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Rosens > 111 Disorders Of Hemostasis > Flashcards

111 Disorders Of Hemostasis Flashcards

1
Q

Vascular endothelium regulates? Formation.

A

Clot

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2
Q

What things does the vascular endothelium secrete to regulate clot formation – list 4

A

Tissue factor pathway inhibitor
Heparin sulfate.
Prostacyclin
Nature oxide.
CD 39
Bon Willebrand factor

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3
Q

What is the role of platelets?

A

One. Adhesion to sub endothelial connective tissue.
Two. Release of adenine diphosphate which amplifies aggregation
Three. Platelet aggregation over area of injury.
Four. Stabilization of haemostatic plug interaction with the coagulation system five. Stimulation of limiting reactions of platelet activity.

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4
Q

What is the intrinsic pathway of the coagulation cascade i.e.? What factors does it include?

A

12, 11, nine, eight

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5
Q

What lab value indicates the intrinsic pathway of the coagulation cascade?

A

APTT

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6
Q

What is the extrinsic pathway of the coagulation cascade and what lab value indicates this?

A

Seven and tissue factor
PT

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7
Q

What is included in the common pathway of the coagulation cascade?

A

Factor 10, prothrombin, which is two, thrombin, fibrinogen, which is one, firing clot, which is 13

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8
Q

Which three things in the coagulation cascade are required to turn the common pathway factor 10 into thrombin

A

Factor five
Calcium
Lipids

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9
Q

How did differentiate the Purpera associated with platelet disorders versus vasculitis?

A

Platelet: typically asymptomatic, not palpable.
Associated with vasculitis: burn/itch and is palpable

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10
Q

Name five features of coagulation disorders that differentiate from platelet disorders

A

One. Bleeding sources often intramuscular or deep soft tissue haematoma from small arterials.
Two congenital form of disease occurs predominantly in men
Three bleeding after surgery or trauma but maybe delayed in onset up to 72 hours
Four. Epistaxis, menorahaggia, G.I. sources of bleeding are rare, whereas haematuria and haemarthrosis are common and severe cases.
Five. Bleeding time is normal accepting patients with von Willebrand disease.

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11
Q

What do I need to include in my clinical evaluation of a bleeding patient for a history?

A

Nature of the bleeding.
Sites of bleeding.
Challenges to haemostasis from prior experience and current.
Medication’s
Associated diseases, such as urea, liver, disease, infection, malignant neoplasm.
Transfusion history.
Family history

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12
Q

What is my physical exam for the bleeding patient?

A

Vital signs
Skin
Mosa
Lymphadenopathy
Abdomen
Joints.
Other sites of blood loss, including pelvic, rectal, urinary tract, intramuscular, or deep soft tissue

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13
Q

What are the main mechanisms of platelet disorders or low platelets?

A

One. Decrease production.
Two. Increased destruction.
Three. Dysfunction.
Four. Sequestration.

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14
Q

In low platelet disorders, what kind of things can cause decreased production

A

Drugs.
Toxins
Infection.
Hereditary concerns.
Can also be splenic sequestration

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15
Q

In disorders with low platelets, what kind of increased destruction categories and diseases might be seen?

A

One. Immunologic – vascular disease, lymphoma, leukemia, drug related, infection, post, transfusion, immune thrombopenia.

Mechanical: DC, TTP, HELLP

Vasculitis

Dilutional

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16
Q

What kind of platelet disfunction does Bon Willebrand’s disease have?

A

Adhesion defects

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17
Q

What different categories of disease can cause elevated platelet

A

Primary
Reactive.
Iron deficiency.
Infection or inflammation
Trauma.
Non-hematologic malignant disease.
Rebound from alcohol, sot, toxic drug therapy, folate, or B12 deficiency

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18
Q

Name 4 collagen vascular disorders that are inherited

A

Ehlers danlos
Osteogenesis imperfecta
Pseudoxanthoma elasticum
Hemorrhagic telangeftasia

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19
Q

Name six different vascular disorders that are acquired

A

Scurvy
Simple or senile Purpera
Purpera secondary to steroid abuse.
Vascular damage: infection, like meningitis, hemolytic, uremic syndrome, hypoxia

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20
Q

Name five different inherited coagulation disorders

A

Von Willebrand disease
Hemophilia/factor eight or haemophilia B/factor nine
Factor 13, factor 11, factor five, factor 10, factor seven or factor two
Afibroginemia or hypo

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21
Q

Name six different acquired coagulation disorders

A

Medication
Snakebite, venom induced consumptive coagulopathy
Liver disease,
DIC
Auto immune
Acquired factor inhibitors from treatment of congenital disease like haemophilia or malignancy

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22
Q

What are haemostasis tests you should order when concerned about a coagulopathy disorder

A

CBC and smear
Platelet count particular
Bleeding time.
PT
PTT
Friden factor levels and inhibited screens possible
As needed: electrolyte levels, glucose, BUN, creatinine and type in cross match

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23
Q

What does an INR test?

A

Extrinsic and common pathways: factor one, two, five, seven, 10

Deficiencies in fibrinogen, Promod, five, seven, 10

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24
Q

If an INR is isolated elevation, which factor deficiency should be suspected

A

Seven

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25
Q

What does a PTT test?

A

Intrinsic and common pathways: one, two, five, eight, nine, 10, 11, 12

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26
Q

If PTT is isolated and elevation, consider what deficiencies

A

12,Prekallikrien, high molecular weight kinogen
Eight, nine, 11

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27
Q

When is an anti-factor 10 a essay useful

A

Monitoring, unfractionated, heparin and low molecular weight heparin or for drug quantification of direct factor 10 a inhibitors, including rivaroxaban, apixaban

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28
Q

What does a fibrinogen level indicate?

A

Balance between production and consumption

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29
Q

Three causes of a decreased fibrinogen level

A

Severe liver disease.
Low production.
Over consumption, such as a DIC

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30
Q

When is a thrombin time helpful?

A

Analyzes the conversion of soluble fibrinogen into insoluble fiber, and is helpful for abnormalities of fibrinogen and inhibitors, dabigatran

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31
Q

What platelet level should patients be given a platelet transfusion as they are at risk, spontaneous bleeding regardless of any other cause?

A

Less than 10

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32
Q

What platelet level should people be given platelets prior to central line insertion

A

Less than 20

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33
Q

Patients receiving a lumbar puncture, should have a platelet transfusion below what level of platelets

A

50

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34
Q

For patients receiving neurosurgery or ophthalmology intervention, below what platelets should they receive a donation of platelets?

A

100

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35
Q

Name six drugs that can cause low platelets

A

Heparin
Quinine
Quinidine
Digoxin
Cell phon amides
Fatau
ASA
GP 2B3A inhibitors
Cocaine

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36
Q

What is heparin induced thrombocytopenia?

A

Antibody against PF4/heparin complex that causes a greater than 50% drop in platelets after 5 to 10 days of heparin

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37
Q

How to treat heparin induced thrombopenia?

A

Stop heparin
Steroids.
Fondaparinux or argatroban
Avoid warfarin until platelets, recover due to risk of thrombosis and skin necrosis

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38
Q

What is idiopathic thrombocytopenia pathophysiology?

A

IGG anti-platelet antibody, diagnosis of exclusion

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39
Q

For adult patients, how to treat idiopathic thrombocytopenia? During a life-threatening bleed.

A

Methyl prednisone 15 mg per kilogram, IV 0.7 mg per kilogram, platelet transfusion, call haematology

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40
Q

Thrombotic thrombocytopenic Purpera: what is this?

A

Antibodies against a DAMTS 13 and can be triggered by meds, including, Plavix, quinine, cyclosporine

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41
Q

How to treat thrombotic thrombopenia Purpera

A

Plasma paresis and rituximab
Prednisone 1 mg per kilogram
Consult heme
Don’t give plt unless they’re going to immediately die

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42
Q

How is the shiva toxin of E. coli 0157: H7 similar to TTP?

A

Shiva toxin inactivation of DAMTS 13, like TTP, but get more renal symptoms

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43
Q

Treatment of HUS

A

Fluids, supportive.
Dialysis.
No antibiotics and no ant motility drugs.
No plasma free ASIS and kids

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44
Q

What does Bon Willebrand factor lead to deficiencies in?

A

Factor eight
Abnormal platelet adhesiveness.
Abnormal vascular endothelium

45
Q

Villa brand disease: platelets, PT, factor, INR, APTT, von Willebrand factor findings

A

Normal platelets, prolonged bleeding time, decrease factor eight, INR normal, APTT increased, von Willebrand factor decreased

46
Q

How to generally treat von Wilburn factor disease

A

DDAVP 0.3 µg per kilogram for milder moderate
Humate p
Factor eight concentrated 50 units per KG for severe bleeding, FFP, TXA

47
Q

What is the severe form of primary immune thrombocytopenia?

A

Acquired auto immune that has no parent trigger or associated condition.
Severe is less than 20

48
Q

In children idiopathic immune thrombocytopenia is usually seen with what kind of prodrome

A

Viral

49
Q

What secondary infections/diseases can cause immune thrombocytopenia

A

SLE, RA
Leukemia/lymphoma.
Post infection ITP with HIV, HRC, EBV, rubella, Veriella
Drug induced

50
Q

How to treat newly diagnosed adult patients with autoimmune thrombocytopenia

A

Supportive.
Less than 30 asymptomatic or minor containers bleeding consider corticosteroids decks 40 mg PO/IV times four days, admission if platelets are less than 20, platelets are less than 10 consider IV in addition to steroids

51
Q

How to manage peds patients with ITP?

A

Even with minor bleeding, typically no meds even if platelets are less than 20
Considered admission if uncertain, diagnosis or lack of close follow up.
New diagnosis: consider prednisone IV Auntie D immunoglobulin, but likely to be discussed with haematology

52
Q

What is the 4T scoring system for heparin induced thrombocytopenia?

A

0 to 2 points considering thrombocytopenia, timing of platelet count, thrombosis sequela, other causes of thrombus piña appear or otherwise.

Less than three points is low probability of hit, 4 to 5 intermediate, 6 to 8 high

53
Q

If 4T score for HIT is intermediate or high what to do?

A

Stop heparin, order immuno acid.
Manage thrombotic complications if necessary - consider Fonda or DOAC

54
Q

Post transfusion Purpera what is this and when to treat

A

Risk factors include middle-aged women in previous pregnancy who had a transfusion recently within a week, platelets fall to less than 10

Treated with IV 1 g per kilogram IV plus or minus steroids

55
Q

What are the two most common thrombotic microangiopathy?

A

TTP
HUS

56
Q

Terrible triad of TTP

A

MAHA (shistocytes, high LDH)
Thrombosis, piña
End organ damage

57
Q

Presentation of TTP symptoms: FATCRN

A

Fever
Anemia with haemolytic and shistocytes
Thrombus piña
Cardiac ischemia.
Renal manifestations, either protein or heme in urine (rarely renal failure)
Neuro symptoms

58
Q

Treatment of TTP

A

Plasma exchange
Glucocorticoids, prednisone versus methyl prednisone.
If waiting for plasma exchange can give FFP.
If a factory rituximab

Avoid platelets as you can potentially just make them more thrombotic

59
Q

What tests will help you to determine whether it’s TTP or DIC

A

IN ttp: PT, fibrinogen, D dimer are all normal wallabies are increased in DIC

60
Q

What is DIC?

A

Disseminated intravascular coagulation affects the common acquired coagulopathy reflecting dysregulation of coagulation, and fibrinolytic pathways: a disorder of throbbing i.e. to a with excess generation of thrombosis, which leads to activation of factors five, eight, 13, 11

Fibrinogen to make a clot

Stimulating fibrinolysis

Stimulating platelets

61
Q

Labs in DIC:

A

Increased INR.
Decreased platelets.
Increased D dimer.
Decreased fibrinogen
Increased APTT

62
Q

How to treat DIC?

A

Platelet goal greater than 50
FFP goal INR 2 to 3, APTT less than 1.5 times normal
Vitamin K 10 mg IV.
Cryoprecipitate 10 units as contains fibrin
If macro clotting give IB heparin free PET

63
Q

DIC:
Support coag magic 5 to consider

A

Fibrinogen greater than 1.5 with cryoprecipitate
Platelets greater than 50 - ply
Hematocrit, greater than 21% - blood
APTT less than 1.5 times control i.e. FFP.
INR less than 2 to 3 related to factor seven

64
Q

What does HUS present with?

A

Abdominal pain, emesis, diarrhea, usually perceive the development of HUS by 5 to 10 days

65
Q

HUS triad

A

Haemolytic anemia
Thrombopenia,
A.k.a.

66
Q

What are three acquired causes of HUS

A

Shiga toxin ecoli
HIV
Strep pneumonia

67
Q

Patient who might be initially thought to have ITP might actually have what hereditary thrombocytopenia disorders?

A

Adhesion defects Bernard soulier syndrome

Aggregation defect: glanzmann thrombasthenia

68
Q

What is haemophilia a?

A

Deficiency in factor eight, X linked recessive disorder which requires replacement theory P and can lead to increase risk of aloe antibodies or inhibitors

69
Q

Management of haemophilia a: chronic

A

Plasma derived factor eight.
Emicuximab - recant immunoglobulin that is used for prophylactic treatment

Needs to see a haematologist have a factor eight activity level, blood type, anti-hemophilic factor antibody status. I need to know their last day of hospitalization.

70
Q

List eight reasons for factor replacement and haemophilia

A

Suspected bleeding into a joint or muscle.
Any significant injury to the head, neck, mouth or eyes.
Any newer, unusual headache, particularly falling trauma.
Severe pain or swelling at any location
All wounds that require surgical closure or wound did he?
History of trauma.
Prior to any invasive procedure surgery
Suspicion of uncontrolled G.I. bleed, or any symptoms of hypovolemia
Acute fracture, dislocation, or sprain
Suspicion of uncontrolled heavy menstrual bleeding, leading to anaemia or symptoms of hypovolemia.

71
Q

When can you give DDAVP and haemophilia a?

A

Mild hemophilia a without inhibitors that are bleeding, but that is not life or limb, threatening, which increases factor 8 3 to 6 times their baseline level

72
Q

What factor eight level to give in haemophilia a

A

One unit per KG factor eight will increase circulating factor eight level by 2%

Therefore, if the factor level is known dose equals desired times 8×0.5×8 KG.
An emergency situation assume level is 0%
Therefore goals for minor bleeding or trauma should be 40 to 50% and 80 to 100% for serious or like threatening bleed

73
Q

In the emergency department, what recombinant factor eight a dose should be given? As compared to those not currently on.Emicuxumab?

A

90 µg per kilogram IV

Also consider giving p complex concentrate at a dose of 75 to 100 units per KGIV

74
Q

What is haemophiliaB?

A

Factor nine deficiency, ex linked, similar clinical findings, and Homophilia a

75
Q

How to treat haemophilia B?

A

Recombinant factor nine preparation or plasma drives concentration and emergency situations. Do not give FFP do not give cryer precipitated as it does not contain nine.

In severe bleeding, give 100 to 140 units per KGIV, which should result in a factor level of 80 to 100%

If they have inhibitors, they need haematology in the hospital: administer at 90 µg per kilogram IV and if not on prophylactic med (emicizumab) get PCC at 75 to 100 units per KGIV

76
Q

What is warfarin?

A

Vitamin K antagonist

77
Q

What are common DOACS?

A

Rivaroxaban
Apixaban

Xa Inhib

78
Q

Dabigatran type of med

A

Direct thrombin inhibitor

79
Q

Medication for reversal heparin

A

Stop the drug
Protamine sulphate 50 mg over 10 minutes - 1 mg of protamine for every hundred units of heparin (fractionated)
0.5 to 1 mg for every 1 mg of low molecular weight heparin: 1 mg protamine for every 1 mg low molecular weight heparin less than eight hours, 0.5 mg pram for every 1 mg low molecular weight heparin greater than eight hours

80
Q

Reversal of dabigatran

A

Idarucizumab 5g iv

If unavailable considered PCC at 50 units per KGIV or 4FPCC
Considered dialysis

81
Q

Reversal apixaban or river

A

Adnexet alpha

Or octoplex at dose of 2000 units IV 25 to 50 units per KG

82
Q

When is a MHP reasonable

A

replacem entire bl vol or 10 units within 24h

transfusion >4 prbc within 1 our w/ ongoing losses

ttransfusion 6 prbc within 1 bleeding episode with ecpectation for ongoing losses

shock index..

83
Q

Major hemorhage management

A

address source

early resus: map 50-60
reverse anticoag

+/- txa

84
Q

MHP - how many plt

A

1 pool = 4 donors

85
Q

MHP at HSC:
first, second receiving of fluids

A

first - 4 rbc
second only ffp

86
Q

MTP rosen’s goals

A

hbg >70
inr <1.5
fibrinogen >1
plt >80

87
Q

Complications MTP

A

coag
thrombocytopenia
hypothermia acidosis
hypomag
high citrate
hypoca

88
Q

Wafarin: when to give vitamin K and octaplex vs supratherapeutic INR

A

octaplex if major, life bleed or active bleed and INR >10 and on warfarin

INR >5 just v and no bleed

89
Q

Heparin: UFH vs LMWH reveral dose of protamine sulfate

A

1mg of protamine q100 units of UFH

0m5-1mg LMWH

90
Q

Dabigatran: dose of reversal?

A

idarucizuab 5g IV
aka praxbind

or octaplex 50u/kg IV or dialysis as 57% drug reasonable in 4h

91
Q

DOACS - reversal?

A

andexanet alfa or 4F PCC

92
Q

d-dimer in DIC levels

A

15000-20000

93
Q

Clinical features of HIT

A

thrombocytopenia
bleeding
thrombosis
skin necrosis
gangrene
organ ischemia/infarction

94
Q

HUS vs TTP - moreso ?

A

HUS -renal vs TTP - cardiac, less renal

95
Q

MC bleeding disorder

A

vwf

96
Q

vwf 2 major functions

A

stabilizes half life of factor 8

promotes plt adhesion at site if issue

97
Q

Type 1 vWD:

A

quantitative defect mc

98
Q

Type 2 vWD:

A

quali defect
2A, B, M, N

99
Q

Type 3 vWD:

A

severe defect pretty much none

100
Q

vWD: nonsp tx

A

control bleeding sx (wo = IUD)
iron suppl
txa
topical hemostasis

101
Q

vWD: if ongoing bleed despite conservative management

A

DDAVP 0.3mcg/kg specifically helpful for type I

IIb - bad to give!! get humate

102
Q

Hemophilia: what percent are new mutations?

A

25-30% new mutations

103
Q

Hemophilia: severe vs mod vs mild

A

sev <1u/dl, spont bleed

1-5

> 5

104
Q

Hemophilia: prophylaxis

A

severe disease - coag factor or emicuzumab

105
Q

Hemophilia: treat with hem A life thr bleed

A

50u/kg of factor 8 –> 100%

106
Q

Hemophilia: treat with hem B life thr bleed

A

100 units/kg –> ~100%

107
Q

Acquired hem A - what

A

plausible in pt >60
inhib against F8 to bypass - med per heme

108
Q

Emergency managemnt of bl disorder

A

tx first, investifate later
ask for card
heme on call
txa 25mg/kg PO q6h

50 x weight = IU

Rosens (48 decks)

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