Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

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1
Q

what things are involved in the Normal Haemostatic Mechanisms?

A
  • Vessel Wall
  • Platelets
  • von Willebrand Factor (a large multimeric glycoprotein that performs two critical functions in primary hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion, and under high shear conditions, it promotes platelet aggregation)
  • Coagulation Factors -Adequate amounts of all of the correct coagulation factors
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2
Q

what is the Normal Haemostatic Response?

A

•Primary - Platelet Plug Formation

  • Platelets, vWF, Wall

•Secondary - Fibrin Plug Formation

When vessel wall is initially damaged the platelets stick to the collagen and the platelets then get sticky and stick to each other

Activators of coagulation released from damaged tissue is tissue factor and it activated the cascade of dominoes of coagulation factors leads to a stable fibrin plug on the platelet plug you formed

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3
Q

what does Diathesis mean?

A

Diathesis is a person’s predisposition or vulnerability to a medical condition, which can be a psychological or physical disorder

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4
Q

what is Haemorrhagic Diathesis?

A

Any quantitative or qualitative abnormality

Inhibition of function

  • Platelets
  • vWF
  • Coagulation factors
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5
Q

what are some important points you want to cover in your bleeding history?

A
  • Has the patient actually got a bleeding disorder
  • How severe is the disorder?
  • Pattern of Bleeding - Deficiency in different part of the process results in different patterns of bleeding - In haemophilia A we see bleeding into joints, cranium and retroperitoneal, But in severe thrombocytonia (platelet count less than 10) we see a much more mucosal pattern of bleeding
  • Congenital or Acquired - For individuals who have a congenital type try figure out the mode of inheritance by plotting a family tree
  • Mode of inheritance
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6
Q

what are some good things to ask about in relation to a history of bleeding?

A
  • Bruising
  • Epistaxis
  • Post-surgical bleeding - Best single question to ask is if they have ever had a surgical procedure and if there was bleeding after that procedure - Dental Surgery, Circumcision, Tonsillectomy, Appendicectomy
  • Menorrhagia - very uncommon not for them to have menorrhagia if they have things like deficiency of vWF
  • Post-partum haemorrhage - Post partum haemorrhage is not that significant as most women that have it will have it due to structural bleeding
  • Post-trauma
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7
Q

what is important to think about in relation to the severity of bleeding?

A

Hit with a baseball bat and bled – appropriate for the level of trauma that was experienced

Bruise after vaccine = normal

Massive haematoma = not normal

Massive haematoma after IM injection – then ask questions especially if on more than one occasion

Man standing and suddenly bleeding into knee = Spontenous bleeding = could be haemaphilia especially if the person is doing absolutely nothing

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8
Q

what is an important question to ask when you are trying to determine the severity of a bleed?

A

Ask the question – what does it take to make you bleed? If nothing then a severe disorder

Severe patters are way more obvious than mild patterns

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9
Q

Depending on what part of the coagulation pathway isn’t working then you get a different _______ of __________

A

pattern

bleeding

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10
Q

what are the different patterns of bleeding?

A

•Platelet type

  • Mucosal
  • Epistaxis
  • Purpura
  • Menorrhagia
  • GI

•Coagulation Factor

  • Articular
  • Muscle Haematoma
  • CNS
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11
Q

what is shown here?

A

Classical picture of bruises

Flecks aswell – bleeds into the dermis

When you see this patients ask and push one of the lesion and see if it blanches and this one shouldn’t

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12
Q

what is shown here?

A

Severe haemophilia

Picture of old haemophilia

Haemophilic athropathy

Inflammatory response in macrophages resulting in synovitis

Inflammation synovium prevents repair

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13
Q

what is shown here?

A

Big haematoma in thigh

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14
Q

what is shown here?

A

Rectus Sheath Haematoma

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15
Q

what is shown here?

A

Intracranial Haemorrhage in Haemophilia

Used to be the commonest cause of death in severe haemophilia

Dense area of bleeding

Midline shifted – not a good outcome

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16
Q

how do you determine if a bleeding disorder is congeital or aquired?

A
  • Previous Episodes ?
  • Age at first event
  • Previous surgical challenges
  • Associated History - Family history of a bleeding disorder

Ask about previous episodes – especially of trauma and surgical episodes, even if it was 20 years ago and want to know if they have abnormal bleeding as if they didn’t then unlikely it is a congenital bleeding disorder they are presenting with now and more likely to be acquired

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17
Q

how do you determine if it is a hereditary disorder?

A
  • Family members with similar history
  • Sex - From sex you can determine the nature of inheritance, weather it being X-linked or autosomal
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18
Q

what is shown here?

A

Classic family tree for a X linked disorder

Circle = women

Square = men

Black square = affected men

Victoria had a mutation in her factor 9 gene

Boy on right - X chromosome from mum and happens to be the one with the disorder one it and Y chromosome from dad – died at age of 31

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19
Q

what is shown here?

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A

Autosomal dominant pattern

vWF diseases tend to be autosomal dominant inheritance

20
Q

what is Haemophilia A and B?

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A
  • X-linked
  • Identical phenotypes - Pattern of bleeding is the same so you can only know by determine what coagulation factor is missing
  • 1 in 10,000 and 1 in 60,000
  • Severity of bleeding depends on the residual coagulation factor activity

Haemophilia is a mostly inherited genetic disorder that impairs the body’s ability to make blood clots, a process needed to stop bleeding. This results in people bleeding for a longer time after an injury, easy bruising, and an increased risk of bleeding inside joints or the brain.

Type A, the most common type, is caused by a deficiency of factor VIII. Type B hemophilia is caused by a deficiency of factor IX

21
Q

what are the different severities of haemophilia?

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A
  • <1% Severe - spontaneous unprovoked bleeding
  • 1-5% Moderate - not spontaneously but will bleed after surgery and trauma if not managed appropriately
  • 5-30% Mild
22
Q

what are the clinical features of Haemophilia?

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A
  • Haemarthrosis (haemorrhage into a joint space)
  • Muscle haematoma (bleeding within a muscle group)
  • CNS bleeding
  • Retroperitoneal bleeding (accumulation of blood found in the retroperitoneal space)
  • Post surgical bleeding
23
Q

what is shown here?

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A

•Haemarthrosis (haemorrhage into a joint space)

24
Q

what is shown here?

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A

Injury

Man hit with tennis ball

Severe injury for being hit with tennis ball

haemophilia

25
what is shown here?
Post mortum sample, retroperitoneal haematoma in severe haemophilia A unprovoked
26
what are some haemophilia clinical complication?
* Synovitis * Chronic Haemophilic Arthropathy (any disease of the joints, A joint is a place where two bones contact each other) * Neurovascular compression (compartment syndromes) * Other sequelae of bleeding (Stroke)
27
what is shown here?
End stage haemophilic arthropathy Malalignment Joint space obliterated
28
how is a Haemophilia Diagnosis made?
* Clinical * Prolonged APTT (Activated Partial Thromboplastin Clotting Time) - measures how long it takes your blood to form a clot. The aPTT test can be used to look at how well those clotting factors are working * Normal PT * Reduced FVIII or FIX * Genetic analysis - Can do a genetic analysis to see what it is in the family that is causing that deficiency, Good for future planning and deciding about future pregnancies
29
what is Haemophilia treatment of bleeding diathesis?
* Coagulation factor replacement FVIII/IX (main method treatment for this disease) * Now almost entirely recombinant products * DDAVP (natural analogue of vasopressin, not used for all haemophilic bleeding) * Tranexamic Acid * Emphasis on prophylaxis in severe haemophilia (severe haemophilia – as they can bleed spontaneously) * Gene therapy? (big improvements in the last few years, taking the normal noninfected gene and put into into the patients hepatocytes so it can make normal factor 8 or 9 for the patient)
30
what is Haemophilia Treatment?
* Splints * Physiotherapy - to prevent muscle loss * Analgesia - episodes of severe pain to allow patients to mobilise and not be stuck in bed * Synovectomy - in advanced joint disease * Joint replacement - when severe haemophilia has been established
31
what are the complications of haemophilia treatment?
* Viral infection - HIV, HBV, HCV,, Others/ vCJD? * Inhibitors - Anti FVIII Ab (In patients may have antibodies against factor 8 concentrate, rare), Rare in FIX * DDAVP - not used in MI, Hyponatraemia(babies)
32
what is von Willebrand Disease?
* Common (1 in 200) * Variable severity * Autosomal (not X-linked) * Platelet Type bleeding (mucosal) * Quantitative and qualitative abnormalities of vWF Commonest of all bleeding disorders a common inherited condition that can make you bleed more easily than normal. People with VWD have low level of von Willebrand factor in their blood, or it does not work very well. Von Willebrand factor helps blood cells stick together (clot) when you bleed. If there's not enough of it or it does not work properly, it takes longer for bleeding to stop. There's currently no cure for VWD, but it does not usually cause serious problems and most people with it can live normal, active lives.
33
what is the inheritence pattern of vWF disease?
autosomal dominant Affects men and women in each generation
34
Great to have around when injured a blood vessel as sticks to many things, one of the key things responsible for holding everything together what are the different kinds of Von Willebrand Disease?
* Type 1 quantitative deficiency - all of it is there, normal molecule and functions well but just not enough of it, may be reduction production or increased clearance * Type 2 (A,B,M,N) qualitative deficency determined by the site of mutation in relation to vWF function – nature of abnormality depends on site of mutation, what bit of vWF is taken out * Type 3 severe (complete) deficiency – there is no molecule, these patients need the most treatment
35
what is the treatment of Von Willebrand Disease?
* vWF concentrate or DDAVP - In type 1 you can use DDAVP to elevate vWF level (cant use in babies or patients with significant cardiac problems) * Tranexamic Acid - useful for help in heavy menstruation * Topical applications * OCP etc - Oral contraceptive pill good for reducing severity of menorrhagia
36
what are examples of Acquired Bleeding Disorders?
* Thrombocytopenia * Liver failure * Renal failure * DIC * Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran, Bivalirudin …….. (Commonest group is the bottom one- bleeding related to drugs)
37
what is the cause of Thrombocytopenia?
* Decreased production - Marrow failure, Aplasia ,Infiltration * Increased consumption - Immune ITP, Non immune DIC, Hypersplenism
38
how does thrombocytopenia present?
* Petechia (tiny red, flat spots that appear on your skin. They're caused by bleeding) * Ecchymosis (bruise) * Mucosal Bleeding * Rare CNS bleeding
39
what is shown here? Petechia Platelet count less than 20 in petechia Non-blanching
Petechia on face Doesn’t blanch on skin Blood blisters on tounge Indicative of mucosal bleeding
40
who gets ITP?
children and adults
41
what are the associations with ITP?
Infection esp EBV,HIV (Particularly seen after infection) Collagenosis Lymphoma Drug induced
42
when you look at a patient with ITP what do you see?
•Blood isolated thrombocytopenia - Blood is normal except they have a very low platelet count
43
how do you treat ITP?
try elevate the platelet count •Steroids, IV IgG (reduces uptake of sensitized platelets by the spleen), Splenectomy, Thrombopoietin analogues (Eltrombopag and romiplostim) (mimic the effect of thrombopoietin on the thrombopoietin receptor)
44
do patients with liver failure bleed and if so why?
Liver failure do often bleed Associated with increased risk of bleeding and thrombosis •Liver Produces - Factor I, II, V, VII, VIII, IX, X, XI patient with liver failure who is not making any of these will have Prolonged PT, APTT Reduced Fibrinogen
45
liver can cause coagulation deficiency through Cholestasis, how does this cause it?
biliary tree is obstructured and no bile in small intestine and therefore cant absorb vitamin K * Vit K dept factor deficiency Factor II, VII, IX, X. * Replacement FFP * Vitamin K
46
explain what is shown here:
Iiver responsible for making procoagulants and anticoagulants – all produced in the liver Balance of procoagulants and anticoagulants in a healthy individual B. In liver disease, any little insult in any direction can result in bleeding or thrombosis In patients with liver disease definitive increase in both bleeding and thrombosis
47
what is Haemorrhagic Disease of the Newborn?
* Immature Coagulation Systems * Vitamin K (2,7 9 and 10) deficient diet (esp Breast) * Fatal and incapacitating haemorrhage * Completely preventable by administration of vitamin K at birth (I.M vs P.O) Given vitamin K injection to prevent haemorrhagic disease of the new born Vitamin K supplementation – condition that has become completely avoid – almost no cases in the western world
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