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Heme/Genetics > Clin Med - Clinical Approach to Bleeding Patient > Flashcards

Clin Med - Clinical Approach to Bleeding Patient Flashcards

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1
Q

What constitutes a referral to hematologist?

A

In a generally healthy patient:

  • unusual, spontaneous ,prolonged or delayed bleeding
  • abnormal coag test results obtained as part of preop eval

Ill patient w/ underlying medical issue

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2
Q

What is the significance of the age of onset in bleeding disorders?

A
  • early age onset correlates w/ severity and indicates it’s congenital
  • bleeding later in life may indicate an aquired problem or suggestive of milder congenital disease
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3
Q

Normal bruising in kids

A
  • bruising over forehead, knees and shins appears when children begin to crawl
  • small bruises over bony prominences common in preschool/school aged children
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4
Q

Abnormal bruising in kids

A
  • in non-mobile (<9 mo) infant, significant bruising is unusual
  • abnormal sites for bruising: back, buttocks, arms and shoulder
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5
Q

Good predictors of bleeding disorders

A
  • family members diagnosed
  • profuse bleeding w/ small wounds
  • profuse surgical related bleeding
  • muscle/joint- related bleeding
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6
Q

Fair predictors of bleeding disorder

A
  • bruising
  • epistaxis
  • menorrhagia
  • post-partum hemorrhage
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7
Q

Poor predictors of bleeding disorder

A
  • family member that bruises easily
  • gum bleeds
  • hematuria
  • BRBPR
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8
Q

What are important things to find out in a PMH about bleeding disorders?

A
  • response to trauma (surgery, injury, dental procedures)

- rule out renal disease, malabsorption, Ehlers-Danlos

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9
Q

Why are women w/ bleeding disorders more likely to be diagnosed?

A

menstruation, therefore women w/ bleeding disorders are also more likely to be symptomatic

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10
Q

Most gynecological conditions present with what?

A
  • bleeding, such as fibroids and polyps

- symptoms secondary to bleeding, such as endometriosis or ovarian cysts

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11
Q

menorrhagia

A

heavy menstrual bleeding that lasts for more than 7 days or results in the loss of more than 80mL of blood per menstrual cycle

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12
Q

How do you determine what constitutes blood loss more than 80mL in menstruation?

A
  • clots greater than 1 inch in size
  • low ferritin (Fe deficiency)
  • changing a pad/tampon more than hourly (flooding)
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13
Q

Prevalence of bleeding disorders in women w/ menorrhagia across all age groups

A

~ 20%

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14
Q

How is the prevalence of bleeding disorders in women w/ menorrhagia effected in other populations?

A
  • in adolescent pts w/ heavy periods since menarche: increases to 40%
  • in women w/ idiopathic menorrhagia: increases to 50%
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15
Q

hemostasis

A

responsible for maintaining fluidity of blood in the vessels and thrombus formation upon loss of vascular integrity

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16
Q

What 2 things are required for normal hemostasis?

A
  • normal number and function of platelets

- normal levels of clotting factors

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17
Q

If there are abnormalities in the hemostatic system what can be the result?

A

excessive bleeding or thrombosis

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18
Q

primary hemostasis

A
  • formation of primar platelet plug

- involves platelets, blood vessel wall and vWF

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19
Q

Mucous membrane bleeding (epistaxis, menorrhagia, gums) points to a problem w/ what?

A

-primary hemostasis (platelet disorder or VWD)

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20
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease

-deficiency or dysfunction of VWF

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21
Q

Functions of vWF

A
  • initiates platelet adhesion and mediates platelet aggregation
  • transports and stabilizes factor 8
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22
Q

secondary hemostasis

A

-formation of fibrin through the coagulation cascade

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23
Q

What is the central event in blood coagulation?

A

generation of the enzyme thrombin from its precursor prothrombin

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24
Q

What is suggestive of problems in secondary hemostasis?

A

bleeding into soft tissues or joints

-e.g: deficiency of a coag factor

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25
Q

coagulation cascade

A

review flow chart

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26
Q

What does PT measure?

A

factor 7

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27
Q

What does aPTT measure?

A
  • factor 8
  • factor 9
  • factor 11
  • factor 12, HMWK, prekallikrein
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28
Q

if there is an increase in PT but PTT is normal what is the likely cause?

A

-factor 7 is the only thing that could be going wrong

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29
Q

What factors are associated with both PT and aPTT?

A

-fibrinogen
-prothrombin
-factors 5 and 10
(common pathway)

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30
Q

What are the 2 options for causes of prolonged PT and PTT?

A

-a protein/factor is missing
OR
-there is interference/inhibitor in the sample

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31
Q

mixing study

A

distinguishes between factor deficiency and factor inhibitors

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32
Q

examples of factor inhibitors that could cause elevated PT/PTT

A
  • lupus anticoagulant

- specific factor inhibitor

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33
Q

If a mixing study corrects a prolonged PT/PTT, what was the causative source?

A

factory deficiency

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34
Q

If a mixing study does not correct a prolonged PT/aPTT, what was the causative source?

A

presence of inhibitor or lupus anticoagulant

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35
Q

Case:
elevated PT
normal platlets and aPTT

what is going on?

A
  • factor 7 deficiency
    possibilities:
  • Warfarin (milk vit.K deficiency)
  • mild liver disease (Factor 7 is rate limiting)
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36
Q

What are the vit. K dependent factors?

A
  • 2
  • 7
  • 9
  • 10
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37
Q

Effect of warfarin on labs

A
  • it is an anticoagulant

- works by interfering w/ vit. K so all vit. K dependent factors are effected

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38
Q

Case:

  • elevated aPTT
  • normal platelets and PT
  • pt is bleeding

what is going on?

A
  • factor 8, 9, 11 deficiency – hemophilia, VWD

- acquired inhibitor to above factors

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39
Q

Case:

  • elevated aPTT
  • normal platelets and PT
  • pt is not bleeding

what is going on?

A
  • factor 12, HMWK, prekallikrein deficiency

- presence of heparin or lupus anticoagulant

40
Q

Case:

  • elevated aPTT
  • elevated PT
  • normal platelets
A
  • supra therapeutic warfarin - severe vit. K dependent factor deficiency
  • severe liver disease
  • factors 5, 10 thrombin deficiency
41
Q

Case:

  • elevated aPTT
  • elevated PT
  • decreased platelets
A
  • DIC: check for fibrinogen and d-dimer

- severe liver disease (not common)

42
Q

Case:

  • normal aPTT
  • normal PT
  • normal platelets
  • bleeding pt
A
  • VWD
  • platelet function disorder
  • factor 13 deficiency (rare)
  • collagen disorder
43
Q

diagnostic approach to the bleeding patient?

A
  • CBC w/ peripheral smear
  • PT/INR and PTT - consider mixing study
  • fibrinogen
  • VWF testing
44
Q

What are the different tests used for VWD to distinguish b/w a deficiency or disorder?

A
  1. VWF:Ag - VW factor antigen
    - quantatative measure
  2. VWF:RCoF - VS ristocetin cofactor
    - functional measure
  3. Factor 8 level - factor 8 stabilizes VWF
45
Q

Most patients w/ VWD will have what results on PT and PTT?

A

normal

46
Q

hemophilia testing results

A
  • prolonged PTT (normal PT and fibrinogen)

- decreased factor levels (8, 9 and rarely 11)

47
Q

hemostatic system in neonates is profoundly affected by what?

A
  • gestational age

- postnatal age

48
Q

coagulation factors in neonates

A
  • most are decreased <50% adults levels
  • procoagulant: vit. K dependent factors 2, 7, 9, 10
  • anticoagulant: antithrombin 3, protein S and C
49
Q

During a routine health supervision visit of a 3 y/o, you note several 1-1.5cm purpuric lesions located over both tibias of a previously healthy boy. There are no purpura on any other areas and no petechiae. Most appropriate initial diagnostic approach?

A

no lab studies

50
Q

A 4 y/o is referred for eval of easy bruising. His mother has noticed multiple small hematomas over various areas, as well as large sized bruises over his torso, w/o any hx of injury. He often has nosebleeds that may last for 20-30 min. What labs would you send?

A

tier 1 testing:

  • CBC
  • PT
  • aPTT
  • fibrinogen
  • von Willebrand testing

*if aPTT is normal but VWF ag and function are both low, diagnosis is VWD

51
Q

A 6 y/o boy developed hematemesis after vomiting and undergoes upper GI scope. He subsequently developed a large gastric hematoma that had to be surgically resected. What labs would you send?

A
  • CBC
  • PT
  • aPTT
  • fibrinogen
  • VW testing
  • CBC, PT, VWF are normal. aPTT prolonged and corrects w/ mixing study. low factor 9. diagnosis?
  • mild hemophilia B
52
Q

Review lecture for more case studies

A

if you feel like punishing yourself

53
Q

What is the most severe inherited bleeding disorder?

A

hemophilia

-1/5000 males; all ethnic groups

54
Q

inheritance patterns of hemophilia

A
  • X-linked recessive: 1/4 may be new mutations
  • female child of affected male is obligate carrier
  • male child of female carrier has 50% chance of being affected
  • female child of female carrier has 50% chance of being carrier
55
Q

hemophilia A is a deficiency of what factor?

A

-factor 8

56
Q

hemophilia B is a deficiency of what factor?

A

-factor 9

57
Q

hemophilia C is a deficiency of what factor?

A

-factor 11 (rare)

58
Q

Although bleeding can occur at any site, the hallmark of hemophilia is what?

A
  • deep bleeding into joints and muscles

- chronic arthropathy is major morbidity in severe patients

59
Q

mild hemophilia

A
  • factor level 6-50%

- typically presents w/ bleeding provoked by surgery or trauma

60
Q

moderate hemophila

A
  • factor level 2-5%

- frequently experience bleeding after minor trauma and less commonly may develop spontaneous bleeding

61
Q

severe hemophilia

A
  • factor level <1%
  • often develop spontaneous musculoskeletal bleeding, life-threatening hemorrhage and excessive bleeding w/ minimal trauma
62
Q

joint bleeding characteristics in hemophilia

A
  • pain, tingling and warmth, followed by decreased ROM
  • usually starts at toddler age
  • ankle is particularly prone when the child is learning to stand - then knee
  • severe chronic arthropathy can develop later in life
63
Q

muscle bleed characteristics in hemophilia

A
  • intramuscular bleeding occurs deep in the body of the muscle
  • vague feeling of pain on motion, often difficult to palpate, circumference of limb is increased
  • iliopsoas bleeding can hold significant amount of blood
64
Q

life thratening hemorrhages in hemophilia

A
  • CNS - may occur w/o significant trauma
  • around airway
  • exsanguination
65
Q

If life threatening hemorrhage is expected in a hemophiliac, what should the tx be?

A

-aggressive tx w/ factor replacement BEFORE radiological eval

66
Q

von willebrand disease

A
  • inherited bleeding disorder caused by deficiency or dysfunction of von Willebrand factor
  • most common inherited bleeding disorder
  • effects men and women equally but women more likely to be diagnosed
67
Q

discovery of VWD

A
  • 1st described by dr. erik von willebrand in 1926

- called pseudohemophilia to describe sever mucocutaneous bleeding in a family

68
Q

genetic characteristics of VWD

A
  • autosomal dominant
  • each child has 50% chance of inheriting affected gene
  • variable penetrance
  • type 3 and type 2N exhibit autosomal recessive
69
Q

type 1 VWD

A
  • partial quantitative defect of VWF

- most common - 70-80%

70
Q

type 2 VWD

A

-qualitative VWF defect

71
Q

type 3 VWD

A
  • virtually complete deficiency of VWF

- autosomal recessive

72
Q

clinical presentation of VWD

A
  • excessive and prolonged mucocutaneous bleeding
  • bruising, epistaxis, gum bleeding
  • family hx of bleeding
  • abnormal VWF testing
73
Q

treatment fundamentals in mild hemophilia and most VWD

A

-factor replacement therapy may not be required for minor injuries

74
Q

tx fundamentals in moderate hemophilia

A

-factor replacement therapy w/ bleeding episodes or to prevent bleeding that could occur during a procedure

75
Q

tx fundamentals in severe hemophila

A

-factor replacement therapy is the mainstay of treatment

76
Q

treatment options in hemophilia

A
  • raising factor (8 or 9) or VWF levels with:
  • DDAVP
  • Blood component therapy - factor replacement
  • adjunctive therapies:
  • antifibrinolytic agents
  • hormome therapy for manorrhagia
77
Q

DDAVP

A
  • desmopressin
  • synthetic analogue of the antidiuretic hormone vasopressin
  • increases plasma factor 8 and VWF elvels
78
Q

candidates for treatment w/ DDAVP

A
  • pts w/ type 1 VWD, mild hemophilia A and some platelet disorders
  • indicated for menorrhagia, bleeding or minor procedures
79
Q

how DDAVP is given

A
  • IV or intranasal
  • peak effect achieved w/i 30-90 minutes
  • for menorrhagia, given on day 1 and 3 of period
80
Q

treatment w/ antifibrinolytics - tranexemic acid

A
  • stabilizes blood clot - helpful in almost any type of bleeding
  • can be used alone in mild cases or along w/ factor replacement in severe cases
  • another option is aminocaprioc acid
81
Q

hormone therapy for menorrhagia in bleeding disorders

A
  • combined hormonal contraception is superior to oral progesterone
  • continuous is preferable to 21 day schedule
  • higher doses often needed
  • Mirena IUD
82
Q

bleeding in newborns w/ hemophilia

A
  • 44% of pts w/ hemophilia had neonatal bleeding
  • sites: circumcision, intracranial, heel stick, IM inj sites
  • risk of symptomatic intracranial hemorrhage in newborns w/ hemophilia is 4%
83
Q

what is the major risk of delivery in infancy w/ suspected bleeding disorder?

A
  • intracranial hemorrhage

- generally recommend c-section

84
Q

in an otherwise healthy infant, the most common causes of bleeding are what?

A
  • thrombocytopenia
  • vit. K deficiency
  • inherited coag defects
85
Q

in a sick appearing baby, bleeding is often the result of what?

A
  • liver injury

- DIC

86
Q

neonatal thrombocytopenia

A
  • consumption of platelets: ITP, neonatal alloimmune thrombocytopenia, hepatic hemangioma
  • inadequate production: bone marrow suppression or infiltration
87
Q

NAIT

A
  • neonatal alloimmune thrombocytopenia

- placental transfer of maternal IgG antibodies against fetal platelet ags inherited from father

88
Q

presentation of NAIT

A
  • bruising, petechiae or rarely ICH - otherwise well appearing
  • normal maternal platelet count
89
Q

prenatal management of NAIT

A
  • planned c-section
  • avoid aspirin and NSAIDs
  • tx w/ maternal IVIG
  • maternal steroid
  • serial intrauterine platelet transfusions
90
Q

neonatal management of NAIT

A
  • can closely monitor if plat cout is >30k and no bleeding
  • if <30k:
  • random platelet transfusion
  • HPA-1a negative platelets
  • maternal platelets
  • IVIG
91
Q

inhibitory systems to terminate clotting

A
  • AT3 (antithrombin)
  • APC (activated protein C/S)
  • TFPI (tissue factor pathway inhibitor)
92
Q

AT3 function in clot termination

A
  • passive

- inactivates thrombin, factor 9 and 10

93
Q

APC function in clot termination

A
  • dynamic

- inactivates co-factors - 5 and 8

94
Q

TFPI function in clot termination

A
  • dynamic

- inhibits tissue factor

95
Q

how does heparin work

A

-by potentiating the activity of AT3

Heme/Genetics (15 decks)

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