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Phase 4 > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

What is the epedemiology of Von Willebrand syndrome?

A

Males and females affected equally.

More common in Afro-Caribbean population.

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2
Q

Which cells is Von Willebrand Factor protein made in?

A

Endothelial cells and megakaryocytes.

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3
Q

What happens if there is a deficiency of Von Willebrand factor?

A

Platelet dysfunction, predominantly mucosal bleeding.

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4
Q

Describe the features of type 1 von willebrand syndrome?

A

Usually have easy bruising, excessive bleeding from minor wounds and mucosal bleeding.
Bleeding in infancy is rare, but young children may bruise more easily and have problems with nosebleeds.
Girls may have menorrhagia.
Excessive bleeding after procedures involving mucous membranes, e.g. tonsillectomy or wisdom tooth extraction.

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5
Q

What are the features of type 2 and 3 Von Willebrand?

A

Bleeding symptoms may start at an earlier age than with type 1 and are often more severe.
Patients with menorrhagia, chronic epistaxis, or GI bleeding may be anaemic due to ongoing blood loss and iron deficiency.

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6
Q

What might be seen on examination in a patient with Von Willebrand?

A

Physical examination might be normal.
There may be signs of anaemia, e.g. pale conjunctiva etc.
Bruises are a non-specific finding.
Very large bruises or those found on the torso may be more significant.
Evidence of joint bleeding may be seen in those rare patients with a markedly decreased factor VIII

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7
Q

What are the investigations for Von Willebrand?

A

PT (extrinsic path)
APTT (can be normal unless factor VIII also low
FBC
VWF antigen, VWF activity and factor VIII activity
TFTs and immunoglobulins to exclude acquired
Need to concordant and abnormal tests for diagnosis.

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8
Q

What is the treatment for Von Willebrand?

A

Desmopressin (casues release of factor VIII and VWF from endothelial sttores by 3-5x within an hour)
Antifibrinolytic e.g. tranexamnic acid
Factor concentrate
Risk of anaphylaxis or antibody formation is low but can happen.

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9
Q

What is haemophilia characterised by?

A

Factor VIII or IX deficiency

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10
Q

What is the inheritance pattern in haemophilia?

A

X-linked.

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11
Q

What are the types of haemophilia?

A

A or B

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12
Q

What is deficient in haemophilia A?

A

Factor VIII

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13
Q

What is the prevalence of haemophilia A?

A

1 in 5000 males

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14
Q

What is deficient in haemophilia B?

A

Factor IX

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15
Q

What is the prevalence of haemophilia B?

A

1 in 30,000 males

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16
Q

What are factors VIII and IX crucial for in the intrinsic pathway?

A

Thrombin generation

17
Q

What happens to the blood in patients with haemophilia?

A

Delayed clot formation due to reduced thrombin generation. This leads to the formation of an unstable clot that is easily dislodged and can cause excessive bleeding.

18
Q

How is the severity of haemophilia rated?

A

Severe - <1% clotting factor level
Moderate - 1%-5% clotting factor level
Mild >5%-40% clotting factor level

19
Q

What are the investigations for haemophilia?

A

FBC (usually normal, excludes thrombocytopenia)
APTT (usually prlonged, should correct with mixing study)
Should trigger need for factor VIII and IX assays
PT ratio - for extrinsic and common path problems (usually normal, if not then factors V and VII checked)
LFTs (to ensure no other reason for abnormal clotting)
Plain film joint X-rays for acute or chronic haemarthrosis checks +/- arthropathy
MRI may be helpful if thinking of synovectomy
Scotpes/abdo US may be needed depending on suspected source of bleeding

20
Q

What is the management of haemophilia?

A

Long-term management of joint and muscle damage.
Education and promotion of self-guided care.
Physiotherapy and joint exercises for muscle strengthening
When acute bleeding occurs treatment should be instituted early

21
Q

What is the medical treatment for haemophilia?

A 
Factor VIII and IX replacement
Antifibrinolytics e.g. transexamic acid
Analgesia
Desmopressin if mild
Topics haemostatic agents e.g. fibrin sealants
22
Q

What is immune thrombocytopenia

A

Platelet count <100 x 10^9/L

23
Q

What can cause is the pathophysiology of immune thrombocytopenic purpura?

A

Secondary to an autoimmune phenomenon involving antibody destruction of peripheral platelets - T cells involved but unclear trigger. Splenic destruction of platelets, inhibition of platelet production, and destruction in bone marrow

24
Q

What can immune thrombocytopenic purpura be secondary to?

A 
HIV
Hepatitis C
H. pylori
Immunodeficiency
Immunological/autoimmune disorder
Lymphoprolierative disorders
Drug-induced - heparin, alcohol, quinines etc. 
Vaccine exposure (very rare)
25
Q

Who is immune thrombocytopenic purpura typically found in?

A

Children with preceeding viral illness and abrupt onset.

3:1 F:M

26
Q

What are the risk factors thrombocytopenia?

A

Women of child bearing age.

Males and females <10 or >65

27
Q

What are the signs of thrombocytopenia?

A

Petechiae
Haemorrhagic bullae 3-5mm in diameter on the mucosal surface of the oral cavity and tongue
Minor mucocutaneous bleeding (e.g. gum)
Large spontaneous bruising on arms and legs

28
Q

What are the investigations for immune thrompocytopenic purpura?

A 
Diagnosis of exclusion
FBC
Peripheral blood film
HIV
HEP C
TFTs
Immunoglobulins
29
Q

When is thrombocytopenia treated?

A

A platelet count of 20-30 x 10^9/L (patient treated above 30 based purely on platelet count have increased morbidity from therapy rather than from ITP)

30
Q

What is the management of ITP?

A

Life or organ threatening bleeding requires platelet transfusion, a corticosteroid and intravenous immunoglobulin.
Transexamic acid can stabilise clots.
Mycophenalate/rituximab or splenectomy may be required in chronic disease

31
Q

What are the features of thrombotic thrombocytopenic purpura?

A

Autoimmune

Microangiopathic haemolytic anaemia and purpura.

32
Q

What is thrombotic thrombocytopenic purpura caused by?

A

Increased release of Von Willebrand factor due to lack of ADAMTS-13.

33
Q

What does thrombotic thrombocytopenic purpura cause to happen in the body?

A

Massive platelet aggregation in areas of high vascular stress and microemboli.

34
Q

Which patients are most likely to get thrombotic thrombocytopenic purpura?

A

3:1 F:M

30-50 years

35
Q

What is the pentad of symptoms of thrombotic thrombocytopenic purpura?

A 
Microangiopathic haemolytic anaemia
Thombocytopenic purpura
Neurological symptoms (eg. headache, confusion, blurred vision, tinnitus, lethargy, seizures)
Fever
Renal disease
36
Q

How do you test for thrombotic thrombocytopenic purpura?

A 
FBC (anaemia, low Hb, low platelets)
Peripheral smear (shows microangiopathic haemolysis, presence of schistocytes)
Raised reticulocyte count. 
Lactate dehydrogenase increased
Bilirubin increased
Coag (normal)
D-dimer may be raised
Direct Coomb's test (negative - to rule out autoimmune haemolytic anaemia)
Urea and creatinine typically raised
37
Q

What is the management of thrombotic thrombocytopenic purpura?

A

Plasma exchange (90% respond within 3 weeks)
Steroids
Folic acid
Aspirin in selected patients (unless platelets <10)
Immunosuppression and splenectomy may be needed

38
Q

What are the differentials for thrombotic thrombocytopenic purpura?

A 
DIC
Sepsis
Haemolytic uraemic syndrome
ITP
Pre-eclampsia
HELLP
Leuakemia
Lymphoma 
Malignancy
SLE
Alcohol
Cirrhosis

Phase 4 (12 decks)

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