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02 Heme Lymph > 15 Clotting and Coagulopathies > Flashcards

15 Clotting and Coagulopathies Flashcards

0
Q

GPIb alpha

A

Binds vWFand functions in initial adhesion of platelet to endothelium
Platelet activation

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1
Q

GPIIb/IIIa

A

Platelette receptor for Fibrinogen, that functions in aggregation

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2
Q

Dense platelette granules

A

ADP, ATP, Serotonin, CA

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3
Q

Alpha granules

A

Factor-5, fibrinogen, vWF
GF- TGF-B, PDGF
Platelet factor 4

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4
Q

Signaling in platelts

A

ADP, 5HT, TxA2, thrombin receptors all Gq - PLCcreates IP3 and DAG

Causes Integrin activation, granule secretion and cytoskeletal rearrangement

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5
Q

Tissue Factor

A

Sub endothelial derived cofactor to VIIa, which activates 9 and 10

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6
Q

VITAMIN K DEPENDANT COAGULATION FACTORS

A

2, 7, 9, 10 (allows factors to localize to membranes)

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7
Q

VITAMIN K DEPENDENT ANTICOAGULANTS

A

Protein C ProteinS

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8
Q

Factor 13

A

Transpeptidase connecting D termini of fibrin fibers

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9
Q

tPA

A

Activators that bind and activate plasmin- which help it chew up clots.

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10
Q

Thrombomodulin

A

Binds thrombin and modulates it so that it activates proteins C

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11
Q

Endothelial anticoagulation

A

Release NO, and PGI2 - vaso-relaxants and inhibit platelet agregation
Heparin like molecules on surface binds antithrombin

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12
Q

Antithrombin III

A

When bound to heparin like molecules inactivates Thrombin, 10a and 9a

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13
Q

Mucosal bleeding

A

Suggesstive of vWF or Platelette problem

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14
Q

Deep bleeding

A

Usually a factor deficiency

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15
Q

Acute ITP

A

Common in children
Abrupt severe thrombocytopenia caused by autoantibodies to surface glycoproteins following an illness

Blood smear: few giant platelets, Normal reds and whites
BM: Normal to incr. megakaryocytes

Spleen important site of AB production and IgG platelette destruction.
Most children will recover spontaneously- 50% w/i 6 weeks, 90 w/i 6 months

16
Q

Chronic ITP

A

Common in adults
Insidious onset on thrombocytopenia with accumulation of bruising/ bleeding stiggmata- decreased platelet survival and production: RBC and WBC usualy normal

*Splenectomy normalizes platelets and induces remission in 2/3 of patients

17
Q

HIT

A

Heparn induced thrombocytopenia

Heparin and Platelet factor 4 complex autoantibody: IgG mediated
Get platelet activation and destruction
THROBOSIS - Arterial and Venous

Test with Elisa or serotonin release assay

Tx: Stop heparn, Treat with direct thrombin inhibitor: Argatroban Bivalirudan NEVER GIVE PLATELETS

18
Q

Thrombotic microangiopathy

A 
many platelett rich thrombi in microciculation
TTP
HUS
DIC
SEPSIS

Thrombocytopenia and microangiopathich hemolytic anemia
PTT and PT usually normal

19
Q

TTP pentad

A
  • Microangiopathic hemolyticc anemia*
  • Thrombocytopenia**

renal failure
AMS
fever

20
Q

TTP

A

ADAMST13 deficiency, usually autoimmune- vW multimers

Schistocytosis- Treat with Plasma exchange NEVER PLATELETS
Normal coags, negative coombs

21
Q

vWF tests

A

immunoassay

Ristoccetin activation assay

22
Q

Hemolytic uremic syndrome

A

Hemolytic anemia, AKI, Thrombocytopenia

  • usually kids with ecoli infection - SHIGA-likeTOXIN damages endothelials and creates platelet aggregation
  • also mutation or autoantibody deficient in anticomplement protiens factor H,I, CD46

Bloody diarrhea, Renal dailure
no neurologic symptoms

TX: plasma exchange

23
Q

vW Dz

A

autosomal dominant vWF deficiency
Low vW- defect in primary coagulation with mucosal bleeding and extensive wound bleeding

Treat with Desmopressin- vWF releasing drug

24
Q

Platelete disfunction Dz

A

Bernard Soulier- GP1b deficiency

Glanzman’s thromboblasthemia- GPIIb/GPIIIa

25
Q

Hemophilia A

A

Deficient in factor 8 (X-linked recessive) 30% spontaneous
Can have varying activity, <1% activity is severe

Massive hemorrhage after procedures or trauma
Hemarthrosis

Tx: Recombinante factor 8

Prolonged PTT, corrects with 1:1 mix

26
Q

Hemophilia B

A

Factor 9 deficiency x linked recessive disorder
Less common than A

Massive bleeding after procedures, spontaneous hemarthrosis
Long PTT- normalizes with 1:1 mix

Tx: Factor 9 infusion

27
Q

Vitamin K deficiency

A

Problems with Factors 2, 7, 10, 9

Cause: Warfarin, Antibiotics killing vitamin k bacteria, malabsorption/decreased intake, liver DZ, newborns

TX: Plasma or cryoprecipitate

28
Q

Liver Dz coagulopathy

A

Deficient 2,7,9,10,5, antithrombin, C, S

Generally balanced but low levels- balance is easily tipped

Tx: Cryo, FFP, platelets

29
Q

Activated C and s inactivates what

A

8 and 5 (cofactors needed for activation of 10 and 2

30
Q

ATD, PCD, PSD

A

Antithrombin, protein C, or protein S deficiency

Increaseed venous thrombosis- mostly after risk events
70% have clot by age 50

31
Q

Factor 5 leiden

A

Argenine 506, replaced with glutamine, factor 5 insucceptable to deactivation

VENOUS THROMBOSIS (DDVT/PE)

PTT will not slow with addition of activated C

32
Q

Prothrombin gene variation

A

elevated ThrombinII - stronger mRNA

VENOUS THROMBOSIS

33
Q

APLAS

A

Anti-phospholipid antibody syndrome - anticarrdiolipin can be positive too, along with lupis anticoagulant(prolongs dRVVT/PTT in vitro, clots in VIVO)
Possible thrombocytopenia
PTT prolongation that doesn’t correct! and is PL dependent
dilute Russell Viper Venom (dRVVT prolonged)

FETAL DEMISE
Venous and Arterial

34
Q

DIC Tx

A

Fix underlyying problem

Bleeding: CRYO 1st!!! (need fibrinogen to fix bleeding and make clots), FFP, Platelets

35
Q

When is Fibrinogen low

A

Extensive clotting - DIC

Decreased production- Liver Dz

36
Q

When are PT, PTT, and Fibrinogen normal

A

Factor 5 leiden
vW Dz
Factor 13 deficiency

02 Heme Lymph (16 decks)

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