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IHO Week 5 > Krafts- Bleeding Disorders > Flashcards

Krafts- Bleeding Disorders Flashcards

1
Q

What is the difference between platelet bleeding and factor bleeding?

A

Platelet–superficial, petechiae, spontaneous

Factor- deep (joints), big bleeds, trauma

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2
Q

What are the hereditary bleeding disorders?

A

vW Disease
Hemo A
Hemo B
Hereditary platelet disorders

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3
Q

What are the acquired bleeding disorders?

A

DIC
ITP
TTP/HUS

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4
Q

What is the most common BLEEDING DISORDER? How is it inherited?

A

vW disease

autosomal dominant

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5
Q

What happens in vW disease?

A 
vW factor is decreased or abnoromal--> 
variable severity (can die from 1st menses)
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6
Q

What is vW factor?

A

Multimeric protein that is decreased or abnormal in vW disease
made by megs or endothelial cells
glues platelets to endothelium
carries factor VIII (if not carried VIII degrades)

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7
Q

What are the sxs associated w/ vW disease?

A

mucosal bleeding (nose bleeds, heavy menses, bruising)

Deep joint bleeding in severe cases

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8
Q

What are the types of vWD?

A

1: 70% decreased vWF
2: 25% abnormal vWF
3: 5% no vWF

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9
Q

What tests are indicative of vWD disease?

A

Bleeding time: prolonged
PTT: prolonged (d/t increase in factor VIII)
vWF decreased
Platelet aggregation studies abnormal

INR= normal

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10
Q

How do you treat vW disease?

A

DDAVP (raises VII and vWF levels)–releases vWF froms tores
cyroprecipitate (contains vWF and VIII)
Facotr VIII

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11
Q

What is the most common factor deficiency?

How is it inherited?

A

Hemophilia A

X linked recessive (30% are random mutations)

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12
Q

What happens in hemo A?

A

Factor VIII levels are decreased>

variable amt of factor bleeding

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13
Q

What are the sxs of hemo A?

A

Depends on amt of VIII
Typical factor bleeding (usually after trauma)
Prolonged bleeding after dental work

rarely mucosal hemorrahge

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14
Q

What tests are normal in hemo A?

A

INR, TT, platelet count, bleeding time (normal b/c nothing wrong w/ platelets)

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15
Q

What test are abnormal in hemo A?

A

PTT: prolonged
Factor VIII assays
DNA studies

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16
Q

How do you treat hemo A?

A 
DDAVP
FActor VII (don't give often b/c pt will produce Ab against it)
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17
Q

What is the main difference between hemo A and B?

A

A- factor 8

B- factor 9
much less common than hemo A

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18
Q

How are hemo A and B similar?

A

Same inheritance pattern (x-linked recessive)

same clnical and lab findings

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19
Q

What are two other factor deficiencies that are very rare?

A

XI def: bleeding after trauma

XIII def: severe neonatal bleeding

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20
Q

What are the four hereditary platelet disorders?

A

Bournard soulier syndrome
Glanzmann thrmobasthenia
Gray platelet syndrome
gamma granule deficiency

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21
Q

What are the characteristics of bournard soulier syndrome?

A

Abnormal Gp Ib (need it to bind vWF and adhese platelets)
abnormal adhesion (can’t start platelet plug process)
big platelets
severe bleeding

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22
Q

What are the characteristics of glanzmann thrombasthenia?

A

no IIb-IIIa (bind fibrinogen that sticks platelets together)>
no aggregation>
severe bleeding

*doesn’t respond to anythign except ristocetin

23
Q

What are the characteristics of gray platelet syndrome?

A

No alpha granules
big empty platelets
mild bleeding

24
Q

What are the main characteristics of gamma granules deficiency?

A

No gamma granules

can be part of chediak higashi syndrome

25
Q

What are the 5 acquired bleeding disorders?

A

DIC
ITP
TTP
Hemolytic uremia syndrome

26
Q

What causes DIC?

A

Something triggers coagulation causing thrombosis>
platelets and factors get used up causing bleeding>
clotting and bleeding are BOTH problems

**many underlying disorders

27
Q

What type of anemia is commonly seen w/ DIC?

A

Microangiopathic hemolytic anemia

28
Q

What are “dumpers” that initiate coagulation?

A

obstetric complications
adenocarcinoma
acute promyelocytic leukemia

29
Q

What are “rippers” (things that are damaging to endothelium) that cause DIC?

A

Bacterial sepsis
trauma
burns
vasculitits

30
Q

What are the MOST common causes of DIC?

A

Malignancy
OB complications
Sepsis
Trauma

31
Q

Somebody w/ DIC presents w/….

A

insidious or fulminant
multi-system disease
thrombosis/bleeding

32
Q

What lab tests are prolonged in DIC?

A

INR, PTT, TT

prolonged because CFs are increased

33
Q

What lab tests are decreased in DIC?

A

Fibrinogen—decreased b/c you’re using it up

34
Q

WHat lab tests are increased in DIC?

A

FDPs- not a great test for ruling in DIC

35
Q

How do you treat DIC?

A

underlying disorder and support w/ blood products

36
Q

What is idiopathic thrombocytopenic purpura?

A

Don’t know the cause, low platelet count, bleeding into skin

caused by pt making antibodies to platelets

acute or chronic
diagnosis of exclusion

37
Q

What is the pathogeneesis of ITP?

A

autoantibodies to GP IIb-IIIa or Ib>
bind to platelets>
splenic macrophages eat platelets

38
Q

How do you test for ITP?

A

Look for signs of platelet destruction

  • thrombocytopenia
  • normal/increased megakaryocytes (platelets are taken out of circulation so have to make more)
  • big platelets

INR/PTT normal
no specific diagnostic test

39
Q

Chronic ITP is more commonly observed in what population?

A

Adult women
primary or secondary
insidious- nose bleeds, easy bruising

danger–bleeding into brain

40
Q

Acute ITP is more commonly observed in what population?

A

Children
abrupt, follows viral illness
usually self-limiting
may become chronic

41
Q

What is the treatment for ITP?

A

Steroids
splenectomy
IV Ig

42
Q

What are similarities between TTP and HUS?

A

All have thrombi, thrombocytopenia, MAHA

something triggers platelet activation—different from DIC where something is wrong w/ coag problem

43
Q

What is the pentad for TTP?

A 
MAHA
thrombocytopenia
fever
neurologic defects
renal failure
44
Q

What causes TTP?

A

Def in ADAMTS13>

BIG vwf multimers that trap platelets

45
Q

How do you treat TTP

A

Plasmapheresis (remove ab) or plasma infusiosn (replace enz def)

46
Q

What is the pathogenesis of ITP?

A

Just released vWF is UL>
ULvWF causes platelet aggregation>
ADAMTS13 cleaves ULvWF into less active bit

47
Q 
heamaturia, jaundice
bleeding, bruising
fever
bizarre behavior
decreased urine output

are characteristic of what disease?

A

TTP

48
Q

What are the 2 primary characteristics and two types of HUS?

A

MAHA and thrombocytopenia

Epidemic vs. non-epidemic

*toxin damages endotehilum

49
Q

What is the pathogenesis of epidemic HUS?

A

E. coli (raw hamburger)>
nasty toxin>
injures endothelial cells>
kidneys often involved

50
Q

What is the pathogenesis of non-epidemic HUS?

A

Defect in complement factor H

Inherited or acquired

51
Q

Epidemic HUS usually effects…

A

children and elderly
bloody diarrhea and renal failure
fatal in 5%

52
Q

Non-epidemic HUS causes….

A

renal failure
releapsing/remitting course
fatal in 50%

53
Q

How do you treat HUS?

A

supportive care
dialysis
NO antibiotics (can increase toxin release and hurt pt more)

IHO Week 5 (9 decks)

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