5-30 KRAFTS Bleeding Disorders Flashcards

1
Q

What are some differences in bleeding caused by platelet issues versus those caused by Factor issues?

A
Platelet bleeding
		Superficial (skin)
		Petechiae (called purpura when coalesce together)
		Spontaneous
	Factor bleeding
		Deep (joints)
		Big bleeds
		Trauma
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2
Q

What is 1 thing you should absolutely remember about Factor bleeding?

A

Can involve deep joint bleeds

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3
Q

What are the 4 things you must know about Von Willebrand Disease?

A

Most common hereditary bleeding disorder
Auto dom
↓vWF –> platelets can’t adhere to endothelium
Variable severity

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4
Q

What are Sx of Von Willebrand Disease?

A

Mucosal bleeding in most (eg nosebleeds)

Deep joint bleeding in severe cases

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5
Q

What lab results would you see in vW diease (bleed time, PTT, INR, etc)

A

↑Bleed time
↑PTT (probably b/c less Factor VIII)
Normal INR (why?)
Abnormal platelet aggregation test

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6
Q

What surface protein on platelets binds vWF?

A

GP Ib

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7
Q

What does Ristocetin make platelets do?

A

Express their GP Ib

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8
Q

Tx of vW Disease?

A

DDAVP (make patient increase their vWF & Factor VII)

Cryoprecipitate (contains vWF and Factor VIII)

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9
Q

What are the 4 things you must know about Hemophilia A?

A

Most common coag factor deficiency
X-linked recessive (but 30% are random mutations)
↓Factor VIII
Variable amount of “factor bleeding”

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10
Q

Sx of Hemophilia A?

A

Factor bleeding – deep joint bleeds, ↑bleed time, mucosal bleed
Severity varies

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11
Q

What lab test results would you expect in Hemophilia A?

A

↑PTT, ↓Factor VIII

Everything else normal

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12
Q

Tx for Hemophilia A?

A

DDAVP

Factor VIII

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13
Q

What are the 4 things you must know about Hemophilia B?

A

↓Factor IX
Less common than A
X-linked recessive
Same clinical and lab findings

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14
Q

What is Bernard-Soulier Syndrome?

A

Abnormal GP Ib –> bad adhesion, big platelets, bleeding

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15
Q

What is Glanzmann Thrombasthenia?

A

No GP IIb-IIIa –> no platelet aggregation, bleeding

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16
Q

What is Gray Platelet Syndrome?

A

No α granules in platelets –> mild bleeding

17
Q

What is δ Granule deficiency?

A

No δ Granules in platelets, can be part of a syndrome

18
Q

What are the 4 things you must know about Disseminated Intravascular Coagulation?

A

Lots of underlying disorders
Something triggers coag  thrombosis
Platelets & factors get used up  bleeding
Microangiopathic hemolytic anemia (MAHA)

19
Q

What 4 causes of DIC must you for sure remember (MOST)?

A

Malignancy
OB complications
Sepsis
Trauma

20
Q

What lab tests would you see in DIC?

A

PTT, TT, INR prolonged
↑FDPs
↓Fibrinogen

21
Q

How do you Tx DIC?

A

Treat underlying disorder, support patient w/ blood

22
Q

What are the 4 things you must know about ITP?

A

Antiplatelet Ab’s
Acute vs Chronic
Dx of exclusion
Tx: Steroids or splenectomy

23
Q

What do the Ab’s in ITP bind?

A

GP IIb-IIIa or Ib –> spleen eating platelets

24
Q

What is a fear of chronic ITP?

A

Brain bleed (SA hemorrhage)

25
What labs would you see in ITP?
Normal PTT, INR, TT ↓Platelets ↑Megakaryocytes & big platelets (b/c they’re making more)
26
TTP and HUS are a subset of what?
Thrombotic Microangiopathies – thrombi, ↓platelets, and MAHA
27
What are the 4 things you must know about Thrombotic Thrombocytopenic Purpura (TTP)?
Pentad: Thrombi, ↓platelets, fever, neuro deficits, renal failure Deficiency of ADAMTS13 Big vWF multimers trap platelets Tx: Plasmapheresis or Plasma infusions
28
What is ADAMTS13?
Cleaves large vWF. | W/o ADAMTS13, big vWF aggregates plateltes --> thrombi
29
What are the 4 things you must know about Hemolytic Uremic Syndrome (HUS)?
MAHA and ↓platelets Epidemic (E.coli) vs non-epidemic Toxin (or ?) dmges endothelium Tx: support
30
Why don’t we give antibiotics to HUS patient?
B/c bug would burst open releasing it’s toxins
31
What are some other causes of acquired bleeding disorders?
Vitamin K deficiency - ↓coag factors | Liver disease - ↓factors and thrombopoietin, ↓VitK absorb, portal hypertension traps platelets