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Hem/Onc > bleeding disorders > Flashcards

bleeding disorders Flashcards

1
Q

some initial diagnostic testing that should be included for bleeding disorders

A
  1. CBC
    - gives plt count
    - Hgb
  2. peripheral blood smear
  3. coagulation panel
    - INR/PT/aPTT
  4. CMP
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2
Q

coagulation panel includes:

A

PT
PTT
INR
(involves drawing blood, no fasting needed, no special instructions)

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3
Q

what is added to react with XIIa to start clotting through intrinsic/common pathway to evaluate PTT/aPTT

A

contact activator/phospholipid and Ca+

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4
Q

PTT/aPTT is very sensitive to ___

A

thrombin inhibition
(perfect for heparin (unfractionated))

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5
Q
  • A one-stage clotting test, screens for coagulation disorders
  • Standard part of a coagulation panel
A

partial thromboplastin time (PTT, aPTT)

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6
Q

PTT detects deficiencies of the ____ and may reveal defects in the ____

A

intrinsic, extrinsic

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7
Q

What is added to react with VIIa to initiate clotting through extrinsic/common pathway (for PT)?

A

thromboplastin (TF)

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8
Q

which factor has the shortest half-life

A

factor VII

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9
Q

what does prothrombin depend on?

A

vitamin K

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10
Q

what anticoagulant do you monitor with PT/intrinsic pathway

A

warfarin

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11
Q

if a pt has a high INR, what are they in a risk of? low INR?

A

bleeding
clotting

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12
Q

Disorders of primary hemostasis present with:

A
  1. mucous membrane bleeding
  2. epistaxis
  3. petechiae
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13
Q

disorders of primary hemostasis affect:

A
  1. bleeding time
  2. platelet count
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14
Q

Disorders of secondary hemostasis present with:

A
  1. hemarthrosis
  2. intracerebral hemorrhage
  3. deep tissue hematomas
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15
Q

disorders of secondary hemostasis affect:

A
  1. PT
  2. PTT
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16
Q

Mixed bleeding disorders can affect:

A
  1. bleeding time and platelet count
  2. PT and PTT
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17
Q

how can specific factor deficiency be determined?

A

Assessing the PT or aPTT in mixes of test plasma with commercially available plasmas deficient in known factors

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18
Q

which hemophilia is a congenital deficiency of coagulation factor VIII

A

hemophilia A

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19
Q

which hemophilia is a congenital deficiency of coagulation factor IX

A

hemophilia B

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20
Q

signs of hemophilia

A

recurrent hemarthroses and easy bruising/bleeding
(knees MC)

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21
Q

which hemophilia is MC X-linked genetic disease

A

hemophilia A

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22
Q

which hemophilia is X-linked recessive, leading to affected males and carrier females

A

hemophilia B

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23
Q

female carriers of hemophilia are usually

A

asx

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24
Q

Severe hemophilia usually presents in who? with ____ (s/s)

A
  1. infant males or in early childhood
  2. spontaneous bleeding into joints, soft tissues
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25
Q

mild hemophilia typically present with _______ than usually after a significant hemostatic challenge (surgery, trauma)

A

more bleeding
(spontaneous bleeding is rare)

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26
Q

what is the “double whammy”

A

Patients with hemophilia with deficiency in a clotting factor PLUS later develop inhibitors to those factors

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27
Q

the “double whammy” is more common in which hemophilia

A

A

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28
Q

how is the “double whammy” characterized?

A
  1. bleeding episodes that are resistant to treatment with clotting factor VIII or IX concentrate
  2. new or atypical bleeding
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29
Q

what are the labs for hemophilia?

A
  1. low factor VIII or factor IX activity level
  2. aPTT prolonged
  3. PT/INR normal
  4. CBC normal
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30
Q

normal hemostasis requires at least ___ (%) of factor VIII activity

A

25%
mild = 5<x<40%
moderate = 1-5%
severe =<1%

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31
Q

tx of minor bleeding of mild hemophilia A

A

DDAVP
(also for major + factor VIII)

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32
Q

tx of minor/major bleeding of moderate/severe hemophilia A

A

factor VIII

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33
Q

tx of minor/major bleeding of mild-severe hemophilia B

A

factor IX

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34
Q

mainstay tx for hemophilia?

A
  • Plasma-derived or recombinant factor concentrates (mainstay)
  • Celecoxib - arthritis symptoms
  • opioid analgesics - pain control
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35
Q

severe hemophilia require what tx?

A

infusions of factor concentrate up to 3 times a week to prevent recurrent joint bleeding

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36
Q

what should be avoided when taking celecoxib?

A

other NSAIDs and aspirin
- increases risk of bleeding from inhibition of platelet function

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37
Q

what is the most common cause of death of hemophilia pts

A
  • transfusion-obtained HIV/AIDS
  • hepatitis/cirrhosis
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38
Q

MC life-threatening complications of hemophilia

A

intracranial hemorrhage and hemorrhages

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39
Q

what is the SECOND MCC of death in hemophilia pt

A

intracranial hemorrhage

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40
Q

what pt education must you give for hemophilia pts

A
  1. avoid contact sports
  2. monitor for s/s of bleeding
  3. home infusion technique
    - prophylactic factor transfusions
    - early tx of any bleeding
41
Q

what is the most common inherited bleeding disorder

A

Von Willebrand Disease

42
Q

a large multimeric glycoprotein that binds to its receptor, platelet glycoprotein Ib, bridging platelets together and tethering them to the site of vascular injury.

A

vWF

43
Q

what is the most common type of vWD

A

type 1

44
Q

which vWD type is a quantitative defect, with not enough vWF

A

type 1

45
Q

which vWD is only autosomal recessive with a total or near total absence of vWF

A

type 3
most severe

46
Q

which vWD is qualitative defect, with dysfunctional vWF

A

type 2

47
Q

most common signs of vWD

A

nosebleeds
hematomas
prolonged bleeding from trivial wounds

48
Q

women with vWD are 5x more likely to have ?

A

menorrhagia

49
Q

lab findings of vWD

A
  1. “prolonged bleeding time”
    - usually normal in vWD type I
    - may be present but is not specific for vWD!!
  2. slightly prolonged aPTT in half of pts bc of low levels of FVIII
    - may be prolonged with severe form of vWD
  3. Normal PT
    (labs should be repeated to confirm abnormal results)
50
Q

3 tx for vWD

A
  1. DDAVP
  2. rVWF
  3. vWF/FVIII
51
Q

what are the congenital coagulation disorders (coagulopathies) (3)

A
  1. hemophilia
  2. vWD
  3. FXI deficiency
52
Q

autosomal recessive
Jewish descent
mild bleeding

A

FXI def

53
Q

tx for FXI def.

A
  1. FXI concentrate (if available)
  2. FFP (if no FXI concentrate)
54
Q

when you make too few PLTs = inability to make a primary plug or secondary clot

A

thrombocytopenia

55
Q

causes of thrombocytopenia

A
  1. increased destruction
  2. decreased production
    - bone marrow failure
    - chemo
    - nutrition
56
Q

common signs of thrombocytopenia

A

MALT bleeding
epistaxis, gum bleeding, GI bleeds (rare)
cerebral hemorrhage
petechial rash (non-blanchable)

57
Q

cirrhosis can commonly cause?

A

hypersplenism = increased destruction of PLTs = thrombocytopenia

58
Q

type of thrombocytopenia where PLT are being consumed by a patient (not a lack of production)

A

destructive or consumption

59
Q

5 CC of destructive/consumption thrombocytopenia

A
  1. splenomegaly/hypersplenism - liver disease, lymphomas
  2. antibody-mediated destruction (ITP)
  3. drug-related (HIT)
  4. massive bleeding associated with consumption
  5. diffuse thrombus formation
60
Q

patients form auto-antibodies against antigens on PLT surface

A

immune thrombocytopenic purpura (ITP)

61
Q

ITP is usually seen in ?

A

children

62
Q

if a healthy child presents with a sudden petechial rash, with bruising/bleeding, revealing signs of cutaneous bleeding, what are you suspicious of?

A

ITP
cutaneous bleeding = petechiae, purpura, ecchymoses

63
Q

what is the diagonsis of ITP

A
  1. isolated thrombocytopenia (<100k)
    AND
  2. no clinical apparent associated conditions that may cause thrombocytopenia
64
Q

MC management for ITP

A

watchful waiting - most children require no specific tx
some require pharmacologic intervention (rare)
avoid antiplatelet and anticoagulants until thrombocytopenia is resolved

65
Q

when would someone need pharmacological intervention for ITP (3)

A
  1. severe thrombocytopenia (<10k), with signs of substantial cutaneous bleeding
  2. moderate thrombocytopenia (<20k), with mucosal bleeding
  3. past or anticipated factors that increase bleeding risk (recent head trauma)
66
Q

if tx is necessary for ITP, what do you give?

A
  1. corticosteroids (FIRST LINE)
    - oral prednisone, IV methylprednisolone, IV/oral dexamethasone
    - (IVIG can be started first)
  2. PLT transfusion for serious bleeding
  3. splenectomy sometimes
67
Q

mechanism of drug-related PLT destruction is ?

A

immune-mediated mostly

68
Q

typical presentation of drug-related destruction

A

thrombocytopenia
mucocutaneous bleeding 7-14d after exposure to a new drug

69
Q

what kind of medication is well-known to cause drug-related destruction

A

ABX

70
Q

tx for drug-related destruction

A

stop the drug
rare - immunosuppression (corticosteroids)

71
Q

name of massive bleeding that can consume PLTs at rates beyond which they can be produced by bone marrow
can lead to DIC-like states

A

bleeding-associated consumption

72
Q

tx for bleeding-associated consumption

A

blood product infusion
interventions to surgically stop bleeds

73
Q

hypoproliferative thrombocytopenia MC arises from ?

A

bone marrow failure

74
Q

4 hypoproliferative disorders

A
  1. leukemia/lymphoma/myelodysplasia/aplasia
  2. cancers metastatic to bone marrow
  3. severe viral infection
  4. radiation or chemo
75
Q

tx for hypoproliferative thrombocytopenia

A
  1. episodic - resolves once over (viral)
  2. treat underlying cancer
  3. stem cell transplant (esp aplasia)
    - bone marrow loses regenerative capacity
76
Q

which class of PLT disorder has normal PLT count, but dysfunctional PLT
iatrogenic or acquired is common

A

qualitative

77
Q

manifestations of qualitative PLT disorders

A

MALT bleeding

78
Q

tx for most qualitative PLT disorders

A

transfusion of normal donor platelets

79
Q

what drugs are the MCC of acquired PLT dysfunction

A
  1. aspirin
  2. clopidogrel
  3. NSAIDs
80
Q

___ and ___ are irreversible inhibitors of PLT function, ___ induce reversible PLT dysfunction

A

Aspirin, clopidogrel - PLT have no function until CL (5-7d)
NSAIDs - PLT function returns 12-24h

81
Q

2 thrombotic microangiopathies (TMAs)

A

thrombotic thrombocytopenic purpura (TTP)
hemolytic-uremic syndrome (HUS)

82
Q

Pts with larger multimers of vWF, lack plasma protease in their plasma, and has antibodies against ADAMTS13 most likely have ?

A

thrombotic thrombocytopenic purpura (TTP)
common in adults

83
Q

clinical presentations of TTP (3)

A
  1. neurologic dysfunction
  2. petechiae
  3. dark urine
84
Q

TTP diagnostic lab evaluation

A
  1. WBC count - normal or higher
  2. 8-9 g/dL Hgb (decreased)
  3. schistocytosis
  4. PT and aPTT - normal
  5. D-dimer - normal or elevated
  6. fibrinogen - high
  7. direct coombs test - negative
85
Q

tx for TTP

A
  1. FFP
  2. rituximab, corticosteroids, IVIG, vincristine, cyclophosphamide, and splenectomy
    NO PLTS
86
Q

MCC of acute renal failure in children

A

hemolytic uremic syndrome (HUS)

87
Q

clinical syndrome characterized by progressive renal failure associated with microangiopathic hemolytic anemia and thrombocytopenia

A

hemolytic uremic syndrome (HUS)

88
Q

MC clinical feature of hemolytic uremic syndrome

A

prodromal gastroenteritis - prodrome of fever, bloody diarrhea (2-7d) before onset of renal failure

89
Q

MC of typical HUS? second?

A

E. coli serotype
shiga-like toxin [Stx-HUS]

90
Q

Atypical HUS can be ___

A

sporadic or familial

91
Q

lab findings of HUS

A
  1. BMP - elevated BUN/CR
  2. CBC - severe anemia
  3. schistocytes
  4. Bilirubin and LDH - elevated
    obtain stool sample for E. coli and shigella
92
Q

tx for HUS

A

typical - supportive
atypical - plasma exchange

93
Q

essentials of DIC diagnosis

A
  1. frequent cause of thrombocytopenia in hospitalized patients
  2. prolonged aPTT and PT
  3. Thrombocytopenia and decreased fibrinogen levels
94
Q

An uncontrolled local or systemic activation of coagulation, which leads to thrombocytopenia and depletion of coagulation factors and fibrinogen

A

disseminated intravascular coagulopathy (DIC)

95
Q

etiology/causes of DIC

A

S = Sepsis
T = Trauma
O = Obstetric complications (ex: Preeclampsia)
P = Pancreatitis (Acute)
M = Malignancy (esp. Blood and brain cancers)
N = Nephrotic syndrome
T = Transfusion (hemolytic rxn)

96
Q

clinical features of DIC

A
  1. Bleeding at multiple sites - intravenous catheters or incisions (purpura fulminans)
  2. purpura and petechiae
97
Q

lab findings of DIC

A
  1. Prolongation aPTT and PT
  2. Low levels of fibrinogen
  3. D-dimer levels as well as other FDPs are elevated
    - bc of activation of coagulation and diffuse cross-linking of fibrin.
  4. Schistocytes
98
Q

If persistent bleeding of DIC, consider use of ___; do not administer bolus.

A

heparin

99
Q

If persistent bleeding of DIC, consider use of ___; do not administer bolus.

A

heparin

Hem/Onc (13 decks)

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