Krafts- Bleeding Disorders

Question 1

What is the difference between platelet bleeding and factor bleeding?

Answer 1

Platelet–superficial, petechiae, spontaneous

Factor- deep (joints), big bleeds, trauma

Question 2

What are the hereditary bleeding disorders?

Answer 2

vW Disease
Hemo A
Hemo B
Hereditary platelet disorders

Question 3

What are the acquired bleeding disorders?

Answer 3

DIC
ITP
TTP/HUS

Question 4

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

Answer 4

vW disease

autosomal dominant

Question 5

What happens in vW disease?

Answer 5

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

Question 6

What is vW factor?

Answer 6

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)

Question 7

What are the sxs associated w/ vW disease?

Answer 7

mucosal bleeding (nose bleeds, heavy menses, bruising)

Deep joint bleeding in severe cases

Question 8

What are the types of vWD?

Answer 8

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

Question 9

What tests are indicative of vWD disease?

Answer 9

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

INR= normal

Question 10

How do you treat vW disease?

Answer 10

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

Question 11

What is the most common factor deficiency?

How is it inherited?

Answer 11

Hemophilia A

X linked recessive (30% are random mutations)

Question 12

What happens in hemo A?

Answer 12

Factor VIII levels are decreased>

variable amt of factor bleeding

Question 13

What are the sxs of hemo A?

Answer 13

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

rarely mucosal hemorrahge

Question 14

What tests are normal in hemo A?

Answer 14

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

Question 15

What test are abnormal in hemo A?

Answer 15

PTT: prolonged
Factor VIII assays
DNA studies

Question 16

How do you treat hemo A?

Answer 16

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

Question 17

What is the main difference between hemo A and B?

Answer 17

A- factor 8

B- factor 9
much less common than hemo A

Question 18

How are hemo A and B similar?

Answer 18

Same inheritance pattern (x-linked recessive)

same clnical and lab findings

Question 19

What are two other factor deficiencies that are very rare?

Answer 19

XI def: bleeding after trauma

XIII def: severe neonatal bleeding

Question 20

What are the four hereditary platelet disorders?

Answer 20

Bournard soulier syndrome
Glanzmann thrmobasthenia
Gray platelet syndrome
gamma granule deficiency

Question 21

What are the characteristics of bournard soulier syndrome?

Answer 21

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

Question 22

What are the characteristics of glanzmann thrombasthenia?

Answer 22

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

*doesn’t respond to anythign except ristocetin

Question 23

What are the characteristics of gray platelet syndrome?

Answer 23

No alpha granules
big empty platelets
mild bleeding

Question 24

What are the main characteristics of gamma granules deficiency?

Answer 24

No gamma granules

can be part of chediak higashi syndrome

Question 25

What are the 5 acquired bleeding disorders?

Answer 25

DIC ITP TTP Hemolytic uremia syndrome

Question 26

What causes DIC?

Answer 26

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

Question 27

What type of anemia is commonly seen w/ DIC?

Answer 27

Microangiopathic hemolytic anemia

Question 28

What are "dumpers" that initiate coagulation?

Answer 28

obstetric complications adenocarcinoma acute promyelocytic leukemia

Question 29

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

Answer 29

Bacterial sepsis trauma burns vasculitits

Question 30

What are the MOST common causes of DIC?

Answer 30

Malignancy OB complications Sepsis Trauma

Question 31

Somebody w/ DIC presents w/....

Answer 31

insidious or fulminant multi-system disease thrombosis/bleeding

Question 32

What lab tests are prolonged in DIC?

Answer 32

INR, PTT, TT prolonged because CFs are increased

Question 33

What lab tests are decreased in DIC?

Answer 33

Fibrinogen---decreased b/c you're using it up

Question 34

WHat lab tests are increased in DIC?

Answer 34

FDPs- not a great test for ruling in DIC

Question 35

How do you treat DIC?

Answer 35

underlying disorder and support w/ blood products

Question 36

What is idiopathic thrombocytopenic purpura?

Answer 36

Don't know the cause, low platelet count, bleeding into skin caused by pt making antibodies to platelets acute or chronic diagnosis of exclusion

Question 37

What is the pathogeneesis of ITP?

Answer 37

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

Question 38

How do you test for ITP?

Answer 38

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

Question 39

Chronic ITP is more commonly observed in what population?

Answer 39

Adult women primary or secondary insidious- nose bleeds, easy bruising danger--bleeding into brain

Question 40

Acute ITP is more commonly observed in what population?

Answer 40

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

Question 41

What is the treatment for ITP?

Answer 41

Steroids splenectomy IV Ig

Question 42

What are similarities between TTP and HUS?

Answer 42

All have thrombi, thrombocytopenia, MAHA something triggers platelet activation---different from DIC where something is wrong w/ coag problem

Question 43

What is the pentad for TTP?

Answer 43

``` MAHA thrombocytopenia fever neurologic defects renal failure ```

Question 44

What causes TTP?

Answer 44

Def in ADAMTS13> | BIG vwf multimers that trap platelets

Question 45

How do you treat TTP

Answer 45

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

Question 46

What is the pathogenesis of ITP?

Answer 46

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

Question 47

``` heamaturia, jaundice bleeding, bruising fever bizarre behavior decreased urine output ``` are characteristic of what disease?

Answer 47

TTP

Question 48

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

Answer 48

MAHA and thrombocytopenia Epidemic vs. non-epidemic *toxin damages endotehilum

Question 49

What is the pathogenesis of epidemic HUS?

Answer 49

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

Question 50

What is the pathogenesis of non-epidemic HUS?

Answer 50

Defect in complement factor H | Inherited or acquired

Question 51

Epidemic HUS usually effects...

Answer 51

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

Question 52

Non-epidemic HUS causes....

Answer 52

renal failure releapsing/remitting course fatal in 50%

Question 53

How do you treat HUS?

Answer 53

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