Krafts- Bleeding Disorders Flashcards
What is the difference between platelet bleeding and factor bleeding?
Platelet–superficial, petechiae, spontaneous
Factor- deep (joints), big bleeds, trauma
What are the hereditary bleeding disorders?
vW Disease
Hemo A
Hemo B
Hereditary platelet disorders
What are the acquired bleeding disorders?
DIC
ITP
TTP/HUS
What is the most common BLEEDING DISORDER? How is it inherited?
vW disease
autosomal dominant
What happens in vW disease?
vW factor is decreased or abnoromal--> variable severity (can die from 1st menses)
What is vW factor?
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)
What are the sxs associated w/ vW disease?
mucosal bleeding (nose bleeds, heavy menses, bruising)
Deep joint bleeding in severe cases
What are the types of vWD?
1: 70% decreased vWF
2: 25% abnormal vWF
3: 5% no vWF
What tests are indicative of vWD disease?
Bleeding time: prolonged
PTT: prolonged (d/t increase in factor VIII)
vWF decreased
Platelet aggregation studies abnormal
INR= normal
How do you treat vW disease?
DDAVP (raises VII and vWF levels)–releases vWF froms tores
cyroprecipitate (contains vWF and VIII)
Facotr VIII
What is the most common factor deficiency?
How is it inherited?
Hemophilia A
X linked recessive (30% are random mutations)
What happens in hemo A?
Factor VIII levels are decreased>
variable amt of factor bleeding
What are the sxs of hemo A?
Depends on amt of VIII
Typical factor bleeding (usually after trauma)
Prolonged bleeding after dental work
rarely mucosal hemorrahge
What tests are normal in hemo A?
INR, TT, platelet count, bleeding time (normal b/c nothing wrong w/ platelets)
What test are abnormal in hemo A?
PTT: prolonged
Factor VIII assays
DNA studies
How do you treat hemo A?
DDAVP FActor VII (don't give often b/c pt will produce Ab against it)
What is the main difference between hemo A and B?
A- factor 8
B- factor 9
much less common than hemo A
How are hemo A and B similar?
Same inheritance pattern (x-linked recessive)
same clnical and lab findings
What are two other factor deficiencies that are very rare?
XI def: bleeding after trauma
XIII def: severe neonatal bleeding
What are the four hereditary platelet disorders?
Bournard soulier syndrome
Glanzmann thrmobasthenia
Gray platelet syndrome
gamma granule deficiency
What are the characteristics of bournard soulier syndrome?
Abnormal Gp Ib (need it to bind vWF and adhese platelets)
abnormal adhesion (can’t start platelet plug process)
big platelets
severe bleeding
What are the characteristics of glanzmann thrombasthenia?
no IIb-IIIa (bind fibrinogen that sticks platelets together)>
no aggregation>
severe bleeding
*doesn’t respond to anythign except ristocetin
What are the characteristics of gray platelet syndrome?
No alpha granules
big empty platelets
mild bleeding
What are the main characteristics of gamma granules deficiency?
No gamma granules
can be part of chediak higashi syndrome
What are the 5 acquired bleeding disorders?
DIC
ITP
TTP
Hemolytic uremia syndrome
What causes DIC?
Something triggers coagulation causing thrombosis>
platelets and factors get used up causing bleeding>
clotting and bleeding are BOTH problems
**many underlying disorders
What type of anemia is commonly seen w/ DIC?
Microangiopathic hemolytic anemia
What are “dumpers” that initiate coagulation?
obstetric complications
adenocarcinoma
acute promyelocytic leukemia
What are “rippers” (things that are damaging to endothelium) that cause DIC?
Bacterial sepsis
trauma
burns
vasculitits
What are the MOST common causes of DIC?
Malignancy
OB complications
Sepsis
Trauma
Somebody w/ DIC presents w/….
insidious or fulminant
multi-system disease
thrombosis/bleeding
What lab tests are prolonged in DIC?
INR, PTT, TT
prolonged because CFs are increased
What lab tests are decreased in DIC?
Fibrinogen—decreased b/c you’re using it up
WHat lab tests are increased in DIC?
FDPs- not a great test for ruling in DIC
How do you treat DIC?
underlying disorder and support w/ blood products
What is idiopathic thrombocytopenic purpura?
Don’t know the cause, low platelet count, bleeding into skin
caused by pt making antibodies to platelets
acute or chronic
diagnosis of exclusion
What is the pathogeneesis of ITP?
autoantibodies to GP IIb-IIIa or Ib>
bind to platelets>
splenic macrophages eat platelets
How do you test for ITP?
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
Chronic ITP is more commonly observed in what population?
Adult women
primary or secondary
insidious- nose bleeds, easy bruising
danger–bleeding into brain
Acute ITP is more commonly observed in what population?
Children
abrupt, follows viral illness
usually self-limiting
may become chronic
What is the treatment for ITP?
Steroids
splenectomy
IV Ig
What are similarities between TTP and HUS?
All have thrombi, thrombocytopenia, MAHA
something triggers platelet activation—different from DIC where something is wrong w/ coag problem
What is the pentad for TTP?
MAHA thrombocytopenia fever neurologic defects renal failure
What causes TTP?
Def in ADAMTS13>
BIG vwf multimers that trap platelets
How do you treat TTP
Plasmapheresis (remove ab) or plasma infusiosn (replace enz def)
What is the pathogenesis of ITP?
Just released vWF is UL>
ULvWF causes platelet aggregation>
ADAMTS13 cleaves ULvWF into less active bit
heamaturia, jaundice bleeding, bruising fever bizarre behavior decreased urine output
are characteristic of what disease?
TTP
What are the 2 primary characteristics and two types of HUS?
MAHA and thrombocytopenia
Epidemic vs. non-epidemic
*toxin damages endotehilum
What is the pathogenesis of epidemic HUS?
E. coli (raw hamburger)>
nasty toxin>
injures endothelial cells>
kidneys often involved
What is the pathogenesis of non-epidemic HUS?
Defect in complement factor H
Inherited or acquired
Epidemic HUS usually effects…
children and elderly
bloody diarrhea and renal failure
fatal in 5%
Non-epidemic HUS causes….
renal failure
releapsing/remitting course
fatal in 50%
How do you treat HUS?
supportive care
dialysis
NO antibiotics (can increase toxin release and hurt pt more)
