Bleeding disorders Flashcards

1
Q

Bleeding that is:

  • superficial
  • petechiae
  • spontaneous
A

Platelet bleeding

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2
Q

Bleeding that is:

  • deep joints
  • Big bleeds
  • Trauma
A

Factor bleeding

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3
Q

Most common hereditary bleeding disorder

Autosomal dominant

Variable severity

A

Von Willebrand Disease

**vW factor decreased or abnormal

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4
Q

vW factor made where?

A

megakaryocytes and subendothelial cells

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5
Q

vWF carries what factor?

A

VIII

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6
Q

Three types of vW disease:

A

Type 1 (70%) : decreased vWF

Type 2 (25%) : abnormal vWF

Type 3 (5%) : no vWF

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7
Q

Sx of vW disease?

A

mucosal bleeding

deep joint bleeding (severe cases)

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8
Q

What test will be NORMAL in a pt with vW disease?

A

INR

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9
Q

Platelet membrane protein that binds vWF?

A

GP Ib

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10
Q

Tx fro vW disease?

A

DDAVP (for type I)

Cryoprecipitate (contains vWF and VIII)

Factor VIII

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11
Q

X-linked recessive bleeding disorders:

A

hemophilia A and B

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12
Q

most common FACTOR deficiency

factor VIII decreased

A

hemophilia A

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13
Q

common sx of hemophilia A:

A

deep joint bleeding

prolonged bleeding after dental work

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14
Q

NORMAL lab results in hemophilia A and B?

A

INR

TT

Platelet count

bleeding time

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15
Q

What does PTT look like in hemophilia?

A

prolonged (low factor VIII or IX)

**corrects with mixing study

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16
Q

Tx of hemophilia A:

A

DDAVP

Factor VIII

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17
Q

What is the difference between hemophilia A and B

A

A = VIII problem

B = IX problem, MUCH LESS COMMON

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18
Q

Rare; bleeding only after trauma:

A

XI deficiency

19
Q

severe neonatal bleeding

A

XIII

20
Q

Crosslinks fibrin –> clot

A

XIIIa

21
Q

Abnormal GP Ib

Big platelets

Severe Bleeding

A

Bernard-Soulier Syndrome

22
Q

No IIb-IIIa

No aggregation

Severe bleeding

A

Glanzmann Thrombasthenia

23
Q

No alpha granules in platelets

Big, empty platelets

Mild bleeding

A

Gray Platelet Syndrome

24
Q

Platelet prob related to Chediak-Higashi?

A

delta Granule deficiency

25
Q

Lots of causes

coag triggered causing thrombosis

platelets and factors get used up, causing bleeding

MAHA

A

DIC

**Disseminated Intravascular Coagulation

26
Q

DIC triggers that “dump” coag factors causing DIC:

A

OB complications

Adenocarcinoma

AML (promyelocytic)

27
Q

DIC triggers that “rip” endothelial cells apart causing DIC:

A

Bacterial sepsis

Trauma

Burns

Vasculitis

28
Q

Top 4 causes of DIC*****:

A

Malignancy

OB complications

Sepsis

Trauma

29
Q

INR, PTT, TT in DIC?

A

prolonged

30
Q

FDP’s in DIC?

A

increased

31
Q

Fibrinogen in DIC?

A

decreased

32
Q

Antiplatelet antibodies

Acute vs Chronic

Dx of exclusion

Tx steroids or splenectomy

A

Ideopathic Thrombocytopenic Purpura (ITP)

33
Q

What do the Igs bind to in ITP?

A

GP IIb-IIIa or Ib

***get eaten by splenic mac’s

34
Q

Demographic for chronic ITP?

A

adult women

35
Q

Demographic for acute ITP?

A

kids

36
Q

ADAMTS13 deficiency

A

Thrombotic Thrombocytopenic Purpura

37
Q

MAHA, ____________, fever, neurologic defects, renal failure

Big vWF multimers trap platelets

A

TTP

Thrombotic Thrombocytpenic Purpura

38
Q

Multimers of Unusual Size

A

TTP

39
Q

Clinical findings of:

Hematuria, jaundice (MAHA)

bleeding, bruising

Fever

Bizarre behavior (NEUROLOGICAL DEFICITS)

Decreased urine output (RENAL FAILURE)

A

TTP

40
Q

MAHA, Thrombocytopenia

E. coli***

Toxin or damage to endothelium

A

Hemolytic Uremic Syndrome

41
Q

Defect in complement factor H

A

Non-epidemic (E. coli related) Hemolytic Uremic Syndrome

42
Q

Brain involvement think?

A

TTP

43
Q

Kidney involvement think?

A

HUS

44
Q

contraindicated tx in HUS?

A

Abx!!!!

may increase toxin release