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CM 1A: Heme/Onc/Endo > Lecture 7: Bleeding Disorders > Flashcards

Lecture 7: Bleeding Disorders Flashcards

1
Q

What two things in the history help determine bleeding disorder etiology?

A

Bleeding site
Underlying bleeding tendency presence

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2
Q

What does a mucocutaneous bleed suggest?

A

Qualitative/quantitative platelet disorder

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3
Q

What does a joint or soft tissue bleed suggest?

A

Coag factor disorder

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4
Q

What should an initial panel of lab tests include to help determine bleeding disorder etiology?

A

CBC
Peripheral blood smear
Coag panel (INR/PTT/PT)
CMP

Additional:
Bleeding time
PFA-100

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5
Q

What is a PFA for?

A

Whole blood through a capillary with platelet agonists is measured.

More sensitive and reproducible than bleeding time.

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6
Q

What AC is measured with aPTT? Why?

A

UFH, because aPTT measures intrinsic pathway, which has thrombin.

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7
Q

What AC is measured with PT? Why?

A

Warfarin, because PT measures extrinsic pathway, which is more Vit-K dependent.

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8
Q

What other disease can PT monitor?

A

Liver, bc Vit K

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9
Q

What is INR?

A

International normalized ratio.

It is the ratio of PT to a standard sample.

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10
Q

What does high INR indicate?

A

Higher risk of bleeding.

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11
Q

What is the mnemonic for PTT and PT?

A

PTT = play table tennis (inside)
PT = play tennis (outside)

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12
Q

How are coag factors measured?

A

PT and aPTT can be compared in a sample plasma to a commercial plasma deficient in certain factors.

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13
Q

When do I use aPTT and PT tests together?

A

To monitor the transition between heparin and warfarin

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14
Q

What does a primary hemostasis disorder affect?

A

Mucous membrane bleeding, epistaxis, and petechiae.

Affects bleeding time and platelet counts.

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15
Q

What does a secondary hemostasis disorder affect?

A

Hemarthrosis, intracerebral hemorrhage, and deep tissue hematomas.
Affects PT and PTT.

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16
Q

What is hemophilia A?

A

Congenital deficiency of Factor 8. (vWF related)

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17
Q

What is hemophilia B?

A

Congenital deficiency of Factor 9

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18
Q

What kind of inheritance is hemophilia A?

A

X-linked recessive

1 in 5000 male live births

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19
Q

What kind of inheritance is hemophilia B?

A

X-linked recessive

1 in 25000 male live births

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20
Q

How do female carriers of hemophilia present?

A

Asymptomatic.

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21
Q

How does severe hemophilia present commonly?

A

Early childhood spontaneous bleeds into joints or soft tissues.

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22
Q

How does mild hemophilia present commonly?

A

Excess bleeding post hemostatic challenge like surgery.

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23
Q

What is the secondary concern in patients with hemophilia?

A

They can develop inhibitors to the factors they are deficient in.

30% in A

5% in B

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24
Q

How does factor inhibition present in hemophiliacs?

A

Bleeding episodes resistant to factor 8 or 9 concentrate.

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25
Q

How do we diagnose hemophilia?

A

Low factor 8 or 9 ACTIVITY.
Prolonged aPTT

Note:
NORMAL PT/INR
NORMAL CBC

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26
Q

How much activity of the factors does hemostasis normally require?

A

At least 25%

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27
Q

What is considered mild factor 8 activity?

A

5-40%

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28
Q

How do I treat a bleed in a mild hemophilia A?

A

DDAVP

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29
Q

How do I treat bleeds in a moderate-severe hemophilia A?

A

Factor 8 concentrate

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30
Q

How do I treat bleeds in hemophilia B?

A

Factor 9 concentrate

DDAVP DOES NOT WORK.

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31
Q

What is a common NSAID used to treat hemophilia?

A

Celebrex only. COX-2 inhibitor

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32
Q

How often do hemophilia patients require factor concentrate infusions?

A

up to 3x a week

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33
Q

What other med is common for a hemophiliac to take besides celebrex?

A

Opioid analgesics

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34
Q

What is the leading cause of death in hemophiliacs?

A

Transfusion-obtained HIV/AIDS

Hepatitis/Cirrhosis

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35
Q

What is the second leading cause of death in hemophiliacs?

A

ICH

It is also the most common cause of death dt hemorrhage.

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36
Q

What is the prognosis for a mild hemophiliac?

A

Normal life if they are treated prophylactically.

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37
Q

What are counseling tips for hemophiliacs?

A

AVOID CONTACT SPORTS
Monitor body for bruising/etc

Home infusion technique
Have a hematologist and hemophilia center you attend regularly.

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38
Q

What is the most common inherited bleeding disorder?

A

vW disease

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39
Q

What are the types of vWD?

A

Type 1 = Quantitative defect
Type 2 = Qualitative defect
Type 3 = Profound quantitative defect.

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40
Q

How is vWD inherited?

A

1 is autosomal dominant
2 is autosomal dominant primarily, recessive sometimes.
3 is autosomal recessive.

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41
Q

What is the most common type of vWD?

A

Type 1 (75% of cases)

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42
Q

What vWD type presents similarly to hemophilia A?

A

Type 3

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43
Q

What are the most common signs of vWD?

A

Nosebleeds and hematomas

44
Q

What is a concern in women with vWD?

A

Menorrhagia is 5x more likely.

45
Q

How does bleeding time present in vWD?

A

Can be normal in type 1.

Non-specific.

46
Q

How does aPTT present in vWD?

A

Mildly prolonged in only half of pts.

47
Q

How does PT present in vWD?

A

Normal.

48
Q

What kind of blood type has the lowest levels of vWF?

A

Type O
):

49
Q

What causes levels of vWF to vary from person to person?

A

Stress
ABO type

50
Q

What is the treatment protocol for vWD?

A

DDAVP
rVWF
vWF/F8 concentrates

DDAVP is only for mild.

51
Q

What is factor 11 deficiency sometimes known as?

A

Hemophilia C.

52
Q

How is Factor 11 deficiency inherited?

A

Autosomal recessive.

Most prevalent among Ashkenazi jewish descent.

53
Q

How does Factor 11 deficiency present?

A

Mild bleeding.

54
Q

How is Factor 11 deficiency treated?

A

F11 concentrate.

FFP if no concentrate available.

55
Q

What are the two primary causes of thrombocytopenia?

A

Increased platelet destruction
Decreased platelet production

56
Q

What can cause increased sequestration of platelets?

A

Hypersplenism

57
Q

What are common causes of a destructive or consumption thrombocytopenia?

A

Splenomegaly/hypersplenism
Antibody mediated destruction
Drug related destruction
Massive bleeding associated with consumption
Diffuse thrombus formation

58
Q

What is ITP?

A

Immune thrombocytic purpura

Auto-antibodies against antigens on the PLT surface, causing destruction.

59
Q

When is ITP commonly seen?

A

60% in children post URI
Rare in adults
Diagnosis of exclusion

60
Q

How does ITP present?

A

Sudden appearance of petechial rash in an otherwise healthy child.

PE should reveal cutaneous bleeding (also mucosal bleeding common in 40%)

61
Q

What is the most severe consequence of ITP?

A

ICH

62
Q

How is ITP diagnosed?

A

Isolated thrombocytopenia with no other apparent causes.

ITP = ISOLATED

63
Q

What are the initial labs for ITP?

A

CBC and peripheral blood smear
Findings INCONSISTENT with ITP

64
Q

How do you manage ITP?

A

Watch it.

Intervene if any life-threatening stuff happens.
AVOID ACs and APs until thrombocytopenia is cleared.

Refer to heme :)

65
Q

What is the treatment for ITP if needed?

A

Corticosteroids FIRST LINE

IVIG can be added.

Platelet transfusions can be given for serious bleeding.

Splenectomy may be necessary if >6mo duration.

66
Q

What is the underlying mechanism in drug-related platelet destruction?

A

Immune-mediated.

67
Q

How does drug-related platelet destruction present?

A

Severe thrombocytopenia & mucocutaneous bleeding 7-14 days post exposure to new drug.

68
Q

What is the primary drug class to be wary of in drug-related platelet destruction?

A

Antibiotics

69
Q

How is bleeding-associated consumption treated?

A

Blood product infusion and stoppage of bleeds.

70
Q

What is the most common cause of hypoproliferative thrombocytopenia?

A

Bone Marrow Failure (AKA lack of PLT production)

71
Q

What is the tx for hypoproliferative thrombocytopenia?

A

Infectious/viral should return to normal on its own.

Malignancies may require cancer tx.

Aplasia will require stem cell transplant.

72
Q

What kind of platelet disorder is ITP, drug-induced thrombocytopenia and hypoproliferative thrombocytopenia?

A

Quantitative

73
Q

What is the most common type/origin of qualitative PLT disorders?

A

Iatrogenic or Acquired.

Congenital is rare.

74
Q

What is a lab test that helps determine qualititative PLT disorders well?

A

Peripheral blood smears.

75
Q

How do qualitative plt disorders typically manifest initially?

A

Mucous membrane bleeding

76
Q

How are most qualitative plt disorders treated?

A

Platelet infusion

77
Q

What is the most common iatrogenic cause of qualitative plt disorders?

A

ASA
Plavix
NSAIDs

78
Q

What drugs cause irreversible plt dysfunction?

A

ASA and Plavix.

Must wait for PLTs to be cleared.

79
Q

What drugs cause reversible plt dysfunction?

A

NSAIDs.

Function returns in 12-24 hrs.

80
Q

If we have an emergency and a patient was still on plavix prior to surgery with a qualitative plt defect, what should we do?

A

PLT infusion should be sufficient to overcome acquired defect.

81
Q

What are the two types of Thrombotic microangiopathy?

A

TTP
HUS

82
Q

What are the primary characteristics of thrombotic microangiopathy?

A

Incorporation of plts into the microvasculature.
Shearing of erythrocytes in microcirculation results in microangiopathic hemolytic anemia

83
Q

What is the main cause of TTP?

A

Unknown.

HOWEVER…

Usually have large multimers of vWF and lack ADAMTS13, which is the protease to break it down.

84
Q

Who is TTP more common in?

A

Adults

85
Q

Who is ITP more common in?

A

Children post URI

86
Q

How does TTP present?

A

Neurologic dysfunction (Stroke-like presentation)
Petechiae
Fever (50%)
Hemoglobinuria

87
Q

What is uncommon in TTP presentation?

A

Severe bleeding.

88
Q

How is TTP diagnosed?

A

CBC with minor leukocytosis, minor Hgb depression, and thrombocytopenia.

Peripheral smear should show mod-sev schistocytes

Normal PT/PTT
Normal - mild D-Dimer
High fibrinogen
LDH and bilirubin elevation to check hemolysis status.

NEGATIVE direct Coombs.

89
Q

What is the treatment for TTP?

A

Plasma exchange via FFP.

If no exchange possible, FFP is sufficient until transferred.

90
Q

What is the mortality of TTP without an FFP transfusion?

A

95%

91
Q

What is in second-line treatment for TTP?

A

Rituximab, corticosteroids, IVIG, vincristine, cyclyphosphamide, and splenectomy

92
Q

How is HUS characterized?

A

Progressive renal failure with associated hemolytic microangiopathy and thrombocytopenia.

93
Q

What is HUS the most common cause of?

A

Acute renal failure in CHILDREN.

94
Q

What is the primary pathophysiological event in HUS?

A

Endothelial cell damage

95
Q

How does HUS commonly present?

A

Prodromal GE with fever and bloody diarrhea for 2-7 hrs prior to onset of renal failure.

96
Q

How does typical HUS categorize as?

A

Shiga-like toxin (Stx) secondary to E Coli serotype 0157:H7

97
Q

How is HUS diagnosed?

A

BMP w/ elevated BUN/CR
CBC with severe anemia and thrombocytopenia.
Peripheral smear with schistocytes.
Bilirubin/LDH elevation.

Stool sample for E.coli and shigella

98
Q

How is HUS treated?

A

Typical HUS is supportive care only.
Atypical is Plasma exchange.

99
Q

What are the key differences between HUS and TTP?

A

HUS usually has no fever post GE phase and present with antecedent GE.

100
Q

What is DIC caused by?

A

Uncontrolled/systemic activation of coagulation, causing thrombocytopenia and depletion of coag factors and fibrinogen.

AKA you clot so much that you can’t clot anything after.

101
Q

What is the mnemonic for DIC causes?

A

STOP Making New Thrombi

Sepsis
Trauma
Obstetric complications (pre-eclampsia)
Pancreatitis (acute)
Malignancy (blood/brain)
Nephrotic syndrome
Transfusion (Hemolytic reaction)

102
Q

How does DIC present?

A

Bleeding at multiple sites.
Purpura fulminans
Petechiae

103
Q

How is DIC diagnosed?

A

Elevated PT and PTT
Low levels of fibrinogen
D-dimer elevated and FDPs
Schistocytes in 10-20% of pts.

104
Q

What are the main tx options for DIC?

A

Get plts above 20k or 50k if ICH
Cryoprecipitate
FFP
PRBCs

105
Q

What is the secondary tx option for DIC?

A

Heparin in persistent bleeding, NO BOLUS

CM 1A: Heme/Onc/Endo (28 decks)

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