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Year 2 Heme lymph > RBC II Putthoff, Bleeding Disorders > Flashcards

RBC II Putthoff, Bleeding Disorders Flashcards

1
Q

excessive bleeding can result from what

A

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

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2
Q

PT time asses what

A

extrinsic and common coagulation pathways

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3
Q

what can cause increased PT time

A

dysfunction factor V VII X prothrombine or fibrinogen

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4
Q

What does PTT assess

A

intrinsic and common clotting pathways

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5
Q

prolonged PTT indicates what

A

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

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6
Q

bleeding due to vessel wall abnormalities are characterized how clnicallys

A

small hemorrhages (petichiae and purpura)

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7
Q

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

A

normal

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8
Q

what infections can cause petechial and purpuric hemorrhages

A

meningococcemia, infective endocarditis and some of the rickettsial

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9
Q

How do drugs induce cutaneous petechiae and purpura

A

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

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10
Q

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

A

microvascular bleeding that results from collagen defects that weaken vessel walls

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11
Q

What is Henoch Schonlein purpura

A

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

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12
Q

what Ig is assoc with henoch schonlein

A

IgA

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13
Q

What is weber-osler rendu syndrome

A

hereditary hemorrhagic telangiectasia

dilted tortuous blood vessels with thin walls that bleed readily

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14
Q

what type of hereditary pattern is seen in hereditary hemorrhagic telangiectasia

A

autosomal domintant

mutations in TBF bet signaling

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15
Q

where are the common sits of bleeding in hereditary hemorrhagic telangiectasia

A

nose, tongue, mouth, eyes and GI tract

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16
Q

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

A

amyliod light chain amyloidosis

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17
Q

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

A

<20,000

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18
Q

what will PT and PTT be in bleeding from thrombocytopenia

A

normal

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19
Q

where are common hemorrhage sites for bleeding from thrombocytopenia

A

skin and mucous membranes of GI and gU tracts

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20
Q

what is most feared thromcytopenic bleeding site

A

intracranial

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21
Q

4 main causes of thromcoytopenia

A

decreased platelet production
decreased platelet survivial
sequestration
dilution

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22
Q

decreased platelet productiosn generally occurs when

A

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

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23
Q

What is immune thrombocytopenia

A

destruction caused by the deposition of Ab or IC on platelets

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24
Q

What are Ab that recognized self Ag

A

autoAb

25
Q

what are Ab that recognize non-self Ag

A

alloAb

26
Q

When can alloAb arise

A

when platelets are transfused

cross placenta durign pregnancy

27
Q

Most important non immunoe causes of decreased platelet survival are what

A

DIC, thrombotic microangiopathies

mechanical injury

28
Q

how much of platelets can spleen sequester

A

80-90%

29
Q

what is dilutional thrombocytopenia

A

massive transfusions can produce a dilutional thrombocytopenia

30
Q

What causes chronic ITP

A

autoAb mediated destruction of platelets

31
Q

What conditions can lead to secondary chronic ITP

A

SLE, HIV, B cell neoplasms like CLL

32
Q

autoAb are usually against what in chronic ITP

A

membrane glycoproteins IIb-IIIa or Ib-IX

IgG

33
Q

What is a good Tx for chornic ITP

A

splenectomy because thats where the opsonized platelets from autoAb are remobed

34
Q

is there splenomegaly in chronic ITP?

A

no

35
Q

what does BM look like in chronic ITP

A

increased # megakaryocytes

36
Q

what will PS look like in chronic IT

A

abnormally large platelets

sign of thrombopoiesis

37
Q

population affected by chronic ITP

A

adult women younger than 40 y.o

3:1 F:M

38
Q

clinical signs ITP

A

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

39
Q

fatal complications of chronic ITP

A

subarachnoid hemorrhage and intracerebral hemorrhage

40
Q

PT and PTT in chronic ITP

A

normal

41
Q

What causes acute ITP

A

autoAb to platelets

42
Q

When does acute ITP occur

A

childhood disease 1- 2 weeks post viral illness

43
Q

Tx for acute ITP if severe

A

glucocorticoids

44
Q

Which cases of acute ITP are mroe severe

A

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

45
Q

What drugs most commonly assoc with inducing thrombocytopenia

A

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

46
Q

how do platelet inhibitory drugs lead to thrombocytopenia

A

induce conformational changes in glycoprotein IIb IIIa and create immunogenic epitope

47
Q

What is type I Heparin induced thrombocytopenia

A

rapidly after onset of therapy,

from direct platelet aggregating effect of heparin

48
Q

What is type II HIT

A

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

49
Q

What cuases HIT type II

A

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

50
Q

How does HIV lead to thrombocytopenia

A

impaired platelet production and increased destruction

development of auto Ab in B cell hyperplasia

51
Q

Thrombotic microangiopathy includes what

A

many syndromes

TTP HUS

52
Q

what are thrombotic microangiopathies

A

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

53
Q

What is the pentad of TTP

A

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

54
Q

how is HUS distinguished from TTP

A

absence of nuerologic Sx and has acute renal failure

frequent in children

55
Q

Intravascular thrombi in TTP and HUS lead to what

A

microangiopathic hemolytic anemia with widespread organ dysgunction and thrombocytopenia

56
Q

why are TTP and HUS different than DIC

A

actication of coagulation cascade not primary importance

normal PT and PTT

57
Q

what mutation is assoc with TTP

A

deficiency of ADAMTS13

58
Q

what is Typical HUS

A

E coli strain O157:H7 infection

59
Q

what is atypical HUS

A

alternative Cā€™ pathway inhibitor deficiencies like factor H, membrane cofactor protein CD46 or facor I

Year 2 Heme lymph (28 decks)

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