Bleeding disorders Flashcards

(37 cards)

1
Q

What does APTT measure?

A

Intrinsic pathway factors VIII, IX, XI, XII

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2
Q

What does PT measure?

A

Extrinsic pathway factors factor VII

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3
Q

What does TCT measure?

A

Conversion of fibrogen to fibrin clot

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4
Q

What does bleeding time measure?

A

Primary haemostasis e.g. platelet function

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5
Q

What are causes of an isolated prolonged PT?

A

Factor deficiency
Liver disease
Drugs e.g. warfarin apixaban..

Liver disease can however cause PT and APTT prolongation

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6
Q

What are causes of isolated prolonged APTT?

A

Haemophilias
Lupus anticoagulants
Factor deficiencies
Heparin (PT sometimes prolonged)

VWF, along with prolonged bleeding time

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7
Q

What are causes of prolonged PT and APTT?

A
Liver disease 
DIC
Common pathway deficiencies e.g II, V, X 
Combination factors deficiencies 
Vitamin K deficiency 

Anticoagulation

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8
Q

What are causes of prolonged bleeding time?

A

Aspirin (isolated prolonged)

DIC

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9
Q

How is warfarin over-anticoagulation identified and treated?

A

May present with IC bleed, other bleeding.

INR prolonged

Stopping and/or reducing dose
Oral or IV vitamin K
IV coagulation factors

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10
Q

How does haemophilia present and how is it treated

A
A = 8 (X-r)
B = 9 (X-r)
C = 11 (A-r)

Often presents with haemarthroses with prolonged APTT.

Give tranexamic acid, DDAVP, recombinant factor.

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11
Q

What is VWD?

A

Reduction in functioning VWF.

most common inherited bleeding disorder

Autosomal dominant

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12
Q

How does VWD present?

A

Symptoms of platelet disorder e.g. epistaxis, menorrhagia

Prolonged bleeding time, maybe APTT prolongation

Factor 8 reduction

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13
Q

How is VWD treated?

A

Tranexamic acid, DDAVP, factor 8

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14
Q

What is antiphospholipid syndrome?

A

Acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent foetal loss and thrombocytopenia.

Associated with SLE

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15
Q

How does antiphospholipid syndrome present

A

Paradoxical rise in APTT
Thrombosis
foetal loss
thrombocytopenia

other autoimmune disease, drugs

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16
Q

How is antiphospholipid syndrome treated?

A

Low dose aspirin in primary prophylaxis, warfarin for secondary

17
Q

What is DIC?

A

In DIC, the processes of coagulation and fibrinolysis are dysregulated, and the result is widespread clotting with resultant bleeding

18
Q

What are causes of DIC?

A

• Sepsis
• Trauma
• Malignancy
Obstetric complications

19
Q

What Ix and Rx in DIC?

A
  • low platelets
    • prolonged APTT, PT, and bleeding time
    • fibrin degradation products are often raised
    • schistocytes due to microangiopathic haemolytic anaemia

treat caues
FFP +/- platelets

20
Q

What Ix changes seen in liver coagulopathy

A

Causes prolonged APTT and PT due to decreased synthetic function of liver. Patients are also often vitamin K deficient

21
Q

What is ITP?

A

Immune mediated reduction in platelet count, with antibodies directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.

Kids get it post-infection, adults have more chronic disease

22
Q

How does ITP present?

A
  • may be detected incidentally following routine bloods
    • symptomatic patients may present with
    • petichae, purpura
    • bleeding (e.g. epistaxis, mucosal)
    • catastrophic bleeding (e.g. intracranial, retroperitoneal) is not a common presentation, occuring when platelets <10

Prolonged bleeding time

Platelets < 100

23
Q

What are triggers for ITP/

A

Infection, autoimmune condition e.g. SLE, RA

Often no trigger

24
Q

What is management of ITP?

A

Oral pred
Pooled IVIG

third will either have refractory disease or a subsequent relapse and require further and often long-term therapy such as with tPO-mimetics or splenectomy.

25
What is Evan's syndrome?
ITP in association with autoimmune haemolytic anaemia (AIHA)
26
What is MAHA?
A type of intravascular haemolysis
27
What are causes of MAHA>
``` HUS TTP Cardiac valves Malignancy DIC Vasculitis ```
28
What Ix in MAHA?
``` • Anaemia • Jaundice (elevated bilirubin) • Haemoglobinuria • Elevated LDH • Elevated reticulocyte count • Negative DAT Features specific to condition (renal failure in thrombotic microangiopathy) ```
29
What are thrombotic microangiopathies?
MAHAs associated with thrombocytopenia and thrombosis.
30
What is TTP?
A microangiopathy in the context of severe ADAMTS13 deficiency (<10%), responsbile for breaking down VWF multimers. This causes schiscotcytes and MAHA
31
What are causes of TTP?
Acquired: IgG antibodies to AMAMTS13 * Idiopathic * Pregnancy * Malignancy * HIV associated * Post-infective * Drugs e.g. OCP, ciclosporin, penicillin
32
What are features of TTP?
* rare, typically adult females * fever * fluctuating neuro signs (microemboli) * microangiopathic haemolytic anaemia * thrombocytopenia * renal failure
33
What is Rx of TTP?
• Plasma Exchange-cornerstone of acute therapy. | Immunosuppression
34
What is HUS?
A triad of • acute kidney injury • microangiopathic haemolytic anaemia • thrombocytopenia
35
What are causes of HUS?
Shiga toxin from E coli pneumococcal infection HIV SLE primary HUS: complement dysregulation
36
What Ix in HUS?
• full blood count: anaemia, thrombocytopaenia, fragmented blood film • U&E: acute kidney injury • stool culture ○ looking for evidence of STEC infection ○ PCR for Shiga toxins
37
What Rx in HUS?
Supportive e.g fluids, transfusion, dialysis Abx: not useful Plasma exchange if severe Eculizumab: C5 inhibitor MAB