
What factor deficiency leads to hemophilia B
Factor 9
What does the severity of hemophilia depend on
The amount of active protein produced
How does a pt with mild hemophilia usually discover the disease
after a trauma or surgery (no spontaneous bleeds)
What are common sites of random bleeds in pts with hemophilia
joints
soft tissue
urine/stool
brain
What lab values will be abnormal in pts with hemophilia
aPTT- usually prolonged
Factor assay is low (8 or 9 depending on type of hemophilia)
How do you tx hemophilia
avoid drugs that can interfere with clotting- ASA and NSAIDS
prophylaxis with factor replacement before surgery
lifestyle mods
What is the most common congenital coagulopathy
Von Willebrand’s Disease
What are the s/s of Von Willebrand’s Disease
epistaxis
gingival bleeding
menorrhagia
Gi bleed
or incidentally noticed after dental procedure
Is Von Willebrand’s Disease a d/o with a high bleed risk/concern
no
What labs values will be out of range in Von Willebrand’s Disease
prolonged bleeding time (spontaneously or after ASA challenge)
Low plasma vWF
Factor VIII might also be low
What role does vWF play with Factor VIII
It prevents degradation of Factor VIII
What is the tx for Von Willebrand’s Disease
Majority of cases are mild and require little to no tx
avoid ASA
supplement with desmopressin prior to surgery to release any stored vWF
What clotting factor is not produced in the liver
Factor VIII (8)
When trying to diagnose liver dysfunction, what is an effective test
PT/INR- pts with liver issues will have increased levels
If a pt has a coagulopathy of the liver, how can you prophylax them for procedures
tx with FFP transfusion so they can supplement missing factors
What factors require Vit K
Factor 2,7,9, and 10
Why does Vit K deficiency prolong the PT and aPTT
It is involved in both the intrinsic and extrinsic pathway
When should you check for clotting disorders
When a pt has an unprovoked PE or DVT
What are the 4 clotting disorders you can screen for
antiphosphlipid antibody syndrome
Factor V Leiden
Protein C and S deficiency
Polycythemia Vera
What do all clotting disorders put a pt at risk of
DVT
PE
CVA
MI
Miscarriage
What is the acute tx of any and all hypercoagulable states
heparin/lmwh
thrombectomy or thrombolysis if indicated
What is the preventative tx for any and all clotting disorders
anticoagulants or aspirin
Avoiding OCP
Minimizing risk like cigarettes, and mgmt of comorbities
What is antiphospholipid antibody syndrome (APS)
An autoimmune disease in which people produce abnormal proteins called APA that create turbulent and stagnate blood flow leading to thrombi
What disease is antiphospholipid antibody syndrome commonly associated with
SLE
What other diseases are commonly associated with antiphospholipid antibody syndrome
miscarriage
DVT/PE
How do you tx antiphospholipid antibody syndrome
same as tx of all hypercoagulability states (in another notecard)
What is the most common inherited form of coagulopathy
Factor V Leiden
How do you dx factor V leiden
Activated Protein C resistance test
PCR
What is Protein C deficiency
A genetic coagulopathy disease
What lab is used to dx Protein C Deficiency
Protein C Assay
What is Protein S deficiency
A genetic coagulopathy disease
How do you dx Protein S deficiency
Protein S assay
What are the s/s of polycythemia vera
Pruritis (unexplained and provoked by things like showering)
HA
Fatigue
Splenomegaly
What labs can diagnose polycythemia vera
Crit >60%
What is Polycythemia vera
An acquired myeloproliferative d/o causing increased RBC and PLTs leading to hypercoagulability
What is the tx for PV
one unit of blood weekly until crit <45% then PRN
