Bleeding disorders Flashcards

1
Q

HIgh INR

Etiology

A
  1. Some anticoagulants;
  2. Decreased synthesis of clotting factors (liver disease, Vitamin K deficiency including malnutrition, malabsorption, antibiotics);
  3. Increased consumption of clotting factors (sepsis/DIC)
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2
Q

PT

What does it test for?

A

Extrinsic and common pathway

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3
Q

PTT

What does it test for?

A

Intrinsic and common pathway

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4
Q

High PTT

Etiology?

A
  1. Some anticoagulants;
  2. Von Willebrand disease
  3. Hemophilia
  4. Antiphospholipid antibodies
  5. Sepsis/DIC
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5
Q

HIgh fibrinogen

Etiology

A

Acute phase reactant

Pregnancy

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6
Q

Low fibrinogen

Etiology

A

DIC

Liver failure

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7
Q

Acute phase reactant

What does it include?

A

C3, C4, fibrinogen, ESR, CRP, ferritin, hepcidin

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8
Q

D-dimer–high

Etiology

A
PE/DVT
Arterial clots
Sepsis/DIC
Malignancy
Recent surgery or trauma
Liver disease
Pregnancy
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9
Q

Coagulation profile

A

Plt, PT, PTT, D-dimer, fibrinogen

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10
Q

Algorithm for investigation of abnormal coagulation tests.

If PT or PTT and PT both prolonged, what’s your next step?

A

Give Vitamin K

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11
Q

Algorithm for investigation of abnormal coagulation tests.

PT or PTT and PT both prolonged s/p Vitamin K

A
  1. If responded–>Vitamin K deficiency

2. If not responded–>Liver disease assessment

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12
Q

Algorithm for investigation of abnormal coagulation tests.

PT or PTT and PT both prolonged 2/2 not vitamin K nor liver disease

A

Check mixing study

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13
Q

Algorithm for investigation of abnormal coagulation tests.

If Mixing study corrected

A

Due to factor deficiency

  1. Von Willebrand disease;
  2. Specific factor activity levels
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14
Q

Algorithm for investigation of abnormal coagulation tests.

If Mixing study not corrected

A

Due to antibody presentation

  1. Check antiphospholipid antibodies;
  2. Specific factor inhibitors.
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15
Q

Normal hemostasis

Intrinsic pathway factors

A

XII, XI, IX, VIII

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16
Q

Normal hemostasis
Intrinsic pathway factors
What is the test for?

A

PTT

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17
Q

Normal hemostasis

Extrinsic pathway factors

A

VII

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18
Q

Normal hemostasis
Extrinsic pathway factors
What is the test for?

A

PT

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19
Q

Normal hemostasis

What is the common pathway factors

A

X, V, II, fibrinogen

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20
Q

Prolonged PT, normal aPTT

DDx

A
Factor VII deficiency
DIC
Liver disease
Vitamin K deficiency
Warfarin ingestion
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21
Q

Normal PT, prolonged aPTT

DDx

A

Deficiency of VIII, IX, XI or XII;
von Willebrand disease (if severe and factor VIII level is low);
Heparin exposure

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22
Q

Prolonged PT and aPTT

DDx

A
Deficiency of factor V, X, II or fibrinogen;
Severe liver disease;
DIC;
Vitamin K deficiency;
Warafin use;
Heparin overdose;
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23
Q

Normal PT and aPTT

DDx

A

Platelet dysfunction (acquired and congenital);
von Willebrand disease (if mild and factor VIII level is not too low);
Scurvy;
Ehlers-Danlos syndrome;
Hereditary hemorrhagic telangiectasia;
Deficiency of factor XIII

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24
Q

Bleeding into muscles and joints

A

Humoral clotting factor disorders

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25
Q

Mucosal bleeding

A

Primary hemostasis

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26
Q

Bleeding disorders

What signs in women menstrual history indicating underlying bleeding disorder?

A

Nighttime “flooding”;
Passing clots larger than a quarter;
Duration longer than 8 days;
Development of iron deficiency.

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27
Q

Congenital bleeding disorder
Hemophilia A and B
What is the genetic?

A

X-linked recessive

28
Q

Congenital bleeding disorder
Hemophilia A and B
What are the deficient factors?

A

Hemophilia A: VIII

Hemophilia B: XI

29
Q

Congenital bleeding disorder
Hemophilia A and B
Sx of severe hemophilia?

A

Factor level <1%
Joint bleeding, retroperitoneal bleeding, intramuscular bleeding and intracranial bleeding;
Children: bleeding after circumcision or loos of deciduous teeth.

30
Q

Congenital bleeding disorder
Hemophilia A and B
Sx of moderate hemophilia?

A

Factor level between 1% to 5%

Present in adult, unlikely to be spontaneous

31
Q

Congenital bleeding disorder
Hemophilia A and B
Sx of mild hemophilia?

A

Factor level>5%

Present in adult, unlikely to be spontaneous

32
Q

Congenital bleeding disorder
Hemophilia A and B
What are the concerns for older patients?

A

HIV and HCV infection from blood product transfusion

33
Q

Congenital bleeding disorder
Hemophilia A and B
Rx

A

Replacing factor VIII and IX

34
Q

Congenital bleeding disorder
Hemophilia A and B
What are the complications 2/2 to treatment?

A

Antibody development to factor VIII and IX.

35
Q

Congenital bleeding disorder
Hemophilia A and B
How to test alloantibodies against VIII and IX?

A

Bethesda titer

36
Q

Congenital bleeding disorder
Hemophilia A and B
What is the typical lab results show?

A

Prolonged aPTT and normal PT;
aPTT mixing study will fully correct;
Measure baseline factor activity/

37
Q

Congenital bleeding disorder
von Willebrand disease
Epi:

A

Most common inherited bleeding disorder.

38
Q

Congenital bleeding disorder
von Willebrand disease
Sx:

A

Nosebleeds in children, easy bruising, bleeding gums, postsurgical bleeding, GYN problems

39
Q

Congenital bleeding disorder
von Willebrand disease
What are the GYN presentations?

A

Heavey menstrual bleeding;
Endometriosis;
Miscarriages
Postpartum hemorrhage

40
Q

Congenital bleeding disorder
von Willebrand disease
How many types of VW disease are there?

A

3

41
Q

Congenital bleeding disorder
von Willebrand disease
Dx:

A
vWF antigen and activity level <30%;
Factor VIII levels may be normal or low;
Platelet function analyzer-100 (PFA-100) is prolonged;
PT normal;
PTT may be normal or slightly prolonged.
42
Q

Congenital bleeding disorder
von Willebrand disease
Treatment for heavy menstrual bleeding?

A

Estrogen-containing oral contraceptives;
Desmopressin;
Antifibrinolytic agents such as tranexamic acid or e-aminocaproic acid during menses

43
Q

Congenital bleeding disorder
von Willebrand disease
Rx:

A

Desmopressin preoperative at surgical prophylaxis

If severe bleeding episodes or prophylaxis for major surgeries- give factor VIII and vWF

44
Q

Congenital bleeding disorder
von Willebrand disease
Type 2

A

Production of an abnormally functioning vWF molecules

45
Q

Congenital bleeding disorder
von Willebrand disease
Type 3

A

Near lack of vWF, very low factor VII levels

46
Q

Acquired bleeding disorder
Coagulopathy of liver disease
Which factor is not produced in liver?

A

Factor VIII, produced in extrahepatic endothelia cell.

47
Q

Acquired bleeding disorder
Coagulopathy of liver disease
What is the lab results?

A

Prolonged PTT and PT;
Low fibrinogen level;
Supranormal factor VIIIlevels

48
Q

Acquired bleeding disorder
Coagulopathy of liver disease
Rx

A

If bleeding:
Cryoprecipitate, FFP and platelet
If vitamin K deficiency–> Vitamin K

49
Q

Acquired bleeding disorder
Acquired hemophilia
Presentation

A

Elderly people without family or PMHx of bleeding disorder;
Acute new onset bleeding;
Mucocutaneous or intramuscular

50
Q

Acquired bleeding disorder
Acquired hemophilia
Lab findings

A

Normal PT;
Significantly prolonged aPTT;
1:1 mixing study unsuccessful at completely correting the aPTT

51
Q

Acquired bleeding disorder
Acquired hemophilia
Mechanism

A

Autoantibody against factor VIII

52
Q

Acquired bleeding disorder
Acquired hemophilia
Etiology

A
Associated with underlying disease:
SLE
Lymphoproliferative disorder
Solid tumors
More common idiopathic
53
Q

Acquired bleeding disorder
Acquired hemophilia
Rx

A

Immunosuppression to eradicate inhibitor;

Recombinant activated factor VII

54
Q

Acquired bleeding disorder
DIC
Lab features:

A

Elevated PT (due to consumption of factor VII);
Elevated PTT;
Low fibrinogen;
Elevated D-dimers
Low plt;
Microangiopathic hemolytic anemia with schistocytes on the peripheral blood smear

55
Q

Acquired bleeding disorder
DIC
Rx

A
  1. Underlying causes;
  2. If bleeding or at risk for bleeding–> plt, ffp, or replacing clotting factors or cyroprecipitate to replace fibrionoten
56
Q

ITP

What to test if found to have ITP

A

HIV, hepatitis (HBV, HCV), MPNs, Collagen vascular disease

57
Q

ITP

What to do if plt<10

A

Spontaneous bleeding, bleeding precautions

58
Q

ITP

What to do if plt>30

A

NTD

59
Q

ITP

what to do if plt<30

A

Dexamethasone->IVIG->Rituximab->Romiplostin/Splenectomy

60
Q

ITP

What to do if plt<50 and needs surgery

A

IVIG and transfuse

61
Q

ITP

What to do if plt<50 and bleeding

A

IVIG and plt transfuse

62
Q

ITP

What to do if plt<100 with intracranial bleeding

A

IVIG and plt transfusion

63
Q

ITP

Plt threshold for central line

A

> 20

64
Q

ITP

plt threshold for abdominal tap

A

> 30

65
Q

ITP

Plt threshold for spinal tap

A

> 50