Bleeding Disorders

Question 1

What is a bleeding disorder?

Answer 1

Where the person fails to clot there blood

Question 2

What are the main 3 we are going to discuss?

Answer 2

Haemophilia
Von Willebrand’s disease
Thrombocytopenia

Question 3

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

Answer 3

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

Question 4

What is Haemophilia A and B

Answer 4

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

Question 5

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

Answer 5

Christmas disease

Question 6

What kind of inheritance do the haemophilia show?

Answer 6

X-linked recessive

Question 7

Can women get affected by haemophilia?

Answer 7

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

Question 8

Are all haemophilia familial?

Answer 8

No, 1/3 is a new mutation

Question 9

What is the epidemiology for the 2 haemophiliac

Answer 9

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

Question 10

What is the bleeding severity dependant on

Answer 10

The amount of residual coagulation factor activity

Question 11

What are the patterns of bleeding?

Answer 11

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

Question 12

What are some complications of Haemophilia?

Answer 12

Sudden onset ICH
Synovitis 
Chronic haemophilic arthroplasty 
Neurovascualr compression and 
Stroke

Question 13

What are the diagnostic tests for haemophilia?

Answer 13

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

Question 14

What is the management of A and B T?

Answer 14

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

Question 15

What is the management of severe TP?

Answer 15

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

Physio, analgesia

Question 16

What is vW disease?

Answer 16

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

Question 17

What is the genetic inheritance of VWD?

Answer 17

Autosomal dominant

Question 18

What is type 1 deficiency in VWD?

Answer 18

Quantative deficiency

Question 19

What is type 2 deficiency in VWD?

Answer 19

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

Question 20

What is type 3 deficiency in VWD?

Answer 20

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

Question 21

What is the prevalence of VWD?

Answer 21

1 in 200

Question 22

What are the clinical presentations of VWD?

Answer 22

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

Question 23

What are the findings on FBC?

Answer 23

Raised APTT and Bleeding time

Decreased Factor IIIc and vWF

Question 24

What is the treatment of VWD?

Answer 24

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

Question 25

What is the definition of thrombocytopenia?

Answer 25

Aquired bleeding disorder where there are low platelets

Question 26

What are the decreased production causes of TP?

Answer 26

Marrow failure aplasia Infiltration

Question 27

What are the increased consumption causes of TP?

Answer 27

Immune ITP | Hypersplenism

Question 28

What is ITP?

Answer 28

Immune Thrombocytopenia purpura

Question 29

What are the common causes of ITP?

Answer 29

Infection EBV Collagenosis Lymphoma Drug induced

Question 30

What is the adult and childhood presentation of ITP?

Answer 30

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

Question 31

What is non-immune DIC

Answer 31

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

Question 32

What are the characteristic features of TP?

Answer 32

Non-blanching petechiae Eccymosis Mucosal bleeding CNS bleed

Question 33

What is the diagnosis of ITP?

Answer 33

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

Question 34

What is the management of ITP?

Answer 34

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

Question 35

What is a Liver failure associated bleeding disorder?

Answer 35

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

Question 36

What are the 2 types of liver failure deficiencies?

Answer 36

Factor I, II, V, VII, VIII, IX, X, XI | Cholestasis Vit K dept factor deficiency: factor II, VII, IX, X.

Question 37

What is the classical sign of liver failure bleeding?

Answer 37

Bleeding form abnormal structural lesions such s varies

Question 38

What are the 2 FBC findings in liver failure bleeding?

Answer 38

Protongued PT and APTT | Decreased fibrinogen

Question 39

How you going to treat the LFB?

Answer 39

Replacement with FFP and Vitamin K

Question 40

What is haemorrhage disease of the newborn caused by ?

Answer 40

Immature coagulation systems so low in vitamin K | Vitamin K deficient diet (especially breast)

Question 41

What is the hallmark of HDofN?

Answer 41

Fatal and incapacitating haemorrhage?

Question 42

How are we going to prevent the outcome of HDofN?

Answer 42

vitamin K administration to mum PO/IM at birth