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Haematology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

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
Q

What is Evan’s syndrome?

A

ITP in association with autoimmune haemolytic anaemia (AIHA)

26
Q

What is MAHA?

A

A type of intravascular haemolysis

27
Q

What are causes of MAHA>

A 
HUS
TTP
Cardiac valves
Malignancy 
DIC
Vasculitis
28
Q

What Ix in MAHA?

A 
• Anaemia
	• Jaundice (elevated bilirubin)
	• Haemoglobinuria
	• Elevated LDH
	• Elevated reticulocyte count
	• Negative DAT
Features specific to condition (renal failure in thrombotic microangiopathy)
29
Q

What are thrombotic microangiopathies?

A

MAHAs associated with thrombocytopenia and thrombosis.

30
Q

What is TTP?

A

A microangiopathy in the context of severe ADAMTS13 deficiency (<10%), responsbile for breaking down VWF multimers. This causes schiscotcytes and MAHA

31
Q

What are causes of TTP?

A

Acquired: IgG antibodies to AMAMTS13

* Idiopathic
* Pregnancy
* Malignancy
* HIV associated
* Post-infective 
* Drugs e.g. OCP, ciclosporin, penicillin
32
Q

What are features of TTP?

A
  • rare, typically adult females
    • fever
    • fluctuating neuro signs (microemboli)
    • microangiopathic haemolytic anaemia
    • thrombocytopenia
    • renal failure
33
Q

What is Rx of TTP?

A

• Plasma Exchange-cornerstone of acute therapy.

Immunosuppression

34
Q

What is HUS?

A

A triad of
• acute kidney injury
• microangiopathic haemolytic anaemia
• thrombocytopenia

35
Q

What are causes of HUS?

A

Shiga toxin from E coli
pneumococcal infection
HIV
SLE

primary HUS: complement dysregulation

36
Q

What Ix in HUS?

A

• 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
Q

What Rx in HUS?

A

Supportive e.g fluids, transfusion, dialysis

Abx: not useful

Plasma exchange if severe
Eculizumab: C5 inhibitor MAB

Haematology (6 decks)

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