RBC II Putthoff, Bleeding Disorders

Question 1

excessive bleeding can result from what

Answer 1

increased fragility of vessels, platelet deficiency or dysfunction and derangement of coagulation

Question 2

PT time asses what

Answer 2

extrinsic and common coagulation pathways

Question 3

what can cause increased PT time

Answer 3

dysfunction factor V VII X prothrombine or fibrinogen

Question 4

What does PTT assess

Answer 4

intrinsic and common clotting pathways

Question 5

prolonged PTT indicates what

Answer 5

deficiency in : V VII IX X XI or XII, prothrombin or finbrinogen

Question 6

bleeding due to vessel wall abnormalities are characterized how clnicallys

Answer 6

small hemorrhages (petichiae and purpura)

Question 7

What are PT and PTT times in vessel wall abrnomality bleeding disorders

Answer 7

normal

Question 8

what infections can cause petechial and purpuric hemorrhages

Answer 8

meningococcemia, infective endocarditis and some of the rickettsial

Question 9

How do drugs induce cutaneous petechiae and purpura

Answer 9

vascular injury from drug induced IC in vessel walls which leads to HS vasculitis

Question 10

Scurvy and Ehlers-Danlos syndrome and Cushings are assoc with what type bleeding disorder

Answer 10

microvascular bleeding that results from collagen defects that weaken vessel walls

Question 11

What is Henoch Schonlein purpura

Answer 11

systemic immune disorder of unknown cause characterized by purpuric rash, colicky abdominal pain, polyarthralgia and acute glomerulonephritis

Question 12

what Ig is assoc with henoch schonlein

Answer 12

IgA

Question 13

What is weber-osler rendu syndrome

Answer 13

hereditary hemorrhagic telangiectasia

dilted tortuous blood vessels with thin walls that bleed readily

Question 14

what type of hereditary pattern is seen in hereditary hemorrhagic telangiectasia

Answer 14

autosomal domintant

mutations in TBF bet signaling

Question 15

where are the common sits of bleeding in hereditary hemorrhagic telangiectasia

Answer 15

nose, tongue, mouth, eyes and GI tract

Question 16

Perivascular amyloidosis weakening of blood vessels walls is a complication seen in what disorder

Answer 16

amyliod light chain amyloidosis

Question 17

platelet numbers are how low to be considered associated with nontraumatic bleeding

Answer 17

<20,000

Question 18

what will PT and PTT be in bleeding from thrombocytopenia

Answer 18

normal

Question 19

where are common hemorrhage sites for bleeding from thrombocytopenia

Answer 19

skin and mucous membranes of GI and gU tracts

Question 20

what is most feared thromcytopenic bleeding site

Answer 20

intracranial

Question 21

4 main causes of thromcoytopenia

Answer 21

decreased platelet production
decreased platelet survivial
sequestration
dilution

Question 22

decreased platelet productiosn generally occurs when

Answer 22

depressed arrow output like in anemias and leukemias
drugs or alcohol that can supress production
HIV
myelodysplastic syndromes

Question 23

What is immune thrombocytopenia

Answer 23

destruction caused by the deposition of Ab or IC on platelets

Question 24

What are Ab that recognized self Ag

Answer 24

autoAb

Question 25

what are Ab that recognize non-self Ag

Answer 25

alloAb

Question 26

When can alloAb arise

Answer 26

when platelets are transfused | cross placenta durign pregnancy

Question 27

Most important non immunoe causes of decreased platelet survival are what

Answer 27

DIC, thrombotic microangiopathies | mechanical injury

Question 28

how much of platelets can spleen sequester

Answer 28

80-90%

Question 29

what is dilutional thrombocytopenia

Answer 29

massive transfusions can produce a dilutional thrombocytopenia

Question 30

What causes chronic ITP

Answer 30

autoAb mediated destruction of platelets

Question 31

What conditions can lead to secondary chronic ITP

Answer 31

SLE, HIV, B cell neoplasms like CLL

Question 32

autoAb are usually against what in chronic ITP

Answer 32

membrane glycoproteins IIb-IIIa or Ib-IX | IgG

Question 33

What is a good Tx for chornic ITP

Answer 33

splenectomy because thats where the opsonized platelets from autoAb are remobed

Question 34

is there splenomegaly in chronic ITP?

Answer 34

no

Question 35

what does BM look like in chronic ITP

Answer 35

increased # megakaryocytes

Question 36

what will PS look like in chronic IT

Answer 36

abnormally large platelets | sign of thrombopoiesis

Question 37

population affected by chronic ITP

Answer 37

adult women younger than 40 y.o | 3:1 F:M

Question 38

clinical signs ITP

Answer 38

insidious onset, bleeding into the skin and mucosal surfaces pinpoint hemorrhages that can lead to ecchymoses Hx easy bruisning and nosebleeds melena, hematuria or excessive menstural dlow

Question 39

fatal complications of chronic ITP

Answer 39

subarachnoid hemorrhage and intracerebral hemorrhage

Question 40

PT and PTT in chronic ITP

Answer 40

normal

Question 41

What causes acute ITP

Answer 41

autoAb to platelets

Question 42

When does acute ITP occur

Answer 42

childhood disease 1- 2 weeks post viral illness

Question 43

Tx for acute ITP if severe

Answer 43

glucocorticoids

Question 44

Which cases of acute ITP are mroe severe

Answer 44

without viral prodrome the thrombocytopenia persists and usually progress to chronic ITP

Question 45

What drugs most commonly assoc with inducing thrombocytopenia

Answer 45

quinine, quinidine, vancomycin by binding platelet glycoptoeins and creating antigenic determinants

Question 46

how do platelet inhibitory drugs lead to thrombocytopenia

Answer 46

induce conformational changes in glycoprotein IIb IIIa and create immunogenic epitope

Question 47

What is type I Heparin induced thrombocytopenia

Answer 47

rapidly after onset of therapy, | from direct platelet aggregating effect of heparin

Question 48

What is type II HIT

Answer 48

5-14 days post Tx and usually leads to life threatening venous and arterial thrombosis

Question 49

What cuases HIT type II

Answer 49

Ab recognize heparin complexes and paltelet factor 4 so when they bind activates platelets and thrombosis

Question 50

How does HIV lead to thrombocytopenia

Answer 50

impaired platelet production and increased destruction | development of auto Ab in B cell hyperplasia

Question 51

Thrombotic microangiopathy includes what

Answer 51

many syndromes | TTP HUS

Question 52

what are thrombotic microangiopathies

Answer 52

insult that lead to activation of platelets that deposit as thrombi in small blood vessels

Question 53

What is the pentad of TTP

Answer 53

fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurologic deficits and renal failure.

Question 54

how is HUS distinguished from TTP

Answer 54

absence of nuerologic Sx and has acute renal failure | frequent in children

Question 55

Intravascular thrombi in TTP and HUS lead to what

Answer 55

microangiopathic hemolytic anemia with widespread organ dysgunction and thrombocytopenia

Question 56

why are TTP and HUS different than DIC

Answer 56

actication of coagulation cascade not primary importance | normal PT and PTT

Question 57

what mutation is assoc with TTP

Answer 57

deficiency of ADAMTS13

Question 58

what is Typical HUS

Answer 58

E coli strain O157:H7 infection

Question 59

what is atypical HUS

Answer 59

alternative C' pathway inhibitor deficiencies like factor H, membrane cofactor protein CD46 or facor I