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Clin Med2- Hematology > clotting disorders part 1 > Flashcards

clotting disorders part 1 Flashcards

1
Q

What are vitamin K dependent factors

A

2,7,9 and 10

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2
Q

What is Hemophilia A

A

congenital factor VIII (8) deficiency: clotting disorder - prolonged time or inability to clot
most common
x-linked recessive disorder - mostly affects males

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3
Q

what are the results of Hemophilia A

A

lack of factor VIII disrupts the cascade downstream
results in weak platelet plug
leads to weak/delayed fibrin clot
prone to developing antibody inhibitors to exogenous factor VIII

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4
Q

what is Hemophilia B

A

congenital factor IX (9)
also x-linked recessive (mostly males)
less common them Hem A
no racial differences

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5
Q

what are the results of Hemophilia B

A

lack of factor IX still disrupts the cascade
also results in weak platelet plug
leads to weak/delayed fibrin clot
AKA Christmas Disease (named after first known)

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6
Q

What is Hemophilia C

A

Factor XI (11) deficiency
hereditary but not x-linked
M = F
primarily jewish/ashkenazi/eastern european pts

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7
Q

what is the presentation of hemophilia

A

clinically indistinguishable
characterized by multiple episodes of ‘abnormal’ bleeding
*Hemarthrosis - bleeding into a joint
Mucous membrane bleeding
epistaxis
soft tissue hematomas
hematuria
internal hemorrhages

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8
Q

how is hemophilia worked up

A

prolonged activated partial thromboplastin time (aPTT)
thrombin time and prothrombin time (PT) / (PT/INR) are normal
definitive diagnosis with specific factor activity assay

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9
Q

what is a higher aPTT and PT/PT/INR indicative of

A

worse at clotting (higher number) - measure how long it takes to clot

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10
Q

how is hemophilia treated

A

mainstay is factor replacement with factor concentrate (prevention and treatment)
alternative for Hemophilia A is desmopressin (DDAVP)
- synthetic vasopressin - stimulates vWF production, only for mild to moderate disease- severe does not respond

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11
Q

What is the treatment for patients with hemophilia that develop antibody inhibitors

A

factor VII concentrate
this bypasses the defunct intrinsic pathway
increase production of factor X from other side - common pathway continues uninterrupted

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12
Q

what are general adjunctive treatments to AVOID in patients with hemophilia

A

avoid:
NSAIDs, anticoagulants (including ASA), and anti-platelet agents

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13
Q

what is hemophilia health maintenance

A

prenatal counseling/testing routinely done
primarily for mom with +FH and affected dad
done via gene sequencing on Male offspring
females generally not sequenced (Rare)
need to connect with HTC, travel with documentation
conferences and camps for families

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14
Q

what is Von Willebrands Disease (VWD)

A

deficiency in vWF
most common inherited coagulation disorder
autosomal dominant and recessive inheritance
M=F (F more symptomatic)
most not diagnosed until adulthood

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15
Q

what is the effect of VWD

A

vWF main role is to bind factor VIII to extends its half-life
promotes platelet aggregation and adhesion - binds vessel wall at site of injury
also activated factor X

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16
Q

what are the different types of VWD

A

Type 1, 2 and 3
Quantitative and Qualitative

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17
Q

what is Quantitative VWD

A

reduced levels of vWF that is present is still functional
Type 1 - autosomal dominant

Type 3 - most severe form - autosomal recessive

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18
Q

what is qualitiative VWD

A

total vWF levels normal but structure/function is not
Type 2vWFD - mostly autosomal dominant

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19
Q

what is Acquired vWD

A

secondary to some other disease process
mostly over age 40
Multiple myeloma, lymphoproliferative DO, autoimmune, Malignancy, DM

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20
Q

how is vWD screened

A

International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH - BAT)
- cant separate between types
- looks at 14 bleeding domains

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21
Q

what is the presentation of VWD

A

hemarthrosis much more rare*
mucuocutaneous bleeding more prominent*
nose bleeds
easy bruising
heavy menstruation*
GI bleeding
Excessive bleeding with injury/surgery
petechiae/purpura
internal bleeding rare

22
Q

how is VWD worked up

A

CBC often to r/o other causes
PT and aPTT often normal (aPTT may be prolonged, factor 8 still present and functional)
definitive diagnosis by looking at clotting factor function

23
Q

what is the treatment of type 1 VWD

A

DDAVP (desmopressin)
causes release of stored vWF and factor 8
used for episodic bleeding - with procedures
shouldn’t be given long term due to SE
not everyone responds well - usually evaluate prior to regulat use

24
Q

what is the treatment of VWD if DDAVP is failed and acute bleeding, undergoing longer procedures or type 3

A

replacement of vWF
vWF concentrate comes as vWF -containing factor 8 concentrate

25
Q

what the the types of hypercoagulable states

A

Primary and secondary/acquired

26
Q

what is a priamry hypercoagulable

A

impaired in: antithrombin, proteins C and S, Factor V
prothrombin gene mutation
antiphospholipid antibody syndrome

27
Q

what are the secondary/acquired hyperccoagulable states

A

malignancy
pregnancy
immobilization
surgery
trauma
medications
myeloproliferative disorder
nephrotic syndrome

28
Q

what are the natural anticoagulants

A

antithrombin
protein C
Protein S

29
Q

what leads to an overactive coagulation cascade

A

loss of negative feedback

30
Q

what is the presentation of someone in hyper coagulable states

A

primarily thromboembolic disease
- DVT
-PE
- MI
- Stroke
- pregnancy loss ***
- superficial phlebitis
- other venous clots: splenic, mesenteric, portal, hepatic

31
Q

What are the rare inherited deficiencies in clotting proteins

A

protein C deficiency
protein S deficiency
antithrombin deficiency

32
Q

what is the treatment of rare inherited deficiencies

A

anticoagulation

33
Q

what are the common inheritied blood clotting disorders

A

Factor V Leiden Mutation
Prothrombin Gene Mutation

34
Q

what is the treatment of common inherited blood clotting disorders

A

anticoagulation

35
Q

what is an acquired clotting disorder

A

antiphosphlipid antibody syndrome

36
Q

what is antiphospholipid antibody syndrome

A

AKA antiphospholipid syndrome, lupus anticoagulant syndrome, anticardiolipin antibody syndrome
F>M (pregnency relevant)
associated with autoimmune disorders and history of infection (Hep B, Hep C, Lyme)

37
Q

how is antiphospolipid antibody syndome (APS) diagnosed

A

serum test for antiphosphlipid antibodies

38
Q

what is the treatment of APS

A

anticoagulation

39
Q

what is the mechanism of action of heparin

A

binds to antithrombin III to speed up inactivation of Xa and IIb primarily (LMWH different, smaller structure)

40
Q

what falls under Heparin

A

LMWH/Enoxaprin (lovenox)
Unfractionated heparin

41
Q

what are the inibited clotting factors with Heparin use

A

Xa&raquo_space;> IIa (thrombin)
Xa and IIa (thrombin)

42
Q

what is the MOA for ASA

A

anti-platelet agent: blocks converstion of arachidonic acid to prostaglandins and TXA2 - inhibits platelet aggregation

43
Q

what is the inhibited clotting factors with ASA use

A

Thromboxane A2

44
Q

what is the MOA of Clopidogrel

A

anti-platelet agent: active metabolite blocks binding of ADP to platelet - inhibits platelet aggregation

45
Q

what is the MOA for Warfain

A

Vitamin K antagonist: functionally reduces vitamin K stores - reduced synthesis of vitamin K dependent clotting factors

46
Q

what are the vitamin K dependent factors

A

II, VII, IX and X (proteins C and S)

47
Q

what is the MOA for Xa inhibitor Fondaparinux (arixtra)

A

binds to antithrombin III to inhibit Xa

48
Q

what is the MOA for Xa inhibitors Rivaroxaban (xeralto) and Apixaban (eliquis)

A

directly inhibits Xa

49
Q

what is the MOA of Inhibitor Dabigatran (pradaxa)

A

directly inhibits thrombin

50
Q

what labs need to be monitored with the use of Heparin

A

activeated partial thromboplastin time (aPTT)
anti-factor Xa assay

51
Q

what labs need to be monitored with Warfain use

A

prothrombin time (PT/INR)

52
Q

what labs need to be monitored with Xa inhibitors

A

anti-factor Xa assay (not routinely done)

Clin Med2- Hematology (8 decks)

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