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Flashcards in Platelet disorders Deck (15):
1

What is ITP?

immune thrombocytopenic purpura
autoimmune, ab against GP2b3a
spleen destroys the platelet-antibody complexes
increase in megakaryocytic in the bone marrow
thrombocytopenia and increased bleeding time

**commonly due to a viral illness

2

What is the treatment for ITP?

steroids
IVIG
splenectomy b/c 1/3 of platelets are sequestered in the spleen

3

What is TTP?

ADAMTS13 vWF metalloprotease deficiency. Can't degrade the vWF multimers
thrombotic thrombocytopenic purpura
excess platelet activation and thrombosis.
consumptive thrombocytopenia
increased bleeding time, get purpura

4

What types of labs do you see with TTP?

schistocytes: moving pass thrombosis, get sheared. Called microangiopathic hemolytic anemia
increased LDH

5

What are the symptoms seen in HUS v. TTP?

HUS more mild, kidney-centered
triad:
hemolysis
uremia
thrombocytopenia

TTP more severe, neuro involvement
HUNT For The Toilet Paper
Hemolysis
Uremia
Neuro symptoms
thrombocytopenia
fever
TTP

6

What is the treatment for TTP?

plasmaphoresis
steroids

7

What are some other causes of thrombocytopenia?

heparin-induced thrombocytopenia
aplastic anemia (pancytopenia)

8

What's the deal with bernard soulier syndrome?

defect of GP1b on platelets. Can't adhere.
get very little thrombocytopenia, but DO get increased bleeding time

9

What's the deal with Glanzmann thrombasthenia?

defect in GP2b3a
can't have platelet aggregation
normal platelet count, but increased bleeding time

10

What is the most common inherited bleeding disorder? How is it inherited?

Von Willebrand Disease
Aut dom

11

What's the deal with vWF disease?

decreased vWF
causes intrinsic pathway problem b/c not there to stabilize factor 8a, increased PTT
causes platelet adhesion problem b/c not there to bind GP1b, increased bleeding time
no thrombocytopenia, or increase in PT

12

What is the treatment of vWF disease?

ADH (desmopressin) given to increase release of vWF from endothelial cells

13

What's the deal with DIC?

disseminated intravascular coagulation
get thrombosis everywhere!!
get thrombocytopenia as a result.
get increased bleeding time.
deficiency of clotting factors by consumption-->increased PT and PTT
risk of multi organ failure

14

What do you see on labs w/ DIC?

decreased fibrinogen
decreased factors 8 & 5
schistocytes
D-dimers

15

What are some causes of DIC?

STOP Making New Thrombi
Sepsis (gram neg)
Trauma
OB
Pancreatitis (acute)
Malignancy
Nephrotic syndrome
Transfusion