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hemostasis > section 4 - platelet and vascular disorders > Flashcards

section 4 - platelet and vascular disorders Flashcards

1
Q

describe hereditary hemorrhagic telangiestasia

A
  • hereditary vascular disorder
  • vessel walls = single layer of EC -> fragile vessels
  • telangiectasia - dilated superficial vessels BLANCHE UNDER PRESSURE
  • increased bleeding
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2
Q

describe ehlers-danlos syndrome

A
  • hereditary vascular disorder with hypermobile joints
  • collagen disorders impacting platelet adhesion
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3
Q

what is the mechanism of action for allergic and drug-induced purpuras

A
  • acquired vascular disorder
  • antibody to vessel walls deposits and damages
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4
Q

describe Henoch-Schonlein Purpura

A
  • usually child after upp resp. infection
  • IgA deposits in vessels
  • IgA deposits in kidney and damages nephron
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5
Q

Scurvy!

A
  • vitamin C deficiency
  • decrease synthesis of collagen = weak capillary walls
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6
Q

senile purpura!

A
  • loss of collagen and subcut fat = loss of stability for veins
  • vein rupture
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7
Q

describe the defect in Bernard-Soulier syndrome and its signature appearance

A
  • lack of Gp1b
  • adhesion disorder
  • large platelets
  • all normal aggregation except with ristocetin
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8
Q

describe the defect in type 1 vWF disease

A
  • most common
  • decreased amount of all multimers
  • since low vWF = low F8 = impacting coag cascade
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9
Q

describe type 2 vWF disease

A
  • decrease in high MW multimers
  • possible inability to stabilize large multimers
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10
Q

describe type 3 vWF disease

A

all multimers of vWF absent
- either reduced synthesis or rapid breakdown

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11
Q

how are different types of vWF diseases differentiated

A
  • vWF:Ag determining vWF presence in plasma
  • RIPA determining platelet function based on platelet agglutination pt PRP and PLT
  • vWF:Rco determining platelet function based on platelet agglutination pt plasma and donor PLT
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12
Q

list other platelet adhesion problems not described

A
  • autoimmune disorders
  • myeloproliferative disorders
  • multiple myeloma/waldenstroms
  • chronic liver disease
  • drugs
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13
Q

describe Glanzmann’s Thrombasthenia

A
  • disorder of aggregation
  • platelets lack Gp2b3a
  • PT/APTT normal since a platelet issue, not coag
  • platelet aggregation only normal with Ristocetin
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14
Q

describe uremia

A
  • toxins interfering ith platelet funciton
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15
Q

describe aspirin resistance

A
  • 22% of patients become resistant to anti-platelet effect
  • associated with thrombosis
  • detected with platelet aggregation studies -> arachidonic acid curve
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16
Q

describe storage pool diseases generally

A

lack of platelet dense or alpha granules -> defect in release reaction
- abnormal aggregation

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17
Q

delta DPD

A

lack of dense granules

18
Q

describe Hermansky-Pudlak syndrome

A
  • channel system dilated
  • dense granule deficiency
  • swiss cheese platelets
  • deficient release rxn
19
Q

describe gray platelet syndrome

A
  • marked decrease in alpha granules
  • hypo or granular platelets
  • effects platelet aggregation
20
Q

describe Wiskott Aldrich Syndrom

A
  • micro platelets
  • decrease alpha and dense granules
  • plt sequestration and decreased platelet count
  • immunodeficiency disorder
  • congenital hypoplasia
21
Q

describe Chediak-Higashi platelet syndrome

A
  • platelets lack normal dense granules
22
Q

describe reactive thrombocytosis

A

normal body response to blood loss

23
Q

how does myeloproliferative disorders impact platelets

A

thrombocytosis and high platelets

24
Q

describe essential thrombocythemia

A
  • bizarre and giant platelets
25
Q

describe how may hegglin impacts platelets

A

ineffective thrombopoiesis w/ large bizarre platelets

26
Q

describe the differences between chronic and acute ITP

A
  • chronic: may see early manifestation of AIDS w/ fluctuating course
  • acute: mostly kids with history of infection
27
Q

list and describe the three mechanisms of drug induced immune effects

A
  • true auto Ab development - not dependent on presence of the drug
  • hapten - linkage of drug to platelet then Ab forms
  • drug complex - drug and Ab bind then attach to platelets
28
Q

describe HAT (heparin-associated thrombocytopenia)

A

direct non immune platelet activation

29
Q

describe heparin induced thrombocytopenia (HIT)

A

patient with thrombosis put on heparin and develop antibody against platelet factor 4 - heparin complex

30
Q

what is the difference between HIT and HITTS

A

HIT plt count doesn’t fall below 100
HITTS plt count falls below 20 w/ thrombosis

31
Q

describe HITTS

A
  • HIT Ab releases micro particles from platelets and initiates cascade
  • low platelets and thrombosis
  • Ab is against Pf4-heparin complex
  • HIT Ab binds to heparan on the endothelial cell surface
    and stimulates expression of tissue factor
32
Q

true or false
in HIT and HITTS the Ab is only active in the presence of heparin

A

true
- pt taken off heparin to measure

33
Q

which tests are used for HIT

A
  • SRA (reference)
  • PLT aggregation (HIPA): common
  • immunoassay (ELISA): most common
34
Q

describe HIPA test method for HIT

A

test for presenf of heparin-induced Ab in pt plasma (off heparin for 8 hours)
- normal donor PLTs + PPP + dilutions of heparin

35
Q

describe neonatal alloimmune thrombocytopenia

A

mother is HPA-1a neg, baby is positive, mother develops Ab
- HPA-1a version of HDN

36
Q

describe post transfusion purpura

A
  • antibodies form to antigen recipiant is neg for (HPA-1a)
37
Q

describe HELLP acronyms

A
  • hemolysis
  • elevated liver enzymes
  • low platelet count
    -> variant of preeclampsia
38
Q

list etiology of HELLP

A
  • intravascular platelet activation
  • microvascular endothelial damage
  • thromboxane A2 release
  • vasospasm
  • vascular lesions in multiple organs
39
Q

describe thrombotic thrombocytopenic purpura

A
  • endothelial cell injury and platelet thrombus forms
  • either autoantibody to ADAMTD13 or inherited variant of ADAMTS13 deficiency
40
Q

describe the clinical and lab findings of TTP

A
  • hemolytic anemia
  • thrombocytopenia
  • fluctuating neurological dysfunction
  • progressive renal disease
41
Q

describe HUS findings and distinguishing characteristics

A
  • resembles TTP but differentiated based on severity of renal failure and absence of neural symptoms
  • typically occurs after acute infection
  • findings: MAHA, thrombocytopenia, acute renal failure

hemostasis (10 decks)

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