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Flashcards in Cogulation Disorders Deck (54):
1

PTT

Test function of common and intrinsic pathway (all factors except 7 and 13)

2

Platelets abnormalities - sign symptoms labs

1. Microhemorrhage (mucus bleeding , epistaxis, petechiae, purpura
2. Increased bleeding time
3. Possible decreased platelet count

3

Bernard-Soulier - PC, BT

PC: normal or decreased
BT: increased

4

Bernard-Soulier diagnosis

No agglutination on ristocetin cofactor assay

5

Bernard-Soulier pathophysiology

Deficiency of Gp1b (platelets - vWf adhesion)

6

Bernard-Soulier findings

1. Defects in platelet plug formation
2. Large platelets (younger)

7

PT

Test function of common and extrinsic pathway (factors 1, 2, 5, 7, 10)

8

Glanzmann thrombasthenia - PC,BT

PC: -
BT: increased

9

Glanzmann thrombasthenia pathophysiology

GpIIb/IIIa deficiency --> defects in platelet to platelet aggregation

10

Glanzmann thrombasthenia labs

Blood smear shows no platelet climbing

11

Glanzmann thrombasthenia - agglutination

Agglutation with ristocetin

12

Immune thrombocytopenia - PC, BT

PC: decreased
BT: increased

13

Immune thrombocytopenia treatment

1. Steroids
2. IV immunoglobulins
3. splenectomy (for refractory)

14

Immune thrombocytopenia labs

Increased megakaryocytes on bone bone marrow biopsy

15

Immune thrombocytopenia pathophysiology

Anti GpIIb/IIIa antibodies --> splenic macrophages consumption of platelet antibody complex
Commonly after viral illness

16

Immune thrombocytopenia is commonly due to

Viral illness

17

Thrombotic thrombocytopenic purpura - PC, BT

PC: decreased
BT: increased

18

Thrombotic thrombocytopenic purpura treatment

1. Plasmapheris
2. Steroids

19

Thrombotic thrombocytopenic purpura labs and symptoms

LDH increased
Schistocytes
symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

20

Thrombotic thrombocytopenic purpura pathophysiology

Inhibition or deficiency of ADAMTS 13 (vwf metalloprotease) --> decreased degradation of vwf multimers --> large vwf multimers --> platelets aggregation and thrombosis

21

Von willebrand disease - PC, BT, PT, PTT

PC normal
BT increased
PT normal
PTT increased or normal

22

Von willebrand disease mode of inheritance

AD

23

MC inherited bleeding disorder

Von willebrand disease (but mild)

24

Von willebrand disease diagnosis

No agglutination with ristocetin

25

Von willebrand disease - treatment

Desmopressin (release vWF stored in endothelium)

26

DIC - PC, BT, PT, PTT

PC decreased
BT increased
PT increased
PTT increased

27

Causes of DIC - results in

STOP Making New Thrombi
Sepsis (gram -), trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
results in widespread activation of clootting --> deficiency of factors --> bleeding state

28

DIC labs

1. Schistocytes
2. Increased d dimers
3. Decreased fibrinogen
4. DECREASED FACTORS V AND VIII

29

Prothrombin gene mutation

Mutation in 3 untranslated region --> increased production of prothrombin --> increased plasma levels and VENOUS thrombosis

30

Factor V Leiden - definition and mechanism

- Production of mutant factor V, resistant to pr C
- G --> A DNA mutation --> Arg506Gln mutation near the clevage site (from arginine to glutamine)

31

MCC of inherited hypercoagubility in Caucasians

Factor V Leiden

32

Protein C or S deficiency pathophysiology

Decreased ability to inactivate Va and VIIIa

33

Protein C or S deficiency increases the risk of

Thrombotic skin and subcutaneous necrosis with hemorrhage following administration of warfarin

34

Causes of Antithrombin deficiency

1. Inherited deficiency
2. Renal failure/nephrotic syndrome --> loss in urine

35

Antithrombin deficiency pathophysiology

Decreased inhibition of factors 2a and 10a

36

Antithrombin deficiency - PTT, PT, Thrombin time

no direct effects on the PTT, PT, or thrombin time --> diminishes the increase in PTT following heparin

37

Iron poisoning treatment

Chelation (iv deferoxamine, oral deferasixor) and dialysis

38

Iron poisoning mechanism

Cell death due to peroxidation of membrane lipids

39

Iron poisoning symptoms and signs

1. Nausea
2. Vomiting
3. Gastric bleeding
4. Lethargy
5. Scarring leading to GI obstruction

40

Iron poisoning - children

High mortality rate with accidental ingestion (adult iron tablets look like candy)

41

INR - definition/MC test in

international normalized ratio --> calculated from PT
1=normal
more than 1 = prolonged
MC test used to follow patients on warfarin

42

coagulation disorders - types

1. Hemophilia A, B, C
2. Vitamin K deficiency

43

Vitamin K deficiency - PT, PTT, BT

increased PT, PTT
normal BT

44

Vitamin K deficiency --> coagulation disorder due to

low activity of factors 2, 7, 9, 10, C, S

45

Hemophilia A, B, C - mechanism

Intrinsic pathway coagulation defect
A: deficiency of 8 (XR)
B: deficiency of 9 (XR)
C: deficiency of 11 (AR)

46

Hemophilia A, B, C - PTT, PT

normal PT
increased PTT

47

Hemophilia A, B, C - treatment

A: desmopressin + factor 8 concentrate
B: factor 9 concentrate
C: Factor 11 concentrate

48

Hemophilia A, B, C - presentation

Macrohemorrhage -->hemarthroses (bleeding into joints such as knee), easy bruising, bleeding after trauma or surgery (eg. dental)

49

Hemolytic-uremic syndrome - PC, BT

decreased PC
increased BT

50

Hemolytic-uremic syndrome - characterised by

1. thrombocytopenia
2. microangiopathic hemolytic anemia
3. acute renal failure

51

Hemolytic-uremic syndrome - typically seen in/due to

children, accompanied by diarrhea and commonly caused by Shiga-toxin-producing E-coli (eg. 157:H7)
in adults does not present with diarrhea
STEC infection is not required

52

Hemolytic-uremic syndrome - treatment

plasmapheresis

53

Hemolytic-uremic syndrome - symptoms

neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

54

Factor V Leiden - complications

1. DVT
2. cerebral vein thromboses
3. recurrent pregnancy loss