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)