Platelet disorders Flashcards

1
Q

What is ITP?

A

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

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2
Q

What is the treatment for ITP?

A

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

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3
Q

What is TTP?

A

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

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4
Q

What types of labs do you see with TTP?

A

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

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5
Q

What are the symptoms seen in HUS v. TTP?

A
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
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6
Q

What is the treatment for TTP?

A

plasmaphoresis

steroids

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7
Q

What are some other causes of thrombocytopenia?

A
heparin-induced thrombocytopenia
aplastic anemia (pancytopenia)
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8
Q

What’s the deal with bernard soulier syndrome?

A

defect of GP1b on platelets. Can’t adhere.

get very little thrombocytopenia, but DO get increased bleeding time

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9
Q

What’s the deal with Glanzmann thrombasthenia?

A

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

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10
Q

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

A

Von Willebrand Disease

Aut dom

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11
Q

What’s the deal with vWF disease?

A

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

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12
Q

What is the treatment of vWF disease?

A

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

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13
Q

What’s the deal with DIC?

A
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
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14
Q

What do you see on labs w/ DIC?

A

decreased fibrinogen
decreased factors 8 & 5
schistocytes
D-dimers

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15
Q

What are some causes of DIC?

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