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Haematology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

In normal haemostasis what is the function of the vessel wall?

A
  • Maintain “intactness”
  • Encourage blood flow
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2
Q

What is the primary normal haemostatic response?

A
  • Platelet Plug Formation, Platelets, vWF, Wall
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3
Q

What is the secondary haemostatic response?

A
  • Secondary Fibrin Plug Formation
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4
Q

What is haemorrhagic diathesis?

A

Unusual susceptibility to bleeding

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5
Q

What is usually the cause of haemorrhagic diathesis?

A

Any quantitative or qualitative abnormality

Inhibition of function

  • Platelets
  • vWF
  • Coagulation factors
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6
Q

What are the key points for bleeding history?

A
  • Has the patient actually got a bleeding disorder
  • How severe is the disorder?
  • Pattern of Bleeding
  • Congenital or Acquired
  • Mode of inheritance
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7
Q

What is included in the history of bleeding?

A
  • Bruising
  • Epistaxis
    • (The above two are less relevent)
  • Post-surgical bleeding
    • Dental Surgery
    • Circumcision
    • Tonsillectomy
    • Appendicectomy (if answer is no to any post surgical then unlikely to have a bleeding disorder
  • Menorrhagia
  • Post-partum haemorrhage
  • Post-trauma
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8
Q

What is the relevance of spontaneity of bleeding?

A
  • Unprovoked bleeding is completely pathological
  • Easily provoked bleeding needs investigated
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9
Q

Pattern of bleeding

How can we tell if it’s a platelet problem?

Coagulation factor problem?

A
  • Platelet type
    • Mucosal
    • Epistaxis – recurrent and doesn’t stop
    • Purpura – red/purple discoloured spots
    • Menorrhagia
    • GI
  • Coagulation Factor Articular – bleeding into joints (severe haemophilia)
    • Muscle Haematoma
    • CNS
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10
Q

How can we tell if it is petichia?

A

Press them and will not blanch

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11
Q

Which joints are worse for bleeding disorders?

A
  • hinge joints
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12
Q

What are characteristics of Intracranial Haemorrhage in Haemophilia?

A
  • Headache, focal signs, often death – massive haematoma, oedema, poor prognosis
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13
Q

How can we tell if a bleeding disorder is congenital or acquired?

A
  • If we think it is a new bleeding disorder then that must have been acquired.
  • If had previous episodes then likely to be congenital
    • Age at first event
    • Previous surgical challenges
    • Associated History
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14
Q

What are other characteristics that point to a hereditary disorder?

A
  • Family members with similar history
  • Sex
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15
Q

Please see image as example of x linked disorder:

A
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16
Q

Please see image as example of autosomal dominant:

A
17
Q

What are the genetic and general characteristics of haemophilia A and B?

A
  • X-linked
  • Identical phenotypes
  • 1 in 10,000 and 1 in 60,000
  • Severity of bleeding depends on the residual coagulation factor activity
  • <1% Severe – bleeds without provication, coagulation factors of less than 1 %
  • 1-5% Moderate
  • 5-30% Mild
18
Q

What are the haemophilia clinical features?

A
  • Haemarthrosis (bleeding into joints)
  • Muscle haematoma
  • CNS bleeding
  • Retroperitoneal bleeding
  • Post surgical bleeding – preventable
19
Q

Which age group suffer from more severe sinovitis?

A
  • Old men have more swollen sinovitis compared with young men
20
Q

What are the clinical complications of haemophilia?

A
  • Synovitis
  • Chronic Haemophilic Arthropathy
  • Neurovascular compression (compartment syndromes)
  • Other sequelae of bleeding (Stroke)
  • Patients often need joint replacement
21
Q

Dx of haemophilia?

A
  • Clinical
    • Present within 6 months and 2 years: kid stops walking.
    • Do not consider inappropriate trauma without exploring bleeding disorder
    • Present with joint swelling
  • Prolonged APTT
    • Normal PT
    • Reduced FVIII or FIX
  • Genetic analysis
22
Q

What is the treatment of haemophilia?

A
  • Coagulation factor replacement FVIII/IX
  • Now almost entirely recombinant products
  • DDAVP
  • Tranexamic Acid
  • Emphasis on prophylaxis in severe haemophilia
    • given factor 8 and 9, aiming to keep the trough level high enough so that bois do not have a reduced factor level, so can expect them to have zero bleeds.
23
Q

Other treatments for haemophilia?

A
  • Splints
  • Physiotherapy
  • Analgesia
  • Synovectomy
  • Joint replacement
24
Q

What are complications of treatment of haemophilia?

A
  • Viral infection
    • HIV
    • HBV, HCV,
    • Others/ vCJD?
  • Inhibitors Anti FVIII Ab
    • Rare in FIX (because there are fewer null mutations)
  • DDAVP (releases stored factor 8): MI
    • Hyponatraemia(babies)
25
Q

What are the genetic and general characteristics of von Willebrand disease?

A
  • Common (1 in 200)
  • Variable severity
  • Autosomal
  • Platelet Type bleeding (mucosal)
  • Quantitative and qualitative abnormalities of vWF
26
Q

How do we treat vWB disease?

A
  • vWF concentrate or DDAVP
  • Tranexamic Acid
  • Topical applications
  • OCP etc
27
Q

What are some of the common acquired bleeding disorders?

A
  • Thrombocytopenia
  • Liver failure
  • Renal failure
  • DIC
  • Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran, Bivalirudin ……..
28
Q

What is thrombocytopenia?

A

condition characterized by abnormally low levels of thrombocytes, also known as platelets, in the blood

Not making enough platelets or you are but you are breaking them down.

  • Decreased production: marrow failure, aplasia, infiltration
  • Increased consumption: Immune ITP, Non immune DIC, hypersplenism
29
Q

What are the clinical signs of thrombocytopenia?

A
  • Petechia
  • Ecchymosis
  • Mucosal Bleeding
  • Rare CNS bleeding
30
Q

What is ITP?

A

Immune thrombocytopenic purpura

Associated with: infection, lymphoma, drug induced

Treat? Steroids, IV IG, Splenectomy, Thrombopoietin analogues

31
Q

What can we do in liver failure?

A
  • Replacement FFP
  • Vitamin K

Haematology (16 decks)

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