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BL/DD USMLE and Basics > BL - Hemostasis Defects > Flashcards

BL - Hemostasis Defects Flashcards

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USMLE® Step 1 flashcards
1
Q

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

A

Common Screening tests

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2
Q

Activated Partial Thromboplastin Time(aPTT) pathway

A

Citrated Plasma
–>

**Kaolin (surface activator)
phospholipid

–> incubated
–> recalcified
Measure time to clot

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3
Q

aPTT Measures the activity of the entire pathway except _________

A

Factor VII

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4
Q

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

A

Activated Partial Thromboplastin Time(aPTT)

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5
Q

Prothrombin Time(protime, PT) pathway

A

Citrated Plasma
–>

**Thromboplastin
(Tissue Factor)

–> incubated
–> recalcified
Measure time to clot

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6
Q

Excess thrombin is added to plasma

Detects low or abnormal fibrinogen, fibrin split products or heparin

Fibrinogen activity should also be measured

A

Thrombin Time

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7
Q

Protime/International Normalized Ratio(PT/INR)

A

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

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8
Q

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

A

fibrinogen

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9
Q

PT/INR Prolonged with vitamin K deficiency and________

A

liver disease

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10
Q

_______ measures platelet function, vessel wall and skin integrity

A

Bleeding Time/PFA

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11
Q

Common Congenital Disorders of Coagulation

A

Hemophilia A – Factor VIII (Classical)

Hemophilia B – Factor IX (Christmas Disease)

Hemophilia C – Factor XI

Von Willebrand Disease

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12
Q

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

A

Hemophilia A – Factor VIII (Classical)

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13
Q

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

A

Hemophilia B – Factor IX (Christmas Disease)

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14
Q

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

A

Von Willebrand Disease

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15
Q

Unusual Congenital Disorders of Coagulation

A

Less Common

  • Factor VII
  • Hypo or dysfibrinogenemia

Rare
-Factor XIII, V, X, and II

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16
Q

Differential Diagnosis of Long PTT- non bleeder

A

Non bleeders:

Factor XII deficiency
Lupus anticoagulant  (thrombophilic!)
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17
Q

Differential Diagnosis of Long PTT- Bleeder

A
  • Hemophilia A or B
  • Factor 11 deficiency
  • Acquired Hemophilia Factor 8 autoimmune
  • Severe von Willebrand Disease
  • *Heparin in sample
  • Fibrin split products
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18
Q

Long PTT means

A

It took “too long” for blood clotting

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19
Q

Hemophilia A or B can only be tested with

A

Elevated PTT is ONLY abnormal screening te

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20
Q

Hemophilia Bleeding Complications

A

Soft tissue hematoma

Joint bleeding

CNS bleeds – cause of death

Retroperitoneal or psoas bleeds

Post surgical or traumatic bleeds

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21
Q

Ecchymoses

A

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

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22
Q

________cause muscle damage, calcified pseudotumors and atrophy

A

Hematomas

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23
Q

Hemophilia Treatment

A

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

24
Q

Causes of a Prolonged PT ± PTT

A

Liver disease

Vitamin k deficiency

Warfarin or rat poison ingestion

25
Q

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

A

Abnormalities in Liver Disease

26
Q

Factor 11 deficiency

A

Hemophilia C

27
Q

Post prostate surgery with bleeding can be

A

Hemophilia C

28
Q

Decreased fibrinogen production and increased fibrinolysis can be a result of

A

Abnormalities in Liver Disease

29
Q

The ______measures the procoagulant

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

A

protime (PT)

30
Q

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

A

Thrombin Time (TT)

31
Q

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

A

Bleeding Time (BT)

32
Q

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

A

Platelet Function Analyzer

33
Q

Less than 1% factor activity – Severe _____

A

Hemophilia

34
Q

2% to 5% factor activity- _______ Hemophilia

A

Moderate

35
Q

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

A

PTT

36
Q

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.

A

vitamin K

37
Q

> 10% factor activity- _______- Hemophilia

A

Mild

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

38
Q

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.

A

warfarin

39
Q

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

A

extremely prolonged protime and PTT

40
Q

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

A

disseminated intravascular coagulation

41
Q

________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.

A

Thrombosis

42
Q

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.

A

The Lupus Anticoagulant

43
Q

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

A

protein C

44
Q

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

A

The four most common familial

congenital conditions which cause a hypercoagulable syndrome

45
Q

DIC mantra

A

treat the underlying disorder

Replace platelets and fibrinogen

Rarely: stop progression with anticoagulation

46
Q

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

A

Factor V Leiden

47
Q

Hypercoagulable States

A

Impairment of blood flow

Congenital deficiencies of endogenous anticoagulants

Cancer

Thrombocytosis – high platelets (P vera)

Antiphospholipid Syndrome – Lupus Anticoagulant

48
Q

in vitro prolonged PTT but causes in vivo thrombotic disorders

A

Lupus Anticoagulant

49
Q

Associated with lupus sometimes

Venous and arterial thrombosis

Hepatic vein thrombosis

Recurrent miscarriage

Migraine headaches

may indicate?

A

Antiphospholipid Syndrome – Lupus Anticoagulant

50
Q

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

A

Confirmation of Lupus Anticoagulant

51
Q

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 _________.

A

placental blood vessels

52
Q

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

A

Disseminated Intravascular Coagulation (DIC)

53
Q

Acquired Factor Inhibitors

A

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

54
Q

von Willebrand protein has two functions.

A

adhere platelets to exposed collagen

carry Factor VIII

55
Q

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

A

Factor XI Deficiency

BL/DD USMLE and Basics (55 decks)

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