Hematology 💉 Flashcards
Is ITP preceded by any event?
Usually viral infection
How do you treat hereditary spherocytosis?
Supportive care
Blood transfusion
Splenectomy
What are the sx of megaloblastic anemia (both folate and B12)
Pallor
Glossitis*****
Do kids with chronic anemia have any symptoms?
Not usually because they are used to it and have been compensating
How do you diagnose ITP?
You exclude every other possibility ~diagnosis of exclusion~~~
If there’s bleeding and platelets are low and there’s no other explanation= ITP
When i say frequent or severe infections, you say
Leukocytopenia
What is the most common bleeding disorder of childhood?
Idiopathic Thrombocytopenic Purpura (ITP)
*****
How do you treat von Willebrand disease?
Desmopressin*** (causes release of vWF and factor VIII from endothelium stores)
vWF replacement
What medication increases the risk of venous thromboembolism in people with Factor V Leiden mutation?
Oral contraceptives
35x risk for heterozygous
80x risk for homozygous
What kind of anemia does lead poisoning cause?
Mild normocytic anemia
Anemia is due to hemolysis
How do you treat sickle cell anemia?
Hydroxyurea*****
Treat pain of vaso-occlusive episodes**
Avoid precipitating factors
Stem cell transplant
Supportive
How do you diagnose sickle cell anemia?
Hemoglobin electrophoresis: must see HbS
What happens with von Willebrand disease?
Decrease/impairment of von Willebrand Factor (vWF)
=platelets don’t clump
Do people with G6PD deficiency have hemolysis going on all the time?
No, it occurs episodically due to oxidative stress
What does protein C do?
It deactivates factors V and VIII when you don’t need them anymore
What is the most common ~inherited~ bleeding disorder?
von Willebrand disease****
On test!!
(ITP was most common bleeding disorder)
How do you treat DIC?
Treating the triggering event
Replacement of coagulant factors
Anticoagulation therapy when indicated
How do you treat Fanconi anemia?
Supportive care for the anemia, low platelets, and low WBCs
Eventually you need a stem cell transplant
What is hereditary spherocytosis?
A Red blood cell membrane defect that causes the RBCs to have increased osmotic fragility and spherical shapes.
Leads to increased cell lysis and thus, hemolytic anemia
Which pathways are measured with PT (prothrombin time)?
Extrinsic and common pathways
If your 4 year old patient has frequent nosebleeds, what diagnosis should you consider
ITP
How could someone become defeicient in B12
Intestinal MALABSORPTION (crohn, UC, celiac, etc)
Diet insufficiency (vegans)
What factor is deficient in hemophilia B?
Factor IX
How do you treat thrombotic disorders?
Protein C, Protein S, Factor V Leiden, Antithrombin
If they have an episode of VTE, they need to be on anticoagulant prophylaxis for at least 3 months
Anticoagulant prophylaxis:
Unfractionated heparin
LMW heparin
Warfarin (unless they’re pregnant)
What Hgb measurement qualifies as
“anemia” in children 6 months to 5 years?
<11 g/dL
Both male and female
What are the ONLY 2 causes of Megaloblastic anemia?
Vitamin B12 (cobalamin) deficiency
Folic acid deficiency
You already know this but he said it 3 times I don’t want you to forget
If you have Factor V Leiden Mutation you are prone to (Bleeding/clotting)
Clotting
What will happen to PT and aPTT in liver disease or vitamin K deficiency?
Both will be prolonged
What kind of symptoms will be seen with primary polycythemia?
Headache
Lethargy
Splenomegaly
Plethora ?
Hgb as high as 27
What platelet count will you see in Henoch-Schonlein Purpura?
NORMAL or elevated
The Purpura are caused by immune complexes, NOT platelets
What is the most common reason for pain in sickle cell disease?
Vaso-occlusion
Predict the lab values for hemophilia:
Platelet:
PT:
aPTT:
Platelet: normal
PT: normal
aPTT: prolonged
Which kind of macrocytuic anemia will have a high methylmalonic acid?
B12 deficient only
What are 2 pieces of evidence that someone has thrombotic lesions (like in DIC)
Major vessel thrombosis (ex whole leg is thrombosed)
Purpura fulminans (acute, usually fatal, thrombotic disorder that manifests as blood spots, bruising, and discoloration of the skin)
What are the 2 other names for primary polycythemia?
Congenital erythrocytosis
Familial polycythemia
What is the treatment for primary polycythemia aka congenital erythrocytosis aka familial polycythemia?
Phlebotomy ****
What does protein S do?
It is a cofactors for protein C, and facilitates the action of activated protein C
What are the symptoms of Henoch-Schonlein Purpura?
Palpable Purpura ** (immune complexes stuck under skin)
Arthritis
Abdominal pain
Renal disease
If you’re deficient in protein C or Protein S, you will have too much (bleeding/clotting)
Clotting
Who gets hemophilia more often, men or women?
Men. It is X-linked
How will platelet count differ in liver disease vs Vitamin K deficiency?
Liver disease: normal/low
Vitamin K: normal
What drug therapy is monitored by INR?
Warfarin
What sx ONLY occur with B12 deficiency and NOT folate deficiency>
Neurologic signs
Paresthesias, weakness, unsteady gate, decreased vibratory sensation and proprioception on neurologic exam
What causes acquired aplastic anemia?
50% we don’t know
50% medications, toxic exposures, viruses (mono, HIV, hep)
What is vWF?
A protein that binds to factor VIII and is a cofactors for platelet adhesion to the endothelium
What are some pieces of evidence that someone has diffuse bleeding tendency (like in DIC)?
Hematuria
Melena
Purpura/petechiae
Persistent oozing from needle puncture sites
How do you treat ITP?
Avoid medications that mess with your platelets (Aspirin, NSAIDS)**
Prednisone***** (this is autoimmune)
Bleeding precautions
IVIG
Splenectomy
What is the most common nutritional deficiency in children/
Iron
What are the 2 possible causes of macrocytic anemia
B12 deficiency
Folate deficiency
What disorders will cause an increased bleeding time?
Platelet disorders (von Willebrand)
Severe thrombocytopenia
What is INR?
A more accurate PT (standardized)
What will you see on CBC of someone with acquired aplastic anemia?
Anemia, usually normocytic
Low WBC with marked neutropenia
Thrombocytopenia
Low retic count- Bone Marrow is NOT working duh
What are the 2 days someone might become hypoxic leading to secondary polycythemia?
Cyanotic congenital heart disease (Tetralogy of Fallot, TGA, etc)
Chronic pulmonary disease (COPD, CF, etc)
What happens with Henoch-Schonlein Purpura?
IgA immune complexes deposit in the small vessels of the skin, GI tract, and kidneys
IgA IgA IgA IgA IgA IgA IgA IgA
What will you see on the bloodwork in Fanconi anemia?
Progressive Pancytopenia
ALL cell lines are scanty
What are the symptoms of von Willebrand factor disease?
Prolonged bleeding from mucosa: nosebleeds, heavy periods, GI bleeding
Easy bruising
What is DIC?
- Triggering event
- Activation of coagulation cascade
- Microthrombi everywhere
- Massive consumption of platelets, fibrin, and coagulation factors
- Severe bleeding
BASICALLY bleeding out and clotting small vessels all at the same time
Antithrombin makes this drug not work
Heparin
What kind of lab values will you see with ITP?
Platelets:
WBC:
Hgb:
PT:
aPTT:
Platelets: LOW
WBC: normal
Hgb: normal
PT: normal
aPTT: normal
All tests are normal except for platelet count
What will you see on the peripheral smear in lead poisoning?
Basophilic stippling ****
What is NEEDED to diagnose thalassemia?
Hemoglobin electrophoresis
How do you treat lead poisoning?
chelation
What does the severity of thalassemia depend on
Number of gene deletions
What is the treatment of secondary polycythemia?
Correction of underlying hypoxic disorder
Phlebotomy
Which clotting factors are in the intrinsic pathway?
8
9
11
12
What is fanconi anemia?
An inherited bone marrow failure
What is the most common type of small vessel Vasculitis?
Henoch-Schonlein Purpura (HSP)
If you’re giving your patient heparin and its not working what disease should you consider that they might have
Antithrombin deficiency
Where do hemophiliacs commonly bleed?
INTO joints and muscles***
Hemarthrosis= bleeding into a joint
What usually precedes Henoch-Schonlein Purpura?
Upper respiratory infection
Same as ITP
What are the lab findings of iron deficiency anemia:
RBC
MCV
Hgb
Ferritin
RDW
RBC: low
MCV: low
Hgb: <11 g/dL
Ferritin: < 12mcg/L
RDW: Increased ****
What is the difference between primary and secondary polycythemia?
Primary- congenital
Secondary- acquired due to hypoxemia
Predict the lab values for von Willebrand disease:
Platelet:
PT:
aPTT:
Platelet: normal or low
PT: normal
aPTT: normal or prolonged (vWF works with factor VIII)
What will you see in a peripheral smear of G6PD deficienty?
HEINZ BODIES***** (denatured hemoglobin)
“Bite” deformities
Which is more common: hemophilia A or B
A
What does a high vs low reticulocyte count tell you
High: bone marrow is working
Low: trouble
What can happen if you give Warfarin to someone with Protein C deficiency?
Warfarin-induced skin necrosis***
What kinds of things can cause oxidative stress and precipitate a hemolytic episode in someone with G6PD deficiency?
Infection
Drugs
Food
Which factors are in the common pathway?
1
2
5
10
Why does hereditary spherocytosis cause anemia, jaundice, gallstones, and splenomegaly?
The RBCs are delicate and sphere shaped, causing them to rupture all the time.
Many get stuck in the trabeculae of the spleen= splenomegaly
Why is Fanconi anemia often misdiagnosed as ITP?
Because Fanconi’s starts with either low platelets or low WBC, and if they have low platelets, they’ll be bleeding a lot, so ITP explains the easy bleeding.
(But then the anemia and leukopenia appear which is not part of ITP)
What happens with ITP?
**IMMUNE MEDIATED attack against your own platelets:
- The body makes antibodies that bind to platelets
- Platelets are phagocytized by splenic macrophages
- Decreased platelet lifespan
Which specific clotting factors are measured with PTT/aPTT?
1
2
5
8**
9**
10
11**
12**
**indicated intrinsic pathway
What are the risk factors for iron deficiency anemia?
Low SES
Premature/low birth weight
Lead exposure
Exclusive breast feeding beyond 4 months
Weaning to foods that don’t have iron
Feeding problems
How do you get Fanconi anemia?
You inherit it from both of your parents
Autosomal recessive
What are the 3 possible causes of microcytic anemia?
Iron deficiency
Thalassemia
Lead poisoning
What does bleeding time measure?
The time it takes for hemostasis
What are the clinical manifestations of ITP?
Petechiae
Bruising
Nosebleeds
What labs do you need to order for Henoch-Schonlein Purpura?
Platelets (normal or high)
Antistreptolysin O (ASO) titer (high)
Serum IgA (high)
Hemoccult (blood in poo)
Urinalysis (hematuria)
Which specific clotting factors are measured with PT
1
2
5
7***
10
Tissue Factor**
**indicates extrinsic pathway
What is the prognosis for fanconi anemia?
Many succumb to bleeding, infection or malignancy in adolescence
High risk of developing myelodysplastic syndrome or AML
How do you treat hemophilia?
Desmopressin (for Hemophilia A)
Factor replacement (VIII and IX)
Predict the lab values for ITP:
Platelet:
PT:
aPTT:
Platelet: low
PT: normal
aPTT: normal
Which factors are in the extrinsic pathway?
7
Tissue factor
What will you see in the labs of someone with megaloblastic anemia?
MCV:
MCH:
Neutrophil size and appearance:
Shape of RBC:
Homocysteine:
Methylmalonic acid MMA:
MCV: >100
MCH: high
Neutrophils: large with HYPERSEGMENTED NUCLEI**
RBC: big and oval shaped
Homocysteine: high in B12 and folate
MMA: high ONLY WITH B12 deficiency
What is hemarthrosis and what disorder is it associated with
It is bleeding into a joint
Associated with hemophilia*****
**on test
When i say purpura, petechiae and bleeding, you say
Thrombocytopenia
What does the bone marrow look like in someone with aplastic anemia?
“Bleached”
What kind of anemia will thalassemia cause?
Microcytic, hypochromic
Predict the lab values for DIC:
Platelet:
PT:
aPTT:
Platelet: decreased
PT: prolonged
aPTT: prolonged
Everything is fucked up
Which pathways are measured with PTT/aPTT (activated partial thromboplastin time)
Intrinsic and common pathways
What are the sx of ACUTE anemia ?
Lethargy **
Tachycardia**
Irritability in infants**
Pallor
Poor oral intake
If you have antithrombin deficiency, you are prone to (bleeding/clots)
Clots
Which disorders are associated with epistaxis?
ITP
von Willebrand Disease
For all thrombotic disorders (Protein C, Protein S, antithrombin, Factor V Leiden) should you expect to see a family history of thrombosis?
Yes
What will happen if you have a protein C deficiency?
Blood clots
What are the hallmark lab values of DIC?
Elevated D Dimer
Elevated fibrin degradation products
How do you tell hemophilia apart from von Willebrand disease?
Bleeding time is prolonged in von Willebrand disease
What kind of laboratory findings will you see with DIC?
Decreased platelet
Prolonged aPTT and PT
Decreased fibrinogen level
Elevated D-Dimer**
Elevated fibrin degradation products (FDPs)***
What kind of lab values will you see with von Willebrand disease?
PT
aPTT
Factor VIII levels
vWF levels
Bleeding time
PT: normal
aPTT: normal/prolonged
Factor VIII: normal/low
vWF: normal/low
Bleeding time: PROLONGED***
Do people with sickle cell anemia have spleen problems?
Yes, they have splenomegaly and can also have splenic infarcts leading to functional asplenia
What does Bleeding Time screen for?
Platelet dysfunction**
What is acquired aplastic anemia?
Peripheral pancytopenia with a hypocellular bone marrow***
What kind of cells are affected by primary polycythemia?
RBCs ONLY
What factor is deficient in Hemophilia A?
Factor VIII
What are the 2 types of thalassemia
Alpha
Beta
What is the progression of Fanconi’s anemia?
- Low platelets or low WBC
- Low RBCs and severe aplastic anemia
- Bone marrow hypoplasia/aplasia= no cell lines being produced= Emergency
True or false:
ITP is immune mediated
True.
The body makes antibodies against platelets
Screening for anemia is performed at what age?
12 months**
True or false:
Sickle cell anemia causes chronic hemolysis
True, thats why its an anemia duh
What will happen if you treat B12 deficiency with folate only?
Anemia will resolve but the patient will have PERMANENT neurological deficits**
Which clotting factors are dependent on vitamin K?
2 7 9 10
What are the leading causes of death in people with acquired aplastic anemia?
Overwhelming infection
Severe hemorrhage
What lab findings will you see in hemophilia?
Platelet count:
PT
aPTT:
Bleeding TIme:
vWF:
Platelet count: normal
PT: normal
aPTT: Prolonged
Bleeding time: normal
vWF: normal
What kinds of events can trigger DIC?
Sepsis
Trauma
Malignancies
In Sickle Cell disease, are the RBCs always sickle shaped?
Only when deoxygenated
When i say weakness, fatigue and pallor, you say
Anemia
How do you treat iron deficiency in children?
Iron 6mg/kg
divided into 3 daily doses
