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Haematology Revision > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is a bleeding disorder?

A

Where the person fails to clot there blood

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2
Q

What are the main 3 we are going to discuss?

A

Haemophilia
Von Willebrand’s disease
Thrombocytopenia

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3
Q

What are the 3 main pathophysiological of stopping bleeding and therefore where there can be disorders in?

A
  1. Vasoconstriction
  2. Gap-plugging by platelets
  3. Clotting cascade activation
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4
Q

What is Haemophilia A and B

A

Genetic disorder that impairs the body’s ability to make blood clots

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5
Q

What is the alternative name for Haemophilia B (Clue Ho Ho Ho)

A

Christmas disease

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6
Q

What kind of inheritance do the haemophilia show?

A

X-linked recessive

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7
Q

Can women get affected by haemophilia?

A

No it is X-linked Recessive as girls are XY, they can only be carriers

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8
Q

Are all haemophilia familial?

A

No, 1/3 is a new mutation

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9
Q

What is the epidemiology for the 2 haemophiliac

A

These are rare
1 in 10’000
1 in 60’000

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10
Q

What is the bleeding severity dependant on

A

The amount of residual coagulation factor activity

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11
Q

What are the patterns of bleeding?

A

Haemoarthrosis ie bleeding into the joint spaceL knees, ankles and elbows, patient won’t walk
Muscle haematoma ie big muscle swellingg in the thighs, gluten and ileopsoas
CNS bleed, Post-surgical bleed

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12
Q

What are some complications of Haemophilia?

A 
Sudden onset ICH
Synovitis 
Chronic haemophilic arthroplasty 
Neurovascualr compression and 
Stroke
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13
Q

What are the diagnostic tests for haemophilia?

A

Clinical diagnosis
Prolongued APTT, NORMAL PT, Nomal BT
Decresed factor VIII or IX
Genetic analysis

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14
Q

What is the management of A and B T?

A

Coagulation factor replacement factor VIII or IX, aim for levels of 2-5%
DDAVP = Desmopressin Injection which stimulates the vWF in Sx
Tranexamic Acid

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15
Q

What is the management of severe TP?

A

2-3x/wk coagulation factor replacement FVIII or IX

Physio, analgesia

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16
Q

What is vW disease?

A

Von Willebrand’s disease which is where there is no or decreased amounts of vWF

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17
Q

What is the genetic inheritance of VWD?

A

Autosomal dominant

18
Q

What is type 1 deficiency in VWD?

A

Quantative deficiency

19
Q

What is type 2 deficiency in VWD?

A

2 (A, B, M, N): qualitative deficiency determined by site of mutation in relation to vWF function

20
Q

What is type 3 deficiency in VWD?

A

Severe (complete deficiency) RARE; joint bleeding and co-cutaneous bleeding

21
Q

What is the prevalence of VWD?

A

1 in 200

22
Q

What are the clinical presentations of VWD?

A 
Platelet type bleeding: bleeding of the 
Mucosa
Epistaxis
Purpura
Menorrhagia and 
GI bleeds
23
Q

What are the findings on FBC?

A

Raised APTT and Bleeding time

Decreased Factor IIIc and vWF

24
Q

What is the treatment of VWD?

A 
Replacement of the vWF or DDVAP
Tranexamic acid
Topical applications and 
COC 
AVOID NSAIDs
25
Q

What is the definition of thrombocytopenia?

A

Aquired bleeding disorder where there are low platelets

26
Q

What are the decreased production causes of TP?

A

Marrow failure
aplasia
Infiltration

27
Q

What are the increased consumption causes of TP?

A

Immune ITP

Hypersplenism

28
Q

What is ITP?

A

Immune Thrombocytopenia purpura

29
Q

What are the common causes of ITP?

A

Infection EBV
Collagenosis
Lymphoma
Drug induced

30
Q

What is the adult and childhood presentation of ITP?

A

Adults slow and insidious onset and chronic. Usually DOESN’T follow infection
Children usually follows viral infection and is SELF LIMITING

31
Q

What is non-immune DIC

A

Tissue factor everywhere causing tiny clots everywhere causing multi-organ failure
Uses up platelets elsewhere causing thrombocytopenia

32
Q

What are the characteristic features of TP?

A

Non-blanching petechiae
Eccymosis
Mucosal bleeding
CNS bleed

33
Q

What is the diagnosis of ITP?

A

FBC, blood smear
Bone marrow biopsy
Only the PLATELET COUNT is abnormal

34
Q

What is the management of ITP?

A

AVOID NSAIDs and contact sports
Steroids
IV immunoglobulin
IV anti-D if Rh-ve
Splenectomy if severe acute bleeding or severe chronic bleeding
“Thrombopoietin analogues to stimulate megakaryocytes (ELTROMBOPAG, ROMIPLOSTIM) in chronic pts

35
Q

What is a Liver failure associated bleeding disorder?

A

A haemorrhage and prothrombin disorder due to liver failure because this is where the clotting factors etc are formed

36
Q

What are the 2 types of liver failure deficiencies?

A

Factor I, II, V, VII, VIII, IX, X, XI

Cholestasis Vit K dept factor deficiency: factor II, VII, IX, X.

37
Q

What is the classical sign of liver failure bleeding?

A

Bleeding form abnormal structural lesions such s varies

38
Q

What are the 2 FBC findings in liver failure bleeding?

A

Protongued PT and APTT

Decreased fibrinogen

39
Q

How you going to treat the LFB?

A

Replacement with FFP and Vitamin K

40
Q

What is haemorrhage disease of the newborn caused by ?

A

Immature coagulation systems so low in vitamin K

Vitamin K deficient diet (especially breast)

41
Q

What is the hallmark of HDofN?

A

Fatal and incapacitating haemorrhage?

42
Q

How are we going to prevent the outcome of HDofN?

A

vitamin K administration to mum PO/IM at birth

Haematology Revision (8 decks)

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