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HLB > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Inherited definition

A

traits present from birth and genetically derived from parents/ancestors

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2
Q

Acquired definition

A

traits that develop during a person’s lifetime. Not passed from one generation ot another.

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3
Q

Haemophilia/Bleeding Disorder definition

A

a problem with the blood clotting process, which can lead to abnormally heavy and prolonged bleeding. The bleeding can even be “spontaneous”

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4
Q

Causes of abnormal bleeding

A
  • blood vessel/vascular disorders
  • platelet disorders
  • coagulation pathway disorders
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5
Q

Examples of inherited blood vessel/vascular disorders (2):

A
  • hereditary haemorrhagic telangiectasia aka osler weber rendu syndrome
  • collagen abnormalities: Ehlers-Danlos
    Syndrome
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6
Q

Examples of acquired blood vessel/vascular disorders (3):

A
  • Scurvy (vitamin C deficiency)
  • Steroid Rx (Cushing’s syndrome)
  • Old age
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7
Q

  • common?
  • type of mutation
  • is
  • ## causes (3)
  • effect on platelet count
  • effect on coagulation screen
A
  • aka Osler-Weber-Rendu syndrome
  • uncommon
  • autosomal dominant
  • dilated microvascular swellings: skin,
    hands, fingertips, mucous membrane,
    internal organs
  • nose bleeds, GI bleeds
  • can cause iron deficiencies
  • swellings bleed when traumatised (little
    capillaries near the skin are very sensitive)
  • total platelet count normal
  • coagulation screen will be normal
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8
Q

Management of Hereditary Haemorrhagic Telangiectasia (5):

A
  • iron replacement
  • tranexanic acid (anti-fibrolytic prevents
    bleeds occurs)
  • hormonal therapy eg norethisterone
  • laser therapy on specific areas
  • local measures: nasal packs
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9
Q

Collagen/Connective Tissue Disorders: ehler’s-danlos syndrome:

  • is
  • clinical signs and blood tests
  • management
A
  • fragility of:
    - skin
    - ligaments
    - blood vessels*
    - perivascular connective tissue    *
  • easy bleeding and bruising because
    collagen is in vessel walls
  • total platelet count normal
  • coagulation screen will be normal
  • no specific management but useful to
    know if in labour etc
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10
Q

Scurvy:
- is
- clinical signs (3)
- history
- management

A
  • acquired vitamin C deficiency
  • bleeding gums
  • peri-follicular haemorrhages
  • bruises
  • must ask: alcohol abuse and diet
  • give vitamin C
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11
Q

Thrombocytopenia:
- is
- numbers
- symptoms seen when
- symptoms are

A
  • low platelet count
  • normal range = 150-400 x10^9/L
  • low = <105 x10^9/L
  • symptoms <10 (ish)
  • epistaxis, GI bleeds, menorrhagia, bruising,
    petechiae/purpura
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12
Q

Disorders of platelet numbers can be

A

inherited which are rare
acquired which are common

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13
Q

Disorders of platelet numbers: Acquired:

A

insert diagram

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14
Q

ITP:

A
  • immune thrombocytopenic purpura
  • auto-antibodies against platelets
  • leads to platelet removal by spleen
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15
Q

ITP in children:

A
  • acute condition in children
  • often follows an infection
  • self-limiting
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16
Q

ITP in adults:

A
  • acute/chronic condition in adults
  • many potential triggers
  • history is key
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17
Q

Are males or females affected more by ITP?

A

Both are equally affected

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18
Q

Treatment of ITP:

A
  • treat if bleeding or platelets <30 with:
  • steroids
  • IV immunoglobulins
  • TPO agonists (thrombopeotin hormone in
    bone marrow causes proliferation)
  • monoclonal antibodies
  • occasionally, splenectomy
19
Q

Disorders of platelet function:

A

insert table

20
Q

-
- clinical features (4)

A
  • x linked recessive mutation
  • Haemophilia A: Factors VIII deficiency
  • Haemophilia B: Factor IX deficiency aka
    christmas disease
  • Clinical Features:
  • spontaneous bleeding into joints and
    muscle
  • chronic debilitating joint disease
  • unexpected post-operative bleeding
  • family history in most cases (1 in 3 due to
    spontaneous mutations)
21
Q

Coagulation Cascade

A

insert diagram
- extrinsic: trauma damages blood vessels and exposes cells under the endothelial layer, exposing tissue factor embedded in membrane
F7 in blood binds to TF using calcium making a Via-TF complex

  • intrinsic: Circulating factor XII contacts negatively charged phosphates on platelets/subendothelial collagen, activating it to XIIa
    Which cleaves XI to Xia
    IX to IX a (with Ca)
    Meanwhile FVIII starts being cleaved to VIIIa by thrombin

FXIa and VIIIa enter common pathway

Fxa is generated which turns prothrombin (II) into thrombin Iia

Thrombin works as a co enzyme to turn the stabilising factor XIII into XIIIa as well as helping F1 turn into its activated form- 1a aka fibrin

all this thrombin generation ultimately therefore helps form a stable fibrin clot around the platelet plug already formed in haemostasis

22
Q

X linked recessive inheritance

A

haemophilia

Carrier mums: have 50% haemophiliac sons
and 50% carrier daughters

All the daughters of affected men become carriers
very rare for a daughter to be affected only if X0

23
Q

Coagulation disorders on cascade page

A

insert

24
Q

Tests of coagulation

A

insert

25
Q

Blood results in haemophilia A+B

A

insert
- intrinsic affected
- extrinsic not affected
- low factor 8 levels in haemophilia A
- low factor 9 levels in haemophilia B

26
Q

Treatment of Haemophilia

A

insert
prophylactic (preventive treatment)

27
Q

Von Willebrand’s Factor:

A
  • Von willebrand’s factor = large multimeric
    glycoprotein
  • synthesised by endothelial cells and
    megakaryocytes
  • acts as a brdiging molecule between
    platelet and collagen at sites of vascular
    injury
  • promotes platelet aggregation
28
Q

Von Willebrand’s Disease:
- is
- what % of population
- dominant or recessive
- in health does
- in disease causes

A
  • deficiency of Von Willebrand’s Factor and
    factor VIII in plasma
  • most common inherited clotting
    abnormality (1% population)
  • autosomal dominant
  • the VW factor carries factor VIII in the blod
    and binds platelets to sub-endothelial
    collagen
  • deficiency leads to:
    - mucocutaneous bleeds, nose bleeds,
    menorrhagia
    - clot formation does not happen as it
    should in an injury
29
Q

Blood Results in Von willebrands Disease:

A

insert

30
Q

Treatment of vWD

A
  • desmopressin = increases
    synthesis/release
  • anti-fibrinolytics = tranexamic acid, reduces
    thrombus breakdown
  • plasma products: replace VWF and Facto
    VIII
31
Q

Acquired Haemophilia:
- is
- hence
- how common
- causes
- how to treat

A
  • auto-antibodies act as inhibitors to clotting
    factors
  • effectively renders factor deficient
  • rare: acquired haemophilia A most
    common
  • look for underlying driver that has led to
    antibody formation:
    - malignancy
    - autoimmune
    - viral
    - pregnancy
  • complex to treat:
    - bypassing agents
    - immunosuppression
32
Q

Liver Disease

A
  • defects in synthesis of clotting proteins
  • some degree of “auto-anticoagulation”
  • bleeding disorders and thrombotic
    disorders possible
  • impaired platelet number and function
33
Q

What are phagocytes that destroy pathogens like bacteria that enter the liver called?

A

Kupffer cells e

34
Q

Vitamin K deficiency

A

Dietary:
- fat malabsorption
- biliary obstruction (jaundice)
- newborn state

Warfarin:
- PT time (INR) measures extrinsic
pathway
- Factors II,VII,X

35
Q

Vitamin K dependent Factors:

A
  • X,IX, VII,II and proteins S and C
  • required for gamma carboxylation of VK
    dependent coag factors turning them from
    non-functional to functional forms
  • Mnemonic: 1972 was the diSCo era
36
Q

Disseminated Intravascular Coagulation:

A
  • septicaemia: Mengingococcal, endotoxin in
    G-ve sepsis
  • acute promyelocytic leukaemia
  • cancer, trauma
  • pregnancy complication
  • release of tissue factor
  • breakdown balance of: coagulation,
    fibronolysis
  • clotting factors and platlets used up
37
Q

Blood Results in Disseminated Intravascular Coagulation

A
  • prolonged PT, APTT
  • low fibrinogen
  • low platelets
  • raised D-dimer
  • raised fibrin degradation products (FDPs)
  • red cell fragments on blood film
38
Q

Treatment of Disseminated Intravascular Coagulation:

A
  • ***treat underlying cause: eg antibiotics
    for sepsis
  • supportive Rx with blood products to try and correct coagulation abnormalities if bleeding
  • vitamin K
  • consider anti-coagulation
39
Q

What type of inheritance is seen with Hereditary Haemorrhagic Telangiectasia

1 = Autosomal Dominant
2 = Autosomal Recessive
3 = X-Linked Dominant
4 = X-Linked Recessive
5 = Y-Linked Recessive

A

1

40
Q

Bernard Soulier disease is due to a mutation in which molecule?

1 = GP0
2 = GP1b
3 = GP2b3a
4 = GP10a11b
5 = GP123
A

2

41
Q

In the absence of bleeding, when might you treat ITP?

1 = When platelets <10
2 = When platelets <20
3 = When platelets <30
4 = When platelets <50
5 = When platelets <150

A

3

42
Q

Haemophilia A is the deficiency of which factor

1 = Factor VII
2 = Factor VIII
3 = Factor IX
4 = Factor X
5 = Factor XI

A

2

43
Q

In addition to protein S and C, the Vitamin K dependent clotting factors are:

1 = Factors 1,3,5,7
2 = Factors 2,7,9,11
3 = Factors 2,3,5,10
4 = Factors 2,7,9,10
5 = Factors 7,9,11,12

A

2

44
Q

The APTT can be used as a screening test for the activity of which pathway?

1 = Common pathway
2 = Extrinsic pathway
3 = Intrinsic pathway
4 = Fibrin pathway
5 = Plasmin pathway

A

3

HLB (49 decks)

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