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Gen Path Test 2 > Coagulative Disorders > Flashcards

Coagulative Disorders Flashcards

1
Q

Platelets

A

Adhesion -> Activation -> Aggregation

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2
Q

Coagulation

A

Activation -> Thrombin formation -> Fibrin formation, polymerization and stabilization

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3
Q

Primary Hemostasis:

Secondary Hemostasis:

A

Platelet plug formation.

Fibrin Clot Formation

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4
Q

Primary Hemo = Platelet response to vascular injury.

Membrane Receptor:

Adhesive Protein:

Appropriate Surface:

A

Glycoprotein 1b

von Willebrand Factor (vWF)

Subendothelial matrix (collagen)

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5
Q

Platelet activation:

Secretion = _____ and ____

Activation of ______ receptor

_______ reorganization

Shape Change - ____ to ____ (_____)

A

alpha-granules and dense bodies.

GP 2b/3b receptor

Phospholipid

Rounded discs to flat plates = Increased surface area

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6
Q

Platelet Aggregation

A

GP 2b/3a bind to fibrinogen with ADP = Fibrin

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7
Q

Fibrin Clot formation (2ndary Hemostasis):

Formation of _____

Formation of ____ _____ (with crosslinking of ____ by ______)

A

Thrombin

Fibrin clot

fibrin, factor 13a

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8
Q

Intrinsic Pathway:

Activation of Factor ____ by _____, followed by activation of Factor ____ by factor _____. Factor ____ activates Factor _____

A

12 by kallikrein.

11 by Factor 12a

Factor 11a activates Factor 9

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9
Q

Extrinsic Pathway:

Sequence of activation of Factor _____ by ______

A

Activation of Factor 7

By Tissue Factor

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10
Q

Common Pathway:

Activation of Factor ____ by ____ followed by conversion of ______ to ______, followed by conversion of ______ to ______

A

10 by 10a.

Prothrombin (II) to thrombin.

Fibrinogen (I) to Fibrin monomors

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11
Q

Fibrin Clot:

Formed when fibrin monomers generated by _____ polymerize. Made more stable by covalent crosslinking by factor _____

A

Thrombin.

Factor 13

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12
Q

Primary HemoStasis Regulation

A

NO, Prostacyclin PGI2, ADPase

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13
Q

Secondary Hemostasis Regulation

A

Antithrombin.
Protein C pathway (controls 5a and 8a).
Fibrinolytic system (excess clot)

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14
Q

Anti-Thrombins = _____ + ______

A

Serine Protease plus SERPIN

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15
Q

Antithrombin III:

In presence of ______ becomes activated so that it can _____

A

Heparin, complexes with thrombin = stops fibrin monomer production

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16
Q

Protein C System:

Activated Protein C plus ______ serve to inactivated ____ and _____

A

Protein S.

5a, 8a

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17
Q

Protein C or S deficiencies = ______.

Factor 5 leiden mutation promotes ______ due to _____

A

Hypercoaguable states.

Coagulation. Resistance to enzymatic inactivation by Protein C/S complex.

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18
Q

Plasmin = _______

A

Removes excess fibrin clot by chopping it into FDPs

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19
Q

Prothrombin Time (PT)

Screens or activity of ______.

_____ and ____ are added to patient’s plasma with ____

A

Extrinsic Pathway (Factors 2, 5, 7, 10, fibrinogen)

Phospholipid, tissue factor, Calcium

20
Q

Prothrombin Time (PT):

Forms basis of ______ (______)

11-13 seconds

A

International Normalized Ratio (INR)

Patient/control ratio = 0.8-1.2

Used to monitor patients on warfarin

21
Q

Partial Thromboplastin Time (PTT)
Screens for activity within _______

___ - ___ seconds

A

Intrinsic pathway (factors 12 activated, 11, 9, 7, 10, 5, 2 and fibrinogen)

28-35

22
Q

Disorders of Primary Hemostasis:

Clin Manifestations

Lab Findings

A

Mucocutaneous bleeding, petechial.
Excessive bleeding w/ trauma.

Prolonged BT/PFA-100, thrombocytopenia

23
Q

Secondary hemostasis disorders
Clin mani
Lab findings

A

Soft tissue bleeding and excessive bleeding with trauma.

Prolong PT and PTT and TT

24
Q

Regulatory Disorders of Hemostasis

Clinical Manif

Lab Manifes

A

Soft tissue bleeding, excessive bleeding with trauma.

Normal PT and PTT, normal bleeding time, normal platelet count

25
Q

Congenital Bleeding Disorders

A

Von willebrand dz, factor 8 deficiency, factor 9 deficiency

26
Q

Von Willebrand Dz

_______ disorder

Due to _______

________ is the dominant clinical manisfestation

A

Autosomal dominant.

abnormalities in vW factor

Mucocutaneous bleeding

27
Q

Classification of vWD

Type 1 =

Type 2 =

Type 3 =

A

Quantitative deficiency (partial)

Qualitative deficiency

Quantitative deficiency (total)

28
Q

vWD Clinical Manifestations

A

Epistaxis, ecchymoses, mucosal bleeding

29
Q

vWD Lab Features

BT/PFA-100: 
PTT:
VIII:
vWF:ag:
vWF:Rcof:
A 
Prolong
Usually prolonged
decreased to normal
decreased to normal 
decreased to normal
30
Q

Hemophilia A:

______ disorder

Deficiency in ______

Recurrent ______

Usually start in ____

Therapy =

A

X-linked recessive

Factor 8

Soft tissue bleeding

Early childhood

Factor 8 concentrates

31
Q

Hemophilia A Clinical Manifestations

____, _____ bleeding, _______ w/ trauma, _______ hematomas, ______ ______

A 
Hemarthrosis
Soft tissue
Excessive bleeding
Intramuscular
Intracerebral hemorrhage
32
Q

Lab Features of Hemophilia A

BT/PFA-100
PTT
Factor VIII
vWF:ag:
vWF:Rcof:
Factor 9:
A 
normal
prolonged
decreased
normal
normal
normal
33
Q

Hemophilia classification

Severe = \_\_\_\_% factor 8
Moderate = \_\_\_ % factor 8
Mild = \_\_\_\_%
A

Less than 1%.

1-5%

More than 5%

34
Q

Hemophilia B:

_____, ____ disorder

Defiency in _____

Similar presentation as factor 8

Treatment =

A

Sex-linked, recessive

Factor 9

Factor 9 Concentrates

35
Q

Mechanisms of Thrombocytopenia

A

Decreased platelet production

Increased destruction

Sequestration

Congenital vs acquired

36
Q

Immune Thrombocytopenic Purpura (ITP)

Acute = _____

______ common

____ Thrombocytopenia

Frequent ______

M:F ratio ____

A

Childhood.

Viral prodrome.

Severe

spontaneous remission

1:1

37
Q

Chronic ITP

Seen in ______

No ______

____ onset

_____ thrombocytopenia

______ spontaneous remission

M:F ratio _____

A

Adults

No antecedent infection

Gradual

Moderate

Infrequent

More common in females

38
Q

ITP Pathophysiology

Autoantibodies targeted at ______

____ and ____ common targets

______ bound to surface = ______/______ by _____

A

Platelet membrane antigens

GP Ib and 2b/3a

IgG, sequestration/destruction by reticuloendothelial system

39
Q

ITP clinical features

A

Petechial hemorrhages, gingival bleeding, ecchymoses, bleeding with trauma/surgery

40
Q

ITP Bone Marrow and blood findings:

_______ normal to increased

________ changes on blood smear review

Treatment

A

Megakaryocytes

No microangiopathic

Corticosteroids, IV ig, immunosuppresion, splenectomy

41
Q

Thrombotic Thrombocytopenic Purpura (TTP)

____ disorder characterized by _______ with formation of ______ throughout circulation

Due to defiency of _____, a MMP that degrades _____

Can be ____ or ______

Untreated = ____

A

Acute disorder
intravascular platelet activation
platelet-rich microthrombi

ADAMTS 13, vWF

Inherited or acquired

High mortality

42
Q

DIC:

Systemic formation of ____ and ____ with simultaneous _____ formation and consumption of ____ that promotes _____

A

Thrombin, plasmin

Microthrombi

Clotting factors

Bleeding

43
Q

DIC Clinical settings

A

Infections by gram negative sepsis

Tissue injury

Obstetrical complication

Malignancies

44
Q

DIC Clinical manifestations

A

Bleeding from multiple sites

Thromboembolic problems

Hypotension and shock

45
Q

DIC therapy

FFP =

Cryoprecipitates =

______

A

Coagulation and regulatory proteins

Fibrinogen, Factor 8, vWF

Platelets

Gen Path Test 2 (4 decks)

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