BL - Hemostasis Defects

Question 1

aPTT, Protime (INR), Thrombin time platelet count are all?

Answer 1

Common Screening tests

Question 2

Activated Partial Thromboplastin Time(aPTT) pathway

Answer 2

Citrated Plasma
–>

**Kaolin (surface activator)
phospholipid

–> incubated
–> recalcified
Measure time to clot

Question 3

aPTT Measures the activity of the entire pathway except _________

Answer 3

Factor VII

Question 4

Test Useful to detect Factor VIII, IX, XI, XII deficiencies

Answer 4

Activated Partial Thromboplastin Time(aPTT)

Question 5

Prothrombin Time(protime, PT) pathway

Answer 5

Citrated Plasma
–>

**Thromboplastin
(Tissue Factor)

–> incubated
–> recalcified
Measure time to clot

Question 6

Excess thrombin is added to plasma

Detects low or abnormal fibrinogen, fibrin split products or heparin

Fibrinogen activity should also be measured

Answer 6

Thrombin Time

Question 7

Protime/International Normalized Ratio(PT/INR)

Answer 7

Thromboplastin varies by manufacturer and the time in seconds is “normalized” by adjusting for known potency

Question 8

PT/INR Prolonged with II, VII, V, X and ____ deficiencies

Answer 8

fibrinogen

Question 9

PT/INR Prolonged with vitamin K deficiency and________

Answer 9

liver disease

Question 10

_______ measures platelet function, vessel wall and skin integrity

Answer 10

Bleeding Time/PFA

Question 11

Common Congenital Disorders of Coagulation

Answer 11

Hemophilia A – Factor VIII (Classical)

Hemophilia B – Factor IX (Christmas Disease)

Hemophilia C – Factor XI

Von Willebrand Disease

Question 12

genetic deficiency in clotting factor VIII, which causes increased bleeding and usually affects males.

Answer 12

Hemophilia A – Factor VIII (Classical)

Question 13

a blood clotting disorder caused by a mutation of the factor IX gene, leading to a deficiency of factor IX. It is the second-most common form of haemophilia

Answer 13

Hemophilia B – Factor IX (Christmas Disease)

Question 14

arises from a qualitative or quantitative deficiency of von Willebrand factor (vWF), a multimeric protein that is required for platelet adhesion.

Answer 14

Von Willebrand Disease

Question 15

Unusual Congenital Disorders of Coagulation

Answer 15

Less Common

• Factor VII
• Hypo or dysfibrinogenemia

Rare
-Factor XIII, V, X, and II

Question 16

Differential Diagnosis of Long PTT- non bleeder

Answer 16

Non bleeders:

Factor XII deficiency
Lupus anticoagulant  (thrombophilic!)

Question 17

Differential Diagnosis of Long PTT- Bleeder

Answer 17

• Hemophilia A or B
• Factor 11 deficiency
• Acquired Hemophilia Factor 8 autoimmune
• Severe von Willebrand Disease
• *Heparin in sample
• Fibrin split products

Question 18

Long PTT means

Answer 18

It took “too long” for blood clotting

Question 19

Hemophilia A or B can only be tested with

Answer 19

Elevated PTT is ONLY abnormal screening te

Question 20

Hemophilia Bleeding Complications

Answer 20

Soft tissue hematoma

Joint bleeding

CNS bleeds – cause of death

Retroperitoneal or psoas bleeds

Post surgical or traumatic bleeds

Question 21

Ecchymoses

Answer 21

a subcutaneous purpura (extravasation of blood) larger than 1 centimeter or a hematoma, commonly called a bruise

Question 22

________cause muscle damage, calcified pseudotumors and atrophy

Answer 22

Hematomas

Question 23

Hemophilia Treatment

Answer 23

Recombinant synthetic factor treatment is effective and given 2-3 times per week IV to prevent disability.

Question 24

Causes of a Prolonged PT ± PTT

Answer 24

Liver disease

Vitamin k deficiency

Warfarin or rat poison ingestion

Question 25

Decreased vitamin k dependent carboxylation of II, VII, IX and X can be a result of

Answer 25

Abnormalities in Liver Disease

Question 26

Factor 11 deficiency

Answer 26

Hemophilia C

Question 27

Post prostate surgery with bleeding can be

Answer 27

Hemophilia C

Question 28

Decreased fibrinogen production and increased fibrinolysis can be a result of

Answer 28

Abnormalities in Liver Disease

Question 29

The ______measures the procoagulant | activity of the factors VII, X, V, II and fibrinogen. This is the extrinsic pathway

Answer 29

protime (PT)

Question 30

measures the procoagulant activity of fibrinogen and is also very sensitive to the anticoagulant effect of heparin or fibrin split products.

Answer 30

Thrombin Time (TT)

Question 31

measures the platelet and vessel interaction, as well as the number and the function of platelets.

Answer 31

Bleeding Time (BT)

Question 32

A new device, the _________, can perform an in vitro bleeding time. It also can determine platelet response to agonists.

Answer 32

Platelet Function Analyzer

Question 33

Less than 1% factor activity – Severe _____

Answer 33

Hemophilia

Question 34

2% to 5% factor activity- _______ Hemophilia

Answer 34

Moderate

Question 35

In Factor VII deficiency only the protime (PT) is prolonged. The______is normal.

Answer 35

PTT

Question 36

hepatic function can cause deficiencies of the clotting factors. This is especially true for Factor V and for the ______dependent Factors, II, VII, IX, and X.

Answer 36

vitamin K

Question 37

> 10% factor activity- _______- Hemophilia

Answer 37

Mild diagnosed after a bad traumatic event or after a bad result from surgery.

Question 38

Vitamin K deficiency and _____ administration which interfere with the vitamin K utilization, are very common causes of a prolonged protime with normal or slightly prolonged PTT.

Answer 38

warfarin

Question 39

Long acting fat soluble rat poisons are used in suicide attempts. These patients present with _____ labs and require very intensive vitamin K therapy for months

Answer 39

extremely prolonged protime and PTT

Question 40

Massive trauma, hemorrhagic or septic shock, amniotic fluid embolism, burns, acute leukemia or transfusion and drug reactions can all cause _________

Answer 40

disseminated intravascular coagulation

Question 41

________is the formation and propagation of clot within the vasculature; this term refers to an abnormal or pathologic process with imbalance in the hemostatic system.

Answer 41

Thrombosis

Question 42

a very common acquired abnormality which results in a hypercoaguable state. The anticoagulant is an IgG antibody, which reacts against phospholipid in the platelet membrane or endothelial cell.

Answer 42

The Lupus Anticoagulant

Question 43

Factor V Leiden hypercoagulability results from a mutation in Factor V so that it is not inactivated by______.

Answer 43

protein C

Question 44

deficiencies of antithrombin, protein C and protein S or resistance to protein C (Factor V Leiden) have what in common

Answer 44

The four most common familial | congenital conditions which cause a hypercoagulable syndrome

Question 45

DIC mantra

Answer 45

treat the underlying disorder Replace platelets and fibrinogen Rarely: stop progression with anticoagulation

Question 46

_____________hypercoagulability results from a mutation in Factor V so that it is not inactivated by protein C.

Answer 46

Factor V Leiden

Question 47

Hypercoagulable States

Answer 47

Impairment of blood flow Congenital deficiencies of endogenous anticoagulants Cancer Thrombocytosis – high platelets (P vera) Antiphospholipid Syndrome – Lupus Anticoagulant

Question 48

in vitro prolonged PTT but causes in vivo thrombotic disorders

Answer 48

Lupus Anticoagulant

Question 49

Associated with lupus sometimes Venous and arterial thrombosis Hepatic vein thrombosis Recurrent miscarriage Migraine headaches may indicate?

Answer 49

Antiphospholipid Syndrome – Lupus Anticoagulant

Question 50

Russell Viper Venom Test (RVVT) or Platelet Neutralization Test with correction are common tests for?

Answer 50

Confirmation of Lupus Anticoagulant

Question 51

Even though the PTT is prolonged in vitro, the patients do not have bleeding. instead they have a thrombotic syndrome which includes deep vein thrombosis, pulmonary embolism, thrombotic strokes and recurrent miscarriage due to thrombotic disease of the _________.

Answer 51

placental blood vessels

Question 52

The most consistent laboratory findings are that the fibrinogen level has decreased markedly and the platelet count is low.

Answer 52

Disseminated Intravascular Coagulation (DIC)

Question 53

Acquired Factor Inhibitors

Answer 53

acquired Factor VIII inhibitor only laboratory test to be abnormal will be the PTT excellent long-term prognosis

Question 54

von Willebrand protein has two functions.

Answer 54

adhere platelets to exposed collagen | carry Factor VIII

Question 55

The classic presentation is post-operative hemorrhage since most people do not have spontaneous bleeding.

Answer 55

Factor XI Deficiency