Bleeding Disorder Flashcards by Zahra Kalifa

Define hemostasis:

Balance between (procoagulant: blood in fluid) - (anticoagulant)

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diseases associated w\ Increased procoagulant factors vs increased in anticoagulant proteins

procoagulant: bleeding disorder

- anticoagulant: thrombotic disorder

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Hemostasis is an interplay between

vascular endothelium
platelets
coagulation factors

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Name the 4 phases for hemostasis:

1- vascular phase
2- platelet phase
3- coagulation factors
4- fibrinolytic system

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Vwf is released in which phase of hemostasis?

In the vascular phase

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Describe the vascular phase of hemostasis:

tissue injury
Vasoconstriction
CT exposure (sub-endothelium)
VwF release

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What is primary and secondary hemostasis & what’s the end result?

Primary: platelet phase (platelet plug)
Secondary: coagulation factors (fibrin thrombus(

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Describe the platelet phase of hemostasis:

adhesion: attach to endothelium
activation: activated & release granules (ADP, thromboxane A2, cofactor)
aggregation: w\VwF + w\platelets

[platelet plug]

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Describe the coagulation phase of hemostasis:

initiation: making thrombin (extrinsic)
amplification: further thrombin (intrinsic)
propagation: (common)

[fibrin thrombus]

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What are the factors for the intrinsic & extrinsic pathway?

intrinsic: 12, 11, 9, 8
extrinsic: 3,7
common: 5

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What factor is responsible for stabilization of clot?

Factor 13

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What is the role of protein C and protein S in hemostasis?

Inactivate factor 5 to inhibit thrombin formation

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What is the role of antithrombin, heparin, A2 macroglobulin in hemostasis?

Neutralize the activity of thrombin

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What is the role of TPA in hemostasis?

Converts plasminogen to plasmin to dissolve fibrin clot

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Describe the extrinsic pathway

F7 > TF > F10 > F5

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Describe the intrinsic pathway

F12 > F11 > F9 > to F10 by F8

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Factors to consider when evaluating a bleeding disorder:

Gender
Age at 1st bleed
Site
Frequency
Severity

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When is bleeding in a child considered normal?

If mild and over bony prominance like shaft (1-10yrs)

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Usually the etiology of mucocutanous bleeding vs internal bleeding is caused by:

mucocutanous: primary

- internal: secondary

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Delayed separation of umbilical stump is associated with which coagulation factor deficiency

Factor 13 deficiency

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What are the history taking clues in the neonatal state that may indicate bleeding disorder

umbilical stump, veinipuncture site, cephalohematoma, ICH

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What are the history taking clues in the vaccination and surgical procedure that may indicate bleeding disorder

Circumcision, hamatoma, prolonged oozing

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It’s important to inquire about family history in bleeding disorder, such as:

bleeding in PPP in female relatives

- diagnosed bleeding disorder

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What medications might be associated with bleeding in child

NSAID, warfarin, herbal (ginger, ginko, garlic)

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Name the 3 bleeding disorder mimickers:

- ehlers danlos - hemangioma - hemorrhagic talengectasia - child abusse

What are the symptoms of ehlers danlos syndrome

- ecchymosis | - hyperextensible joints

What are the initial testing for bleeding child

- CBC & film: HUS\TTP\leukemia - PT: F7 - aPTT: F8, F9, F11 - both: F10, F5, F2, F1 - Fibrinogen: DIC & sepsis

In case of heparin contamination, what test is best to identify the problem?

Reptalise time

What is the mode of inheretance for hemophilia A, B, and C?

A & B: X-linked | C: autosomal recessive

In which group of population do we commonly see Hemophilia C?

Ashkenezi jews

Hemophilia A is deficient in factor

Hemophilia B is deficient in factor

When can females be affected by hemophilia A & B?

- symptomatic carrier - lyonization - homozygous recessive - turner syndrome

Investigations for hemophilia:

- aPTT: prolonged - factors: low “8 or 9 or11” - genetic & CVS

What determines severity in hemophilia A & B patients?

Concentrations of factors

Differentiate between mild, moderate and severe hemophilia

Activity of factors, frequncy, spontanous vs major trauma, and pattern - pattern: joint +\- bleed after circumcision +\- ICH [moderate] In severe: all of them w\out +\- In mild: only till circumcision bleed

What are the complications of hemophila A or B

- arthropathy - viral transmission - inhibitors

What is the aim of therapy in hemophilia treatment

Improve QOL, treat on demand for mild, treat prophylactically by factors for moderate to severe.

What is the treatment for hemophilia patients

- factor replacement - desmopressin - antifibrinolytic

Name antifibrinolytics:

Tranxemic acid | Aminocarbonic acid

What is the desired plasma factor level of mild hemophilia A & B

20-40%

What is the desired plasma factor level of major hemophilia A & B

80-100%

What is the FDA approved monoclonal AB used to treat hemophilia

Emicizumab

What is the most common inherited bleeding disorder

VwF disease

VwF is a carrier for which factor

Factor 8

Differentiate between VwD types

- type 1: mild - quantitative - type 2: qualitative - type 3: severe - absent

What is the most common type in vwd?

Type 1

In vWd, which one will be affected | Aptt or pt

Aptt

In which type of vwd is riocetin reduced?

2B along with thrombocytopenia

What is the defect in type 2b vWd

Increased affinity to vWF to GP1b

In which types of vWb is desmopressin contraindicated and why?

- type 2B: thrombocytopenia | - type 3: lack of responsne

What are treatment options for vwd?

- Desmopressin - antifibrinolytic - factor concentration (humate-P) - OCP

What is the role of bleeding time in platelet disorders?

Abandoned

Platelet testings include:

- count - morphology - flow cytometery - PFA - electron microscopy

What is the etiology of bernard soulier syndrome?

AR, Lack of GP1b-IX-V receptor

What are the characterstics of bernard soulier?

- prolonged PFA - thrombocytopenia - large platelet

What is the etiology of Glanzzman thrombasthenia

Defect in GP2b-3a

Differentiate between platelt aggregation in bernard soulier syndrome vs glanzmann thrombosthenia

Glanzzman: reacts to all except risocetin Bernard: doesn’t react to risocetin

What is the origin of acquired bleeding disorders

Underlying disease | “Immune, liver, renal, DIC, vitamin & absorption”

What is the most common cause of acute thrombocytopenia in a healthy child?

ITP

What is the etiology of ITP?

Anti platelet glycoprotein antibodies

What are the viruses that could be associated with ITP?

EBV, CMV, H-pylori, MMR

How are the platelet distroyed in ITP?

Antibodies coat the platelet and are recognized by splenic macrophage to be destroyed

What are the findings in ITP?

All normal except for bleeding mainfestations “epistaxis, petechia, purpora, gum bleeding”

What are the positive findings of ITP?

- platelet count: <20x10^9 | - platelet size: normal to large

What is the triad of ITP?

Mild bleeding - normal PE - isolated thrombocytopenia

What is the management plan for ITP?

Supportive | - w\ IVIG, Prednisolone -

What determines the prognosis of ITP?

The time from presentation - acute: within 3 months - presistant: 3-12 months - chronic: >12months

What is the most common cause of pediatric thromboembolism?

Provoked (2ndary to something)

In which group of pediatric population is pediatric thromboembolism the greatest risk

Neonates | 2nd peak during puberty\adolescence

How to treat pediatric thromboembolism

Acute: heparin LMW | Subacute\chronic: warfarin + Heparin LMW

What is the most common cause of acquired thrombophilia

APS antiphospholipid syndrome

What are the positive lab findings in antiphospholipid syndrome?

- positive lupus anticoagulant AB in 2 testing 2 weeks apart - prolonged aPTT not corrected by mixing

How to treat antiphospholipid

Lifelong anticoagulant